APA Style.
Please note that you have to use the file attached and internet to answer the questions. The most information you can find in the file.
QUESTIONS
1.- Use the following terms for physical challenges in a report that discusses the manifestations or s/s of these disorders:Cleft palateDevelopmental hip dysplasiaHydrocephalusOmphaloceleWhat are the nursing care for these challenges bearing the newborn and family in mind?2.- Make a list of nursing diagnoses and expected outcomes for the following patients:A newborn is born with syndactyly.A newborn is diagnosed with atresia.A newborn is diagnosed with a volvulus.3.- Use the Internet to research a congenital anomaly or physical disability in a newborn. You might want to use the following conditions from the textbook:AnkyloglossiaThyroglossal cystPierre Robin syndrome
CHAPTER 27 Nursing Care of the Child Born With a Physical or Developmental Challenge
Few things can change the usually joyous tone of a birthing room faster than the birth of a baby with a physical
or developmental challenge. Primary care providers who are used to saying mentioning the “perfect” boy or girl
find themselves without words. The usual congratulatory remarks hang unsaid in the air.
When a newborn is born with an apparent physical or developmental challenge, nurses play a major role in
supporting and educating the parents to promote bonding. With some newborns, their congenital disorder may
be corrected with surgery, and they will have no long-term sequela. With other newborns, the congenital
disorders may require long-term care even with surgical correction. This chapter covers physical disorders of
the skeletal, gastrointestinal, and neurologic systems that are apparent at birth or recognized soon after.
Congenital disorders of the cardiovascular system are addressed in Chapter 41.
Birth defects remain a major public health burden. The Centers for Disease Control and Prevention (CDC)
estimates that 3% of all newborns born in the United States are affected by a birth defect and 20% of all infant
deaths are attributed to birth defects (CDC, 2016). Box 27.1 shows 2020 National Health Goals related to
decreasing the number of newborns born with congenital anomalies.
BOX 27.1
Nursing Care Planning Based on 2020 National Health Goals
Many congenital anomalies, such as a cleft lip, an omphalocele, and neural tube disorders, can be detected by
sonogram during intrauterine life. The following 2020 National Health Goals address the importance of
prevention and therapy postbirth:
• Increase the proportion of women delivering a live birth who took multivitamins/folic acid prior to
pregnancy from a baseline of 30.1% to 33.1%.
• Reduce the occurrence of spina bifida from 34.2 per 100,000 live births to 30.8 per 100,000 live births per
year.
• Reduce the occurrence of anencephaly from 24.6 per 100,000 live births to 22.1 per 100,000 live births per
year.
• Increase the proportion of children with special healthcare needs who have access to a medical home from
47.1% to 51.8%.
• Increase the proportion of children aged 0 to 11 years with special healthcare needs who receive their care in
family-centered, comprehensive, and coordinated systems from a baseline of 20.4% to 22.4%; and in
children aged 12 to 17 years from 13.7% to 15.1% (U.S. Department of Health and Human Services, 2010;
see www.healthypeople.gov).
Nurses can help the nation achieve these goals by urging women to enter pregnancy with an adequate folic acid
level, ensuring women obtain prenatal care, and receive comprehensive advice and support after diagnosis of a
fetal or newborn disorder.
FOR CARE OF A NEWBORN WHO IS PHYSICALLY OR DEVELOPMENTALLY
CHALLENGED
ASSESSMENT
The nursing assessment of a newborn who is physically or developmentally challenged focuses on determining
the infant’s immediate physiologic needs required to sustain life and the parents’ immediate emotional needs to
promote bonding. These are not different that those of all newborns. The following eight primary needs of
newborns should be assessed for signs that they are being established considering the newborn’s physical and
developmental challenges:
• Adequate respiration
• Extrauterine circulation
• Body temperature stabilization
• Blood sugar stabilization
• Prevention of infection
• An infant–parent bond
• Adequate stimulation
• Ability to take in adequate nutrients
• Ability to achieve waste elimination
Anomalies that affect a newborn’s appearance may have the most immediate effect on the parents’ ability to
establish a positive bond with their child. It is important, however, not to jump to conclusions about what will
be the parents’ response. An assessment of the family’s verbal and nonverbal responses may reveal that parents
are prepared and agreeable to meet this infant’s special needs. The parent’s adjustment may be affected by their
prior knowledge of their child’s physical or developmental challenge.
NURSING DIAGNOSIS
Many nursing diagnoses established for children who are physically or developmentally challenged address the
effect of the disorder on body function, including the child’s primary needs and the family’s coping ability
(Farrell & Krahn, 2014).
Examples of possible diagnoses include:
• Imbalanced nutrition, less than body requirements, related to inability to take in adequate nutrition
secondary to a physical challenge
• Impaired physical mobility related to congenital anomaly
• Risk for impaired parenting related to the birth of child with a congenital anomaly
• Anticipatory grieving (parental) related to loss of the idea of the “perfect” child
OUTCOME IDENTIFICATION AND PLANNING
Nurses play an important role in providing immediate care to high-risk newborns at birth as well as stabilizing
them until the pediatric team arrives to assume care or transport the newborn to a high-risk nursery.
Consideration of the family’s resources, both emotional and financial, is an important aspect of planning care
and establishing expected outcomes (Nes, Røysamb, Hauge, et al., 2014). It’s important to consider both the
short- and long-term needs of the newborn and how these needs may affect the family. Supportive family
members can be a critical asset to parental adjustment (Carmichael, Ma, Tinker, et al., 2014). Providing nursing
support as part of an interdisciplinary team, including social workers, therapists, nutritionists, medical
specialists, and other community resources can also help with parental adjustment. Refer parents to helpful
websites and other resources when appropriate (see Chapter 20).
IMPLEMENTATION
Nursing interventions for a newborn who is physically challenged include immediate life-sustaining measures
such as providing oxygen or adequate intake of nutrients when a disorder prevents the infant from establishing
respirations or sucking. Encouraging skin-to-skin contact and interacting with the newborn promotes infant–
parent bonding. Educating the parents about procedures the infant may undergo lessens the parental anxiety and
enhances self-esteem.
Parents experience the same stages of grief: denial, anger, bargaining, depression, and adjustment as those
whose child has died at birth (see Chapter 56). It is important for the nurse to provide positive role modeling
when caring for the emotional and physical needs of the newborn; it helps the parents to adjust to parenting a
child born with a physical or developmental challenge.
OUTCOME EVALUATION
Outcome evaluation should focus on establishing expected outcomes for the child’s physical and developmental
health needs as well as the family’s coping ability for current and future health of the child. This includes
addressing the family concerns and providing resources to support the family during and after discharge.
Examples of expected outcomes may include:
• Parent describes positive features of child by 2 weeks.
• Parents state they are comfortable with enteral feeding by 1 month.
• Child is ambulatory with walker or wheelchair by 2 years of age.
Care at Birth of the Newborn Who Is Physically or Developmentally
Challenged
The pediatric provider or neonatologist typically provides the parents with medical information regarding the
health status of their newborn. This information should be provided to the parents as quickly and accurately as
possible. The nurse can provide support to the family by keeping them informed of their newborn’s health status
and facilitating their interaction with the healthcare team. It is distressing for parents, who expect to hold their
newborn immediately after birth, to watch their newborn undergo a medical evaluation and medical intervention
while physically separated from their newborn (Box 27.2).
BOX 27.2
Nursing Care Planning to Respect Cultural Diversity
The causes of most congenital anomalies are unknown, although they probably arise from a combination of
environmental and genetic factors. New parents need an explanation of their child’s disorder and a chance to
talk about why they believe their child’s disorder occurred. Despite the evidence, many cultural myths persist
around congenital anomalies. Many people persist in believing infants with congenital anomalies are born to
people less deserving than others. Common beliefs include myths such as being looked on by an evil eye (mal
de ojo in Spanish) can cause a deformity. Parents may also blame themselves for their child’s condition
thinking something they did or didn’t do may have caused it. Common beliefs also include the idea that eating
raisins causes brown birth marks and eating strawberries causes red hemangiomas. Educating parents relieves
any guilt that they were the cause of the anomaly, helping them regain sufficient self-esteem to raise a child
with a congenital disorder.
When the parents are with their newborn, the nurse can begin by describing the newborn’s physical condition,
related to the diagnosis, to the parents. Medical equipment and its purpose should also be explained. Parents
should be given the opportunity to ask questions and interact with their newborn. Comments by the nurse
related to normal newborn observations can assist the parents in relating their newborn. A typical explanation
for Maia, for example, might be, “When your doctor placed your baby on your abdomen, you might have
noticed that your baby’s spinal cord isn’t completely formed, something called a meningocele. Although that
could be more extensive, at first inspection, it seems to be a problem that can be repaired. Her hips may also
need some treatment. I’ll bring the baby’s Isolette over so you can see her. Notice how bright-eyed and alert she
is for just being born.” Referring to the newborn by their name is helpful to personalize the interaction.
Physical and Developmental Disorders of the Skeletal System
Either genetic or environmental factors can compromise fetal physical growth to such an extent that they result
in skeletal disorders in the newborn.
ABSENT OR MALFORMED EXTREMITIES
Congenital skeletal disorders can result from reasons such as maternal drug ingestion or virus invasion or
amniotic band formation in utero. In most instances, however, the cause of the anomaly is unknown. Children
born without an extremity or with a malformed extremity can be fitted with a prosthesis as early in life as about
6 months so the infant can learn to stand at the normal time or handle and explore objects readily. However, it
may be adventitious to allow the child to grow and learn to use their altered body or limb without a prosthesis.
Introducing a prosthesis early in life may prevent a child from adjusting to a missing extremity, for example,
such as learning to write with their feet or sliding across the floor rather than walking. Often, parents and
therapists will teach children in therapy to function both with and without a prosthesis. Children are resilient
and can become so proficient at these adjustments in their born deformity that later in life they may not see any
advantage to using a prosthesis. Those affected by a skeletal anomaly can choose to use a prosthesis as an adult
or not.
Depending on the condition, in many children, there is a potential for better function if the malformed
portion of an extremity is amputated before a prosthesis is fitted. This creates a difficult decision for parents
because it is one they cannot undo later. They need assurance that hands with malformed fingers, for example,
will not later grow to become normal and that a well-fitted prosthesis will allow their child a more usual
childhood and adult life than if the original disorder was left unchanged (Fig. 27.1).
Figure 27.1 A young child learns to use a hand prosthesis during play. (M. Grecco/Stock Boston.)
Learning to use a hand prosthesis takes weeks to months, and it also involves therapy. It helps if parents can
think of interesting activities when introducing the prosthesis so the child can immediately see how useful it
will be to use. Gait training for the use of lower extremity prostheses begins with the use of parallel bars and
proceeds to independent walking and mastery of steps. Again, suggesting activities the child needs to walk to do
offers motivation for trying to use the prosthesis.
Children who are born with an absent extremity may need help not only in mastering the use of a prosthesis
but also in forming a positive body image of themselves as whole. If possible, in the newborn period, introduce
parents to the rehabilitation team who will be following their child. Further steps will then be outlined to help
them move past the helplessness they may be feeling to more positive actions. Visiting with a child who uses a
prosthesis well can be a great help in convincing parents that their child can lead a normal life. Young children
with a congenital extremity may not grieve over the lost extremity as do adults or older children.
FINGER AND TOE CONDITIONS
Finger or hand deformities occur in about 3% of all births. Polydactyly is the presence of one or more
additional fingers or toes. When an entire extra finger or toe forms, the supernumerary digit is usually
amputated in infancy or early childhood. These extra fingers are often just cartilage or skin tags, and removal is
simple and cosmetically sound. In syndactyly (two fingers or toes are fused), the fusion is usually caused by a
simple webbing (Fig. 27.2); separation of the digits into two sound and cosmetically appealing ones is usually
successful. In other instances, the bones of the fingers or toes are also fused, and cosmetic appearance and
function cannot be fully reconstructed (Sullivan & Adkinson, 2016).
These digit anomalies are always upsetting to parents (one of the first things new parents do is count the
fingers and toes of newborns) and may cause them to view their infant as defective rather than being an infant
with a simple anomaly. Encourage them to air their feelings and concerns as they deliver the news to family and
friends. Because hands are so important for writing, self-care, or computing, they may need reassurance at
health maintenance visits for the first few years of their child’s life that the child is perfect in other ways so they
can accept and help the child develop self-esteem. Children may need this same type of assurance as they grow
older so they can think of themselves as well people. Often, identifying a skill or talent that the child excels in
can provide another avenue for developing self-esteem. There are many examples of children with one limb or a
deformity playing sports, such as swimming, excelling in the arts, or playing musical instruments. If a child
expresses a desire to learn these activities, allowing the child to participate (if medically safe and sound)
typically leads to profound beneficial outcomes for all.
CHEST DEVIATIONS
Pectus excavatum, or “funnel chest,” is an indentation of the lower portion of the sternum. It is the most
common congenital deformity of the anterior chest, occurs in about 1 out of 500 live births, and affects boys 4
times more often than girls. The concern may not be present at birth but becomes more obvious as the child
grows to school age or adolescence. As a result of the deformity, lung volume is apt to be decreased and the
heart is displaced to the left. The condition can be repaired, for either cosmetic reasons or physiologic reasons,
such as to expand lung volume (Abdullah & Harris, 2016). With pectus carinatum, the sternum is displaced
anteriorly, increasing the anteroposterior diameter of the chest. This anomaly can be surgically corrected
(Tikka, Kalkat, Bishay, et al., 2016).
TORTICOLLIS (WRY NECK)
Torticollis is a term derived from the terms tortus (“twisted”) and collum (“neck”). Torticollis (wry neck)
occurs as a congenital anomaly when the sternocleidomastoid muscle is injured and bleeds during birth (Ryu,
Kim, Kim, et al., 2016). This tends to occur in newborns with wide shoulders when pressure is exerted on the
head to deliver the shoulder either with a vaginal or cesarean birth. The infant holds the head tilted to the same
side as the muscle that is involved; the chin rotates to the opposite side. The injury may not be noticeable in the
newborn and may become evident only as the original hemorrhage recedes and fibrous contraction occurs at 1
to 2 months of age. A thick mass over the muscle can usually be palpated at that time.
To relieve torticollis, parents need to begin a program of passive stretching exercises and therapy, laying the
infant on a flat surface and rotating the head through a full range of motion. Often, pediatric physical therapists
are involved in the home therapy treatment for infants with torticollis. In addition, parents should always
encourage the infant to look in the direction of the affected muscle. They can encourage this by holding the
child to feed in such a position that the child must look in the desired direction. Placing a mobile on the child’s
crib can encourage the child to look toward the affected side. Speaking to and handing the child objects from
the affected side is another helpful exercise.
If manual stretching is begun early and performed consistently by parents, further treatment usually is not
necessary. If extreme injury to the muscle occurred, torticollis can lead to the continued elevation of one
shoulder. Although a rare complication, this has the potential to lead to scoliosis later in life. Therefore, help
parents to understand that their actions are important therapy, not just games. Otherwise, the exercises seem so
simple parents may not take them seriously. In the few instances in which simple exercises are not effective and
the condition still exists at 1 year of age, surgical correction followed by a neck immobilizer may be necessary.
CRANIOSYNOSTOSIS
Craniosynostosis is the premature closure of the sutures of the skull. This may occur in utero or early in
infancy because of rickets or irregularities of calcium or phosphate metabolism; it also occurs as a dominantly
inherited trait and occurs more often in boys than in girls (Children’s Craniofacial Association, n.d.). Measuring
the infant’s head circumference during the first 18 months of life is advocated by the American Academy of
Pediatrics (AAP; Mulpuri, Song, Gross, et al., 2015).
This condition needs to be detected early because premature closure of the suture line will close the
fontanelles, seal the skull closed, and compromise brain growth. If the sagittal suture line is the one that closes
prematurely, the child’s head tends to grow anteriorly and posteriorly. If the coronal suture line fuses early, the
orbits of the eyes become misshapen and the increased intracranial pressure may lead to eye disorders such as
exophthalmos, nystagmus, papilledema, strabismus, and atrophy of the optic nerve with consequent loss of
vision. Premature closure of the coronal suture line is associated with syndactyly. Therefore, make a point at
well-child assessments to observe the head circumference for all infants, especially those with syndactyly.
Cardiac anomalies, choanal atresias, or disorders of elbows and knee joints can also be associated.
Craniosynostosis is diagnosed by X-ray or ultrasound, which reveals the fused suture line. If the suture line
involved is the sagittal, treatment may involve only careful observation; if the coronal suture line is involved, it
will need to be surgically opened to prevent brain compression and an abnormally shaped head by 9 to 12
months (Jubbal, Agrawal, & Hollier, 2017).
ACHONDROPLASIA
Achondroplasia (chondrodystrophia) is a failure of bone growth inherited as a dominant trait, which causes a
disorder in cartilage production in utero. The epiphyseal plate of long bones cannot produce adequate cartilage
for longitudinal bone growth; this results in both arms and legs becoming stunted (Ornitz & Legeai-Mallet,
2017).
Because the bones of the cranium are of membranous origin, the head continues to grow normally, causing
children’s heads to appear unusually large in contrast to their extremities. The forehead is particularly
prominent and the bridge of the nose becomes flattened. Children’s trunks are of near-normal size, but a
thoracic kyphosis (outward curve) and lumbar lordosis (inward curve) of the spine may develop. Because this is
a cartilage, not a brain growth concern, gross motor development may be slowed, but intelligence is not
affected.
Achondroplasia can be diagnosed in utero by ultrasound or at birth by X-ray by comparing the length of
extremities to the usual length (in the average child, the arms can be extended to the distance of the midthigh).
An X-ray will also reveal characteristic abnormally flaring epiphyseal lines. Children with achondroplasia
rarely reach a height of more than 4 ft 6 in. (140 cm). Women with this condition may have difficulty with
childbearing because of a small pelvis, generally necessitating a cesarean birth.
Children with achondroplasia become aware of their appearance as early as the preschool years. They are
apt to become acutely aware of their appearance during school age, when they realize they look so different
from other children. In order to help them grow, they may be prescribed growth hormone, or although
controversial, leg lengthening may be possible (Ornitz & Legeai-Mallet, 2017). Ideally, such children have
parents who have helped them adjust well to their short stature as well as help them develop good self-esteem
so they can be happy in their body, no matter what is their final height.
Children need to be informed as they reach adolescence that, as with all dominantly inherited disorders,
there is a high probability their children will inherit the disorder. This can make adolescence a particularly
difficult time for these children as they realize both some occupational and reproductive options may be limited
for them. Continued guidance or counseling can help them to emerge from this period with feelings of high selfesteem as adults.
TALIPES DISORDERS
The word talipes is formed from the Latin talus (“ankle”) and pes (“foot”). The talipes deformities, therefore,
are ankle–foot disorders, popularly called clubfoot. The term “clubfoot” implies permanent crippling to many
people, and because this is no longer true with effective surgery, avoid using the term when discussing talipes
disorders with parents. Concerns that may remain after surgery include that the child’s right and left shoe size
may vary and the child may have asymmetry of leg length.
Approximately 1 child in every 1,000 is born with a talipes disorder, and it occurs more often in boys than
in girls. It probably is inherited as a polygenic pattern, and it usually occurs as a unilateral problem (Sanzarello,
Nanni, & Faldini, 2017).
Some newborns who appear to have a talipes disorder actually have only an unusual foot position (a
pseudotalipes) that developed because of their cramped intrauterine position. In these infants, the foot can be
brought into a straight position by manual manipulation. In a true disorder, the foot cannot be properly aligned
without further intervention. Be certain to demonstrate to parents that, if a pseudodisorder is present, the foot
can easily be brought into line or is not deformed. Otherwise, the first time parents fit booties or shoes on the
infant, they may notice this odd position and worry the foot is misshapen when it is not. Stretching the foot into
line every day will solve the problem in a short time.
A true talipes disorder can be one of four separate types: plantar flexion (an equinus or “horse foot”
position, with the forefoot lower than the heel); dorsiflexion (the heel is held lower than the forefoot or the
anterior foot is flexed toward the anterior leg); varus deviation (the foot turns in); or valgus deviation (the foot
turns out). Most children with talipes deformities have a combination of these conditions or have an
equinovarus (Fig. 27.3A) or a calcaneovalgus disorder (a child walks on the heel with the foot everted).
Assessment
The earlier a true disorder is recognized, the better will be the correction. Make a habit, therefore, of
straightening all newborn feet to the midline as part of the initial assessment to detect this disorder. If there is a
possible questionable deformity, refer to the pediatric physician and orthopedist specialist to begin the process
of evaluating the infant properly.
Therapeutic Management
Correction is achieved best if it is begun in the newborn period. For correction, a series of casts or braces are
applied to gradually mold the foot into good alignment (a Ponseti method) (Sanzarello et al., 2017). Although
the disorder involves the ankle, the cast or brace extends above the knee to ensure a firm correction (see Fig.
27.3B). (Care of the child in a cast is discussed in Chapter 51.) Because talipes casts are high on the leg, change
diapers frequently to prevent a wet diaper from touching the cast and causing it to become soaked with urine or
meconium. Review with parents how to check the infant’s toes for coldness or blueness and how to blanch a
toenail bed and watch it turn pink to assess for good circulation. Because a newborn cannot report pain except
by generalized crying, they must evaluate crying episodes in the infant carefully. Such crying may be because
of colic, hunger, or wet diapers, or it might also be because of the tingling feeling of circulatory compression (as
when a foot is “asleep”) from too tight a cast.
Infants grow so rapidly in the neonatal period that casts or braces for talipes deformities must be changed or
adjusted almost every 1 or 2 weeks. If a mother has a complication or is exhausted from childbirth, be certain
she will be able to make arrangements for another family member or friend to bring the infant to the hospital for
frequent cast changes or brace adjustments.
After approximately 6 weeks (the time varies depending on the extent of the problem), the final cast will be
removed. Following this, the infant may have to sleep in Denis Browne splints (shoes attached to a metal bar to
maintain position) or high-top shoes at night for a few more months to ensure an effective correction. Parents
may need to perform passive foot exercises such as putting the infant’s foot and ankle through a full range of
motion several times a day for several months. These seem to be simple maneuvers, so be certain to stress their
importance to the parents; otherwise, they are easy exercises to omit when people’s lives are busy. Pediatric
physical therapists are often part of the interprofessional therapy team to assist in rehabilitation at the hospital
and at home.
Although a successful correction cannot be guaranteed, the prognosis for a full correction is good. For
children who do not achieve correction by casting, additional surgery is yet another option to achieve a final
correction.
DEVELOPMENTAL DYSPLASIA OF THE HIP
Developmental dysplasia of the hip (DDH) (often referred to as congenital hip dysplasia) is improper
formation and function of the hip socket and is considered a spectrum of abnormalities affecting the hip joint.
DDH is a fairly common musculoskeletal condition found in newborns, with the prevalence estimated to range
from 1.6 to 28.5 per 1,000 infants, averaging about 5% of newborns who have some radiographic abnormality
of the hip (Shaw & Segal, 2016).
DDH is a leading cause of orthopedic disability in childhood and adult life because it can lead to premature
arthritis requiring hip replacement (the disorder is responsible for up to 28% of hip replacements in people
under 60 years of age) (Shaw & Segal, 2016). The disorder may be evident as either subluxation or dislocation
of the head of the femur (Fig. 27.4).
With the disorder, the acetabulum of the pelvis is unusually flattened or shallow. This prevents the head of
the femur from remaining in the acetabulum and rotating adequately. In a subluxated hip, the femur “rides up”
because of the flat acetabulum; in a dislocated hip, the femur rides so far up it actually leaves the acetabulum.
Why the disorder occurs is unknown, but it may be from a polygenic inheritance pattern. It may also occur from
a uterine position that causes less-than-usual pressure of the femur head on the acetabulum.
DDH is more likely to occur with breech birth, a female infant, and a mother’s first pregnancy (Jackson,
Runge, & Nye, 2014). It is usually unilateral and found 6 times more frequently in girls than in boys, possibly
because the hips are normally more flaring in females and possibly because the maternal hormone relaxin
causes the pelvic ligaments to be more relaxed during pregnancy, which causes the femur to not press as
effectively into the acetabulum during intrauterine life, thus deepening the space. Additional risk factors include
family history of DDH, oligohydramnios, large birth weight for gestational age, metatarsus adductus, and
torticollis (Shaw & Segal, 2016). Current literature suggests that the natural history to delineate the reason for
the DDH appears to be dependent both on the type and severity of the hip abnormality and may be the
combination of risk factors presented (Mulpuri et al., 2015).
Assessment
All infants should be screened for DDH from birth and up until 3 months of age by performing the Ortolani &
Barlow maneuver (Shaw & Segal, 2016). Detecting developmental dysplasia of the hip in the newborn is
important because the longer the condition goes undetected, the more difficult it is to correct. On inspection, the
affected leg may appear slightly shorter than the other because the femur head rides so high in the socket. This
is most noticeable when the child is lying supine and the thighs are flexed to a 90-degree angle toward the
abdomen, causing one knee to be lower than the other (a Galeazzi sign; Fig. 27.5A). An unequal number of skin
folds may also be present on the posterior thighs (see Fig. 27.5B). This finding is unreliable, however, because
some infants with normal hips have an uneven number of posterior thigh skin folds. Subluxated or dislocated
hips are best assessed by noting whether the hips abduct (Box 27.3).
BOX 27.3
Nursing Care Planning Using Procedures
ASSESSING ORTOLANI AND BARLOW SIGNS
Purpose: To assist in detecting developmental dysplasia of the hip
Procedure
Principle
In
some
1. Lay the infant supine and flex the knees to 1. Proper positioning helps ensure accurate
infants, the
90 degrees at the hips.
results.
hip abducts
2. Place your middle fingers over the greater 2. Placing your fingers in this way allows for
properly at
trochanter of the femur and your thumb
abduction of the hips.
a newborn
on the internal side of the thigh over the
assessment
lesser trochanter (Fig. A).
, but at the
3. Abduct the hips while applying upward
3. Normally, no sound is heard. A clicking or
time of a
pressure over the greater trochanter, and
clunking sound is a positive Ortolani sign as it
health
listen for a clicking sound.
occurs when a displaced femoral head
maintenanc
reenters the acetabulum.
e visit at 4
to 6 weeks,
4. Next, with your fingers in the same
4. Normally, the hip joint is stable. A feeling of
a
position and holding the hips and knees at
the femur head slipping out of the socket
secondary
90-degree flexion, apply a backward
posterolaterally is a positive Barlow sign,
shortening
pressure (down and laterally) and adduct
which is indicative of hip instability
of the
the hips. Note any feeling of the femoral
associated with the developmental dysplasia
adductor
head slipping (Fig. B).
of the hip.
muscles
will have occurred, and the disorder will be first evident. Hip dysplasia may also be difficult to detect at birth in
an infant who was born from a breech presentation because the knees tend to be stiff and not flex readily.
Because tight adductor muscles occur in children with cerebral palsy, these children also need careful
assessment. Current guidelines recommend physical exam screening of all infants up to 6 to 12 months of age at
all primary care visits via hip assessment and continual screening of those with risk factors until walking age
(Mulpuri et al., 2015). In regard to radiologic screening, current guidelines recommend performing an imaging
study, such as a hip ultrasound, before 6 months of age only in infants with one or more of these risk factors:
such as breech presentation, family history, or history of clinical hip instability (Mulpuri et al., 2015). Hip
ultrasonography has a good negative predictive value (as high as 90%) for ruling out DDH (Shaw & Segal,
2016). Generally plain radiography (X-ray) is not as effective in children less than 6 months of age due to
musculoskeletal immaturity and unreliability to accurately screen for DDH; in children older than 6 months,
radiology may be a useful assessment evaluation tool of treatment progress.
Therapeutic Management
Current recommendations from American Academy of Orthopaedic Surgeons and the AAP (2017) suggest
close monitoring in mild DDH cases because 60% to 80% of clinically identified abnormalities and 90% of
ultrasonographic abnormalities spontaneously resolve without treatment in early infancy (Mulpuri et al., 2015).
In contrast, severe DDH may adversely affect normal hip growth and development and may cause issues into
adulthood, such as osteoarthritis of the hip (Shaw & Segal, 2016). Infants less than 6 months of age are usually
treated by using flexion-abduction splinting devices. Correction of subluxated and dislocated hips involves
positioning the hip into a flexed, abducted (externally rotated) position to press the femur head against the
acetabulum and cause the acetabulum to deepen its contour from the pressure. Brace and splints, such as the von
Rosen, Pavlik, Craig, or Frejka, may be utilized for treatment of an unstable hip, and patient–family preferences
should have a substantial influence on which type is chosen (Mulpuri et al., 2015). The Pavlik harness is shown
to have a high success rate in treating subluxation and reducible DDH. There will be a small number of children
who do not achieve correction by noninvasive methods will have corrective and therapeutic hip surgery, which
may involve having a pin inserted to stabilize the hip.
Nursing Diagnoses and Related Interventions
Nursing Diagnosis: Deficient parental knowledge related to splint, halter, or cast correction for hip dysplasia
Outcome Evaluation: Parents verbalize correct technique for and correctly demonstrate application and
removal of splint or halter device and care of device or cast.
A Pavlik Harness. A Pavlik harness is an adjustable chest halter that abducts the legs. It is the method of choice
for therapy because it reduces the time interval for therapy to 3 to 4 weeks and simplifies care (Fig. 27.6A).
Soft plastic stirrups (booties) with quick-fastening closures attach to leg extension straps and hold the hips
flexed, abducted, and externally rotated. Instruct parents how to lay the infant supine, grasp the infant’s
thighs, and abduct them to place the femoral head into the acetabulum and then apply the harness. The
harness is then worn under clothing continually except for bathing. Advise parents to assess the skin under the
straps daily for irritation or redness or potential skin breakdown from the harness rubbing. Caution them also
that the harness achieves its effect by gentle continual pressure; it will be ineffective if parents remove it
frequently or forget to put it in place.
A Spica Cast. If a hip is fully dislocated or the subluxation is severe, an infant may be placed immediately in a
frog-leg, A-line cast, or a spica cast to maintain an externally rotated hip position (see Fig. 27.6B). These casts
are heavy and are so wide that dressing infants or sitting them in an infant car seat or using a bassinet can be
difficult. Be certain parents have a car seat that can be modified to accommodate a large cast. Like babies with
a talipes, these infants are unable to report a cast is causing circulatory constriction, so they need to be
assessed hourly for circulation to the extremities for the first 24 hours the cast is in place and daily thereafter.
Teach parents how to do this type of neurovascular assessment (check temperature and circulation in toes)
before they take an infant home from the hospital so they can prevent circulatory compression from a rapidly
growing leg outgrowing the cast. Casts will be changed as growth and casting therapy require but maintained
for 6 to 9 months. Casting is a second line of correction because the child is much harder to care for because
of the weight of the cast. In some cases, the joint may become compressed due to the reduction maneuver or
tension in the soft tissues around the hip, blocking the blood supply to the femoral head (avascular necrosis).
In its severest form, this can lead to femoral head death and loss of future growth at the proximal growth
plate, causing unequal leg lengths.
General Care Guidelines. Rarely, but sometimes surgery may still be necessary for a final correction. With
severe hip involvement, some children will be 2 years old before the final cast is removed.
The child and parents will be visiting their orthopedist frequently during these early years. Assess that they
also schedule general health maintenance visits for routine immunizations and overall growth and
development assessment. Spend time during health maintenance visits talking with them about infant
stimulation. Teach parents to hold their child for feeding and to rock and cuddle the infant, even though a
large cast or a brace may be bulky and awkward. Discuss how to bring experiences to the infant because the
child cannot crawl and walk toward interesting objects in the environment. A child’s wagon can supply
convenient and fun transportation. The child may also be able to lie prone and move about on a large
skateboard. Many parents worry the child who is still in a large cast at the normal age for walking (12 months)
will never learn to walk. They can be assured that this is not a problem; when the cast is removed, the child
will quickly catch up with this developmental step. Children who are diagnosed with severe DDH will often
have an adjunct therapy team, such as pediatric physical therapists who are assisting with gross-motor
milestone development while the child is in a brace or recovering from surgery. Nurses must assess that the
family is receiving any needed adjunct therapies, such as physical therapy, and assist with any referrals that
are necessary.
Physical and Developmental Disorders of the Gastrointestinal System
Many of the most common congenital anomalies of the gastrointestinal system, such as cleft lip and cleft
palate, occur because of midline closure failure extremely early in intrauterine life. Others occur because the
tract first forms as a solid tube and then undergoes canalization (hollowing out). At any site where this
hollowing out does not occur, a partial or complete blockage or obstruction will be present. All gastrointestinal
disorders can interfere with an infant’s ability to take in nourishment to some degree at birth.
ANKYLOGLOSSIA (TONGUE-TIE)
Ankyloglossia is an abnormal restriction of the tongue occurring in a small number of newborns caused by an
abnormally tight frenulum, the membrane attached to the lower anterior tip of the tongue (Yoon, Zaghi,
Weitzman, et al., 2017). Tongue-tie (ankyloglossia) is a congenital anomaly in which a tight or shortened
lingual frenulum causes restricted tongue mobility and impaired tongue function (Hazelbaker, 2010).
Incidence rate is estimated to range from 4% to 10% within the newborn population (Yoon et al., 2017).
Normally, the frenulum appears short and is positioned near the tip of the tongue. As the anterior portion of
the infant’s tongue grows, the frenulum becomes located farther back. In most instances, therefore, an infant
suspected of being tongue-tied has a normal tongue at birth; it just seems short to parents who are unaware
of a newborn’s appearance. Tongue tie is often evaluated based on the mobility and how close to the tip of
the tongue the leading edge of the frenulum is attached. Types 1 and 2 are the most common, accounting for
75% of tongue-ties and are often called “classic” and “anterior ties.” Types 3 and 4 account for remaining 25 %
and are often referred as “posterior ties.”
The Hazelbaker Assessment tool can also be used to evaluate an infant’s tongue-tie. The grading levels range
from Class I (least severe) to Class IV (most severe) and are based on the tongue’s appearance and function
(Hazelbaker, 2010). With the increased emphasis on initiating and maintaining breastfeeding, a resurgence of
feeding difficulties due to tongue-tie has been identified. In addition, decreased tongue mobility may cause
short- and long-term consequences including feeding, speech, orthodontic problems, mandibular
abnormalities, and difficulty with oral cleanliness (Academy of Breastfeeding Medicine [ABM], 2017).
This condition may cause difficulty with breastfeeding due to difficulty with tongue mobility and the ability to
latch to create a strong suction at the breast. Mother’s will often note that their infants (with a tongue-tie)
tend to slip off the breast and cannot maintain a long latch without falling asleep. In addition, this mislatch will
often cause moderate to severe nipple pain in about 80% of breastfeeding mothers. If it does, then a
frenotomy release can be performed in the newborn period, typically by a trained physician, pediatric dentist,
or advanced practice registered nurse (APRN). Ankyloglossia or tongue-tie are associated with a 25% to 60%
incidence of breastfeeding difficulty and contributes to 10% to 26% of early cessation of breastfeeding (Wong,
Patel, Cohen, et al., 2017). Current literature supports frenotomy for treatment of clinically significant tonguetie that is causing difficulty with breastfeeding. Early identification of tongue-tie allows for close evaluation of
breastfeeding and possible difficulties that might arise and referral to a specialist for a frenotomy if indicated.
The majority of infants with tongue-tie have the ability to breastfeed successfully but may require extra
lactation support, guidance, and feeding assistance by additional specialists, such as a lactation consultant
(ABM, 2017).
THYROGLOSSAL CYSTS
A thyroglossal cyst arises from an embryogenic fault that leaves a cyst formed at the base of the tongue, which
then drains through a fistula (an abnormal or surgically made passage between a hollow or tubular organ and
the body surface, or between two hollow or tubular organs) to the anterior surface of the neck (Gowda &
Joseph, 2017). Thyroglossal cysts are congenital defects located in or around the midline of the neck,
extending to the base of the tongue. This condition may occur as an autosomal dominant trait. The cyst may
involve the hyoid bone (the bone at the anterior surface of the neck at the root of the tongue) and may
contain aberrant thyroid gland tissue. As the cyst fills with fluid, swelling and obstruction can lead to
respiratory difficulty from pressure on the trachea. If infected, the cyst often appears swollen and reddened,
with drainage of mucus or pus from the anterior neck and requires antibiotic therapy.
The cyst is surgically removed to avoid future infection of the space and, if thyroid tissue is present, the
possibility of developing thyroid carcinoma later in life. Observe infants closely in the immediate postoperative
period for respiratory distress because the operative area will develop at least minimum edema from surgical
trauma. Position infants on their sides so secretions drain freely from their mouths. Intravenous (IV) fluid
therapy is given after surgery until the edema at the incision recedes somewhat and swallowing is safe once
more (approximately 24 hours). If the mother is breastfeeding, encourage her to express her milk via a
hospital-grade pump or manual expression during this time to preserve her milk supply. Observe infants
closely the first time they take fluid orally to be certain they do not aspirate. Be certain parents have a chance
to feed their infant before the infant is discharged from the surgical unit so they can be assured the infant is
swallowing safely, thus allowing them to feel confident at feeding the infant at home in a relaxed and
comfortable way.
OROFACIAL CLEFTS: CLEFT LIP AND CLEFT PALATE
The maxillary and median nasal processes normally fuse between weeks 5 and 8 of intrauterine life. In infants
with cleft lip, the fusion fails to occur in varying degrees, causing this disorder to range from a small notch in
the upper lip to total separation of the lip and facial structures up into the floor of the nose, with even the
upper teeth and gingiva absent. The deviation may be unilateral or bilateral. The infant’s nose generally
appears flattened because the incomplete fusion of the upper lip has allowed it to expand in a horizontal
dimension (Allori, Mulliken, Meara, et al., 2017) (Fig. 27.7A). Overall rates of cleft lip and/or palate in the
United States average to 1 in 600 newborns (American Cleft Palate–Craniofacial Association, n.d.).
Cleft lip is the most common orofacial cleft. It is more prevalent among boys than girls and occurs at a rate of
approximately 20 cases per day and 7,500 cases each year in the United States (Tolarova & Elluru, 2015). This
incidence is highest in the Asian population at 1 out of every 500 people, moderate in the European-derived
population at 1 out of 1,000 people, and significantly lower in the African-derived population at 1 out of 2,500
people. It appears to be caused by the transmission of multiple genes aided by teratogenic factors present
during weeks 5 to 8 of intrauterine life, such as a viral infection, certain seizure medicines such as phenytoin,
maternal smoking or binge drinking, hyperthermia, stress, and maternal obesity (Funato & Nakamura, 2017).
Folic acid deficiency may also be associated with incomplete anterior midline closures (Funato & Nakamura,
2017).
Because of the genetic influence, parents of a child with a cleft lip should be referred for genetic counseling to
ensure they understand they have a small increased chance of having another child with a cleft lip or palate
and that any future children are at a greater risk than usual for this problem.
A cleft palate is an opening of the palate and occurs when the palatal process does not close as usual at
approximately weeks 9 to 12 of intrauterine life. The incomplete closure is usually on the midline and may
involve the anterior hard palate, the posterior soft palate, or both (see Fig. 27.7B). It may occur as a separate
anomaly or in conjunction with a cleft lip. As a single entity, in contrast to cleft lip, it tends to occur more
frequently in girls than boys. Like cleft lip, it appears to be the result of polygenic inheritance or environmental
influences. In connection with cleft lip, the incidence is approximately 1 out of every 1,000 births. As a single
entity, it occurs in approximately 1 out of every 2,000 births. Almost 30% of children with both cleft lip and
palate have associated birth defects, or the cleft palate occurs as only a portion of a larger syndrome (Dixon,
Marazita, Beaty, et al., 2011).
Assessment
Cleft lip may be detected by a sonogram while an infant is in utero. If not detected then, it is readily apparent
on inspection of the mouth at birth. When assessing newborns, be sure you have good lighting so you can
visualize the palate clearly. Because cleft palate is a component of many syndromes, assess the child for other
congenital anomalies as well.
Therapeutic Management
If a cleft lip is discovered while the infant is still in utero, fetal surgery can repair the condition, although this
procedure is not usually attempted. If the disorder is discovered at birth, a cleft lip can be repaired surgically
shortly thereafter, often at the time of the initial hospital stay or between 2 and 12 weeks of age. Because the
deviation of the lip interferes with sucking, infants may be a better surgical risk as newborns than they are
after a month or more of poor nourishment. Early repair also helps infants experience the pleasure of sucking
as soon as possible. It is equally important from a psychological standpoint as a parent may need caring
support to bond with an infant whose face is deformed in this way (Nidey, Moreno Uribe, Marazita, et al.,
2016). Because facial contours change as a child grows, a revision of the original repair or a nasal rhinoplasty
to straighten a deviated nasal septum may be necessary when the child reaches 4 to 6 years of age. Some
infants may have a nasal mold apparatus applied before surgery to shape a better nostril (Funato & Nakamura,
2017).
The optimal time for repair of a cleft palate is controversial because early repair increases speech
development but may result in a necessary second-stage repair as the child’s palate arch expands with growth
(Taub & Piccolo, 2016). Surgery may be recommended as a two-stage palate repair, with soft palate repair at 3
to 6 months of age and hard palate repair at 6 to 18 months of age, called the Malek protocol. This type of
repair results in less need for future surgery and better facial results (Taub & Piccolo, 2016). Using infant
orthodontic devices and delaying hard palate closure until later has not been shown to increase speech clarity
or overall wellness (Taub & Piccolo, 2016).
Currently, the results of surgical repair of cleft lip and cleft palate are excellent (Fig. 27.8). It is helpful to show
parents photographs of babies with good repairs to assure them their child’s outcome can also be as
successful.
One issue that may remain is that because palate repair narrows the upper dental arch, a child may be left
with less space in the upper jaw for the eruption of teeth, creating poor teeth alignment. All children born
with a cleft palate, therefore, need follow-up treatment by a pediatric dentist skilled in children’s dental
problems, so that as the child grows, extractions or realignment of teeth can be done as indicated. Children
also need follow-up to detect if hearing or speech difficulty occurs; because the angle of the eustachian tube
may be changed in surgery, a child may develop more ear infections than usual, possibly leading to some
hearing impairment. After surgical repair, about 80% of children affected by cleft palate progress to develop
normal speech, yet referral to speech therapy early in infancy should always occur to ensure successful speech
development (American Cleft Palate–Craniofacial Association, n.d.).
Children with cleft problems tend to receive better, more frequent, and well-coordinated care when seen in
an interprofessional team setting including pediatric dentists, audiologists, speech pathologists, geneticists,
and craniofacial surgeons, so referring parents to an appropriate interprofessional center before discharge is
critical for these infants and their families (Taub & Piccolo, 2016).
Nursing Diagnoses and Related Interventions
Nursing Diagnosis: Risk for imbalanced nutrition, less than body requirements, related to feeding problem
caused by cleft lip or palate
Outcome Evaluation: Newborn ingests an adequate diet of 110 kcal/kg (50 kcal/lb) in 24 hours; weight is
maintained within 10% of birth weight.
Preoperative Period. Before a cleft lip or palate is repaired, feeding the infant becomes a concern because the
infant has difficulty maintaining suction with a bottle or breast; there is evidence of slower growth when
compared to infants without a cleft disorder (Taub & Piccolo, 2016). In addition, it is important the child does
not aspirate.
It may be possible for an infant with a cleft lip to breastfeed because the bulk of the mother’s breast tends to
form a seal against the incomplete upper lip. Although the baby needs the enjoyment of sucking, some
surgeons do not want a baby to either breastfeed or suck on a nipple, however, before surgical correction of
the disorder to avoid any local bruising of tissue. Therefore, the best feeding method for the child with cleft lip
may be to support the baby in an upright position and feed the infant gently using a soft bottle and a
commercial cleft lip nipple or a spoon. Breck or Haberman feeders are commercial apparatuses similar to a
bulb syringe, which also can be used (Fig. 27.9). If the surgical repair for a cleft lip will be done immediately,
the mother will be able to breastfeed as early as 7 to 10 days after surgery. Typically after the surgical repair
and healing, the baby can latch to the breast. If there is difficulty with latching, a nipple shield can be
suggested to help form a tighter seal at the breast. Review with the mother how to pump or manually express
breast milk to maintain a milk supply prior to surgical correction and after, if needed. If surgery will be delayed
for 1 month, the mother will need continuing support from the nursing staff to support her efforts on pumping
and to remind her that her breast milk will be very beneficial to her infant and the healing process.
If a cleft extends to the nares so the nose and mouth are joined, breathing causes the oral mucous
membranes and lips to become dry. Offering small sips of fluid between feedings can help keep the mucous
membranes moist and prevent cracks and fissures that could lead to infection. Following a feeding, be certain
an infant with a cleft lip is burped well because the inability to grasp a nipple or syringe edge securely causes
the infant to swallow more air than usual.
Infants with a cleft palate cannot suck effectively either because pressing their tongue or a nipple against the
roof of their mouth forces milk up into their pharynx, possibly leading to aspiration. The most successful
method for feeding this infant, like the child with a cleft lip, therefore, is to use a commercial cleft palate
nipple that has an extra flange of rubber to close the roof of the mouth. If the nipple is used with a plastic
bottle that can be squeezed gently to increase the flow of the feeding, this can compensate for poor sucking. A
Breck feeder may also be used to feed infants with a cleft palate.
If surgery will be delayed beyond 6 months of age or the time when solid food would usually be introduced,
teach parents to be certain any food they offer is soft because particles of coarse food could invade the
nasopharynx and be a cause of aspiration.
Postoperative Period. After surgery for cleft lip or palate, an infant is kept nothing by mouth (NPO) for
approximately 4 hours and then introduced to liquids such as plain water. Be certain to begin this process with
only a small amount each time to prevent vomiting.
It’s important that no tension is placed on a lip suture line because this helps keep the sutures from pulling
apart and leaving a large scar. During this immediate postoperative period, therefore, the infant is usually fed
using a specialized feeder because this causes less suture line tension than bottle feeding or breastfeeding.
After palate surgery, only liquids are generally given the first 3 or 4 days, and then a soft diet is followed until
healing is complete. Ask parents before surgery what fluids the child prefers so they can be made available
postsurgery.
When children begin to eat soft food, observe they don’t use a spoon because spoons can invariably be
pushed against the roof of their mouth and possibly disrupt sutures. If being fed rather than allowing the
infant to use a spoon evokes an intense reaction, it is probably better to leave a child on a liquid diet until the
sutures are removed. Be certain milk is not included in the first fluids offered because milk curds tend to
adhere to the suture line and so are difficult to remove. After a feeding, always offer the child a sip of clear
water to rinse the suture line and keep it as clean as possible. Also, educate parents to be diligent about oral
health care. In infants with clefts involving the maxillary alveolar ridge (upper gum), it is common for some
teeth—typically the upper incisors and cuspids on the side of the cleft—to be misshapen or turned (Cleft
Palate Foundation, 2010). Prudent twice-daily gum and teeth brushing with an age appropriate toothbrush
and tooth paste is crucial, as are biyearly dental visits for monitoring.
Nursing Diagnosis: Risk for ineffective airway clearance related to oral surgery
Outcome Evaluation: Child’s respiratory rate remains at baseline level for age without retractions or obvious
distress.
Because of the local edema that occurs after cleft lip or palate surgery, it’s important to observe children
closely in the immediate postoperative period for respiratory distress. Remember that before surgery, the
infant with a cleft lip may have breathed through the mouth. After surgery, the infant now has to learn to
breathe through the nose, possibly adding to respiratory difficulty. Generally, however, this is not a problem
because newborns normally are strict nose breathers.
Following either cleft lip or cleft palate surgery, infants may need their mouth suctioned to remove mucus,
blood, and unswallowed saliva. When doing this, be exceedingly gentle so you don’t touch the suture line with
the catheter. Place infants on their side to allow mouth secretions to drain forward. And, following a cleft lip
repair, be sure a child does not turn onto his or her abdomen because this could put pressure on the suture
line, possibly tearing it. Placing the child in an infant bouncy chair is another possibility.
Nursing Diagnosis: Impaired tissue integrity at incision line related to cleft lip or cleft palate surgery
Outcome Evaluation: Incision line appears clean and intact and free of erythema or drainage during
postoperative period.
After cleft lip surgery, the suture line may be held in close approximation by a Logan bar (a wire bow taped to
both cheeks; Fig. 27.10) or an adhesive bandage such as a Band-Aid simulating a bar that brings together the
incision line but does not cover the incision. Assess the simulated or Logan bar is secure and continues to
protect the suture line from tension after each feeding or cleaning of the suture line. Furnish adequate pain
relief also so the infant does not cry because this puts increased tension on the sutures. To help avoid crying,
try to anticipate the infant’s needs by having formula ready to feed. Help the parents use whatever measures,
such as rocking, carrying, or holding, that are necessary to make the infant feel secure and comfortable.
Nothing hard or sharp must come in contact with a recent cleft suture line. Observe infants after palate repair
carefully, therefore, to be certain they do not put toys with sharp edges into their mouths. It’s also good
practice to not allow them to use a straw to drink or hold a toothbrush to clean their teeth so they don’t brush
the suture line accidentally. Keep elbow restraints in place as necessary so they do not put their fingers in their
mouth and poke or pull at the sutures. Sutures on the lip or palate feel extremely odd, so most children not
only run their tongue over their sutures but also don’t respond to advice not to do this. Because this often
occurs when children have nothing to think about, help the parents provide diversional activities such as
reading or singing to keep the child’s attention off the suture line.
If parents will be continuing to give an analgesic such as acetaminophen (Tylenol) or ibuprofen (Advil) after
they return home, be certain they are aware of the correct dosage and time schedule for administration of the
liquid forms. They must also continue to keep the suture line protected until healing is complete.
Nursing Diagnosis: Risk for infection related to surgical incision
Outcome Evaluation: Infant’s temperature is below 98.6°F (37°C) by tympanic membrane; incision site is clean,
dry, and intact without erythema or foul drainage.
An infection and subsequent scarring may result if crusts from serous drainage are allowed to form on a cleft
lip suture line. Most surgeons, therefore, prescribe cleaning the suture line with sterile water or sterile saline
with sterile cotton-tipped applicators after every feeding or whenever the normal serum that forms on suture
lines accumulates. Do not rub the suture line; use a smooth, gentle, rolling motion to avoid loosening sutures.
Gently dry the suture line with a dry sterile cotton-tipped applicator afterward. Remember that the infant has
sutures on the inside of the lip that need the same meticulous care as those visible on the outside.
Nursing Diagnosis: Risk for impaired parenting related to birth of an infant who is physically challenged
Outcome Evaluation: Parents state they believe there will be a positive outcome for their child and
demonstrate positive coping behaviors evidenced by holding and helping with infant care.
To promote effective bonding, parents need to hold and interact with their infant during both the
preoperative and postoperative periods. Caution them the child’s incision line will appear swollen in the
immediate postoperative period, but its appearance will improve with time. As soon as the child’s sutures
have been removed, parents may breastfeed or feed with an ordinary bottle. Caution both the breastfeeding
mother (who has been maintaining her milk supply through expression) and the formula-feeding mother that
because the infant has never sucked before, time will be needed to learn how to suck, just like a newborn.
Observe whether parents look at their baby’s face while feeding the baby. Help them to understand that any
negative feelings they feel toward the child or themselves, such as sadness or anger, are normal. This
assurance does not instantly make them feel better about what has happened, but the knowledge the feelings
they are experiencing are normal can help them begin to deal with such emotions. Many communities have
support groups for parents of children born with a cleft lip or palate. Referral to these groups can offer
parents additional support. The National Cleft Palate Foundation provides parent education materials at
www.cleftline.org.
Nursing Diagnosis: Risk for situational low self-esteem related to child’s facial surgery
Outcome Evaluation: Child participates in normal childhood activities that involve contact with other people,
states activities he or she enjoys at healthcare visits, and demonstrates age-appropriate developmental
milestones.
If a scar remains after cleft lip surgery, children may need some help adjusting to their appearance until a
second cosmetic repair can be completed later in life. Reinforce children’s positive attributes, stressing that
the scar is only one small aspect of who they are. As children reach adolescence, you may need to review a
familial inheritance pattern of cleft lip so they are aware of the possible risk for transmission to their own
children.
Nursing Diagnosis: Risk for infection (ear) related to the altered angle of the eustachian tube with cleft palate
surgery
Outcome Evaluation: Outcome Evaluation: Parents state signs and symptoms of ear infection and importance
of early treatment; parents list signs of diminished hearing and appropriate agencies for support and
guidance.
Changing the contour of the palate when it is repaired also changes the slope of the eustachian tube to the
middle ear. This can lead to a high incidence of middle ear infection (otitis media) because organisms are able
to reach this area from the oral cavity more readily than usual. Review the signs of infection such as fever,
pain, pulling on an ear, or discharge from the ear with parents. Remind them of the importance of reporting
pharyngeal infection to their primary care provider promptly so it can be treated before the infection spreads
to the middle ear. Because the eustachian tube may remain partially closed in its changed position, serous
otitis media (accumulation of fluid in the middle ear) also tends to occur more frequently in these children
than in others. If this happens, myringotomy tubes may be inserted to drain middle ear fluid and to help
protect hearing (see Chapter 50). Be certain parents understand the need for routine screening for hearing
loss during childhood because this is a common early sign of serous otitis media.
Nursing Diagnosis: Risk for impaired verbal communication related to cleft palate
Outcome Evaluation: Family members voice satisfaction with child’s speech; developmental milestone of
clearly articulated two-word sentences by age 2 years is met.
Infants with a cleft palate will begin to make speech sounds at the normal time (age 2 months). This speech,
however, is usually guttural and unclear as many alphabet sounds are made by touching the tongue against
the roof of the mouth. Depending on the age of the cleft palate repair, some children will continue to have
accompanying speech difficulty because the soft palate must function for the child to pronounce “p” and “b”
sounds (Prathanee, Pumnum, Seepuaham, et al., 2016). If cleft palate surgery is going to be delayed past age 2
years (as might happen if the child has other congenital anomalies, such as heart disease), a plastic prosthesis
to cover the incomplete palate may be prescribed because this allows the child to articulate more efficiency.
Altered speech patterns in children generally do not resolve spontaneously and require intervention by a
speech pathologist prior to school age.
THE PIERRE ROBIN SEQUENCE
The Pierre Robin sequence (also called Pierre Robin syndrome) is a rare triad of micrognathia (small mandible),
cleft palate, and glossoptosis (a tongue malpositioned downward). This triad of conditions can result in severe
upper airway obstruction, which then may cause secondary respiratory distress due to upper airway
obstruction. Estimates of occurrence range from 1 in every 8,500 births to 1 in every 20,000 births; the
primary event that leads to the syndrome tends to be unknown. More than half of the children born with
Pierre Robin syndrome have associated disorders of congenital glaucoma; cataracts; cardiac disorders; or
other genetic, chromosomal, or medical concerns. They need thorough physical and genetic assessments to be
certain that none of these other associated disorders are present (Bütow, Zwahlen, Morkel, et al., 2016).
Monitor infants for possible airway obstruction (as a result of their small jaw, their tongue tends to drop
backward and obstruct their airway). Suctioning to remove secretions may be necessary. A side-lying rather
than a supine sleeping position is recommended for all newborns and infants (until they can roll over) to avoid
obstruction. Occasionally, infants have such extensive airway obstruction that a suture is attached to the
anterior aspect of the tongue and the mucous membrane of the lower lip to pull the tongue forward and
prevent the rear of the tongue from blocking the upper airway. Maintaining positioning as the infant grows
and gains mobility may be increasingly difficult, but sleep studies have shown these children have severe sleep
apnea, with as many as 49 episodes of apnea leading to oxygen desaturation per hour if sleeping supine
(Bütow, Naidoo, Zwahlen, et al., 2016). Nasopharyngeal airways (NPAs) have been shown to be effective in
upper airway obstruction, and 86.5% of infants studied maintained adequate airways with positioning or
temporary placement of an NPA for several months. The airways of older children can be well maintained
using nasal continuous positive airway pressure (CPAP); with this, after several years of therapy, the episodes
of obstructive sleep apnea decrease to about five per hour, possibly decreasing the need for more invasive
surgery.
As the child grows older, the jaw will grow somewhat, although the mandible will always be small. Parents
need instructions on how to feed these infants until the cleft palate can be repaired. The child may have a
gastrostomy tube or button inserted to relieve feeding difficulty (see Chapter 37).
Parents of the child with this syndrome take on a great deal of responsibility when they assume their infant’s
care; it’s best if they are referred to an interprofessional team prior to discharge from the hospital. Be certain
they have the name and number of a primary healthcare provider they can call when they have questions.
Many of these parents grow exhausted during the first few weeks of their child’s life, afraid they may sleep so
soundly at night they miss their child having respiratory difficulty. Using a respiratory monitor at night can be
helpful. As parents’ confidence grows in their ability to provide care, this problem lessens, but it may be
months or even years before a high level of confidence is achieved.
ESOPHAGEAL ATRESIAS AND TRACHEOESOPHAGEAL FISTULAS
Between weeks 4 and 8 of intrauterine life, the laryngotracheal groove in the chest develops into the larynx,
trachea, and beginning lung tissue; the esophageal lumen forms parallel to this. A number of anomalies can
occur if the trachea and esophagus are affected by some teratogen that does not allow the two organs to fully
form separately and possibly become connected.
Esophageal atresia is the incomplete formation of the esophageal lumen, resulting in the proximal (upper)
esophagus forming a “blind pouch,” which then does not connect to any other structure. Tracheoesophageal
fistula occurs when an opening develops between the closed distal (lower) esophagus and the trachea. Five
usual types of esophageal atresia that result are:
1. The esophagus ends in a blind pouch; there is a tracheoesophageal fistula between the distal part of the
esophagus and the trachea (Fig. 27.11A).
2. The esophagus ends in a blind pouch; there is no connection to the trachea (see Fig. 27.11B).
3. A fistula is present between an otherwise normal esophagus and trachea (see Fig. 27.11C).
4. The esophagus ends in a blind pouch. A fistula connects the blind pouch of the proximal esophagus to the
trachea (see Fig. 27.11D).
5. There is a blind end portion of the esophagus. Fistulas are present between both widely spaced segments
of the esophagus and the trachea (see Fig. 27.11E).
Figure 27.11 An esophageal atresia and a tracheoesophageal fistula. (A) In the most common type of
esophageal atresia, the esophagus ends in a blind pouch. The trachea communicates by a fistula with the
lower esophagus and stomach (approximately 90% of infants with the defect have this type). (B) Both upper
and lower segments end in blind pouches (5% to 8% of infants with the defect have this type). (C) Both upper
and lower segments communicate with the trachea (2% to 3% of infants with the defect have this type). (D)
Very rarely, the upper segment ends in a blind pouch and communicates by a fistula to the trachea, or (E) a
fistula connects to both upper and lower segments of the esophagus.
All of these are potentially serious disorders because, during a feeding, milk can fill the blind pouch of the
upper esophagus and then overflow into the trachea, or a fistula can allow milk to enter the trachea from the
esophagus, resulting in aspiration. Even if not fed, the swallowed saliva of the baby will fill the esophagus and
overflow into the airway, resulting in repeated aspiration. The incidence of the disorder ranges from 1 out of
2,500 to 4,500 live births (Vergouwe, IJsselstijn, Wijnen, et al., 2015).
Assessment
Esophageal atresia must be ruled out in any infant born to a woman with hydramnios (excessive amniotic
fluid). This is because hydramnios occurs because, normally, a fetus swallows amniotic fluid during
intrauterine life. A fetus with esophageal atresia cannot effectively swallow, so the amount of amniotic fluid
can grow abnormally large. Many infants with esophageal atresia are born preterm because of the
accompanying hydramnios, compounding their original problem with immaturity. The infant needs to be
examined carefully for other congenital anomalies that could have occurred from the teratogenic effect at the
same week in gestation, such as vertebral, anal, cardiac, tracheoesophageal, renal, and limb anomalies
(VACTERL syndrome) (Vergouwe et al., 2015).
If not diagnosed in utero, diagnosing a child who has an esophageal atresia/tracheoesophageal fistula before
the infant is first fed is important. Otherwise, the infant will cough, become cyanotic, and have obvious
difficulty breathing as fluid is aspirated. Newborns who have so much mucus in their mouths that they appear
to be blowing bubbles should be suspected of having either disorder. Esophageal atresia can be diagnosed
with certainty if a catheter cannot be passed through the infant’s esophagus to the stomach so stomach
contents can be aspirated. If helping with this, supply a firm catheter because a soft one will curl in a blind-end
esophagus and appear to have passed. An X-ray will reveal a radiopaque catheter coiled in the blind end of the
esophagus. A flat-plate X-ray of the abdomen or an ultrasound also may reveal a stomach distended with the
air that has passed from the trachea into the esophagus and stomach, demonstrating that a fistula is present.
Either a barium swallow or a bronchial endoscopy examination can also reveal the blind-end esophagus and
fistula.
Therapeutic Management
Emergency actions are required for the infant with this group of esophageal disorders in order to prevent the
development of pneumonia, one of the major complications that is apt to occur from leakage of oral or
stomach secretions into the lungs. The best position for the infant is an upright, 60-degree angle so acid
stomach fluids stay in the stomach through the use of gravity (sitting the baby in an infant chair does this). If a
nasogastric tube is inserted into the distal end of the esophagus and connected to low intermittent suction,
oral secretions will no longer collect in the blind pouch, decreasing aspiration risks. A gastrostomy may be
inserted into the stomach (under local anesthesia) with the tube allowed to drain by gravity as another way of
keeping the stomach empty of secretions and preventing reflux into the lungs. IV fluids and parenteral
nutrition are begun to prevent dehydration and an electrolyte imbalance from lack of oral intake. IV
medications that restrict the stomach’s ability to produce acid may be prescribed, and an antibiotic may be
prescribed to help prevent an infection.
When the infant has been stabilized by this sequence of measure, surgery consists of closing the fistula and
anastomosing the esophageal segments. If the defect was only a simple fistula, surgery may be done by
thoracoscopy to minimize the time required for surgery (Vergouwe et al., 2015). If the repair will be more
complex, it may be necessary for the surgery to be completed in different stages and to use a portion of the
intestine to complete the anastomosis if the esophageal segments are too far apart to join readily. After an
anastomosis repair, observe infants closely at postoperative days 7 to 10 because this is the point when their
internal sutures will dissolve, possibly allowing a leak from the anastomosis site to occur at this time. If such a
leak occurs, fluid and air can leak out into the chest cavity, and pneumothorax (collapse of a lung) can occur.
In some infants, some stenosis or stricture at the anastomosis site remains. If this occurs, esophageal
dilatation at periodic intervals to keep the repaired esophagus fully patent may be necessary.
Gastroesophageal reflux may also occur after a repair, especially if the esophagus is left shorter than usual;
this can lead to recurrent fistula formation and irritation from the presence of stomach acid in the esophagus
(Vergouwe et al., 2015).
The ultimate prognosis for children with this disorder depends on the extent of the repair necessary, the
condition of the child at the time of surgery, and the presence or absence of other congenital anomalies. Even
with larger disorders, if surgery can be performed before pneumonia develops, the repair of the esophagus
and trachea will be effective. The mortality rate for the condition remains high, however, because of the
presence of other congenital disorders and low birth weight that often accompanies the tracheal abnormality.
ABDOMINAL WALL DEFECTS
Abdominal wall defects, with the exception of umbilical hernia, are potentially serious disorders because they
directly affect the gastrointestinal system and infant nutrition.
Umbilical Hernias
An umbilical hernia is a protrusion of a portion of the intestine through the umbilical ring, muscle, and fascia
surrounding the umbilical cord (Mirza & Ali, 2016). This creates a bulging protrusion under the skin at the
umbilicus. It is rarely noticeable at birth while the cord is still present but becomes increasingly noticeable at
healthcare visits during the first year.
Umbilical hernias occur most frequently in Black children (seen in 30% term infants), low–birth-weight infants,
and more often in girls than in boys. The structure is generally 1 to 2 cm (0.5 to 1 in.) in diameter but may be
as large as an orange when children cry or strain. The size of the protruding mass is not as important as the
size of the fascial ring through which the intestine protrudes. If this fascial ring is less than 2 cm, closure will
usually occur spontaneously after the child begins to walk to around 2 years of age, so surgical repair is often
not necessary for mild (small) cases. If the fascial ring is larger than 2 cm, ambulatory surgery repair is
generally indicated to prevent herniation and intestinal obstruction or bowel strangulation. This is usually
done at 1 to 2 years of age. If the small umbilical hernia has not closed by preschool age, a repair will often be
done when the child is 4 to 5 years old.
Some parents believe holding an umbilical hernia in place by using “belly bands” or taping a silver dollar over
the area will help reduce the hernia. These actions can actually lead to bowel strangulation and so should be
avoided.
The child returns from surgery with a dressing, which remains in place until the sutures are well healed.
Remind parents to sponge bathe the child until they return for a postoperative visit when the dressing is
removed. If the child is not yet toilet trained, caution parents to keep diapers folded well below the dressing
to prevent contaminating the suture line with stool.
Omphaloceles
An omphalocele is a protrusion (herniation) of abdominal contents through the abdominal wall at the point of
the junction of the umbilical cord and abdomen (Fig. 27.12). The herniated organs involved are usually the
intestines, but they may include the stomach and liver. Occurring about 1 out of 5,000 live births, the organs
are usually covered and contained by a thin transparent layer of amnion and chorion with the umbilical cord
protruding from the exposed sac. This condition occurs because, at approximately weeks 6 to 8 of intrauterine
life, the fetal abdominal contents, which grow faster than the fetal abdomen, are pushed out from the
abdomen into the base of the umbilical cord. At 7 to 10 weeks, when the fetal abdomen has enlarged
sufficiently, the intestine normally returns to the abdomen. An omphalocele occurs when abdominal contents
fail to return in the usual way. In 67% of cases, the occurrence is associated with other congenital disorders
such as cardiac, neurologic, genitourinary, skeletal, and chromosomal abnormalities (Tappero & Honeyfield,
2015).
Gastroschisis
Gastroschisis, a term derived from the Greek word for “stomach cleft” or “fissure,” is a condition similar to an
omphalocele, except the abdominal wall disorder is a distance from the umbilicus, usually to the right, and
abdominal organs are not contained by a membrane but rather spill freely from the abdominal wall (Jones,
Isenburg, Salemi, et al., 2016). Also, a greater amount of intestinal contents tends to herniate, increasing the
potential for volvulus and obstruction. The condition occurs because of failure of the abdominal wall to close,
usually during the fourth week of development, and its incidence is about 4 to 5 per 10,000 live births. The
disorder is similar to a neural tube defect (covered later in this chapter) because both are a failure to close at
about the same gestational time. Children with gastroschisis often have decreased bowel motility and, even
after surgical correction, may have difficulty with absorption of nutrients and passage of stool. Long-term
follow-up may be necessary to ensure that nutrition and elimination are adequate (Jones et al., 2016).
Assessment
The incidence of omphalocele remains steady, whereas the incidence of gastroschisis is steadily rising, perhaps
associated with maternal obesity (Jones et al., 2016). The wall defect with the herniated organs may be
identified by sonogram during intrauterine life (Jones et al., 2016). These may also be revealed by an elevated
maternal serum α-fetoprotein (MAFP) examination during pregnancy, which is done at the 15th week of
pregnancy; the level of MAFP will be abnormally increased if there is an open spinal or abdominal lesion. If the
result is elevated, an amniocentesis is then done to assess the level of AFP in amniotic fluid. A prenatal
sonogram is also helpful to determine the presence of both abdominal wall or spinal disorders (see Chapter 11
for further discussion of these prenatal assessments). If an omphalocele or gastroschisis is not identified
during pregnancy, their presence is obvious on inspection at birth. When an omphalocele or gastroschisis is
identified in utero, a cesarean birth may be performed to protect the exposed intestine. If this is the only
disorder identified, however, a vaginal birth may be preferred. Be certain to document the general
appearance of the defect and its size in centimeters at birth to serve as a baseline assessment. Both conditions
can vary greatly in size, from small, where the exposed intestine is the size of a ping-pong ball, to extensive,
where all of the abdominal contents are externalized.
Therapeutic Management
With both omphalocele and gastroschisis, until surgery and the bowel is effectively returned to the abdomen,
the infant will be fed by TPN to supply nutrients and keep the bowel from filling with air or stool. Most infants
with gastroschisis will have surgery within 24 hours to replace the bowel before the blood supply becomes
hampered, the intestinal membranes dry, bowel volvulus occurs, or the bowel becomes infected. It is often
difficult to replace the entire bowel with immediate surgery because the infant’s abdomen, which did not
need to grow to accommodate the abdominal contents, is smaller at birth than usual. Replacing the total
bowel into this small abdomen could result in respiratory distress from the pressure of the visceral bulk on the
diaphragm and lungs. Also, the bowel might not have room for effective peristalsis. If a gastroschisis is small, a
one-stage repair may be possible. If large, one surgical approach is the use of a prosthetic patch repair that
bridges the unformed gap on the abdomen with a synthetic material; the skin is then drawn tight and closed
over the patch. A second approach is to replace only a portion of the bowel at one time. The remainder is
contained by a Silastic pouch termed a “silo” that is suspended over the infant’s bed. Over the next 5 to 7
days, the bowel is gradually returned to the abdomen by multiple surgical procedures (O’Connell, DottersKatz, Kuller, et al., 2016).
For an omphalocele, if the sac is ruptured, the defect is treated like gastroschisis because of the potential for
infection. If unruptured, an external dressing producing mild pressure may be used over the intact membrane.
This gradually compresses the abdominal contents, allows the skin to stretch between treatments, and does
not appear to be a painful procedure.
Intestinal Obstructions
If canalization of the intestine does not occur in utero at any point, an atresia (complete closure) or stenosis
(narrowing) of the fetal bowel can develop, although the most common site is the duodenum.
Obstruction may also occur because the mesentery of the bowel twisted as the bowel reentered the abdomen
after being contained in the base of the umbilical cord early in intrauterine life or from looseness of the
intestine in the abdomen after it was returned (Juang & Snyder, 2012). This twisting pattern is termed a
volvulus and continues to be a potential problem for the first 6 months of life until the infant develops firmer
intestinal supports. Yet another reason obstruction can occur is because of thicker-than-usual meconium
formation, blocking the lumen (meconium plug or meconium ileus).
Assessment
Intestinal obstruction may be anticipated if the mother had hydramnios during pregnancy (i.e., swallowed
amniotic fluid could not be absorbed effectively by the fetus) or if more than 30 ml of stomach contents can
be aspirated from the newborn stomach by catheter and syringe at birth (fluid is not passing freely through
the tract). If the obstruction is not revealed by either of these findings, then symptoms of intestinal
obstruction in the neonate are the same as at any other time in life: The infant passes no meconium or may
pass one stool (meconium that formed below the obstruction) and then not pass any more; the abdomen
becomes distended and tender. As the effect of the obstruction progresses, the infant will vomit. Remember,
many neonates spit up feedings when burped. This rapid ejection of milk smells barely sour. True vomiting
from intestinal obstruction is usually sour smelling (stomach acid has acted on it), includes bile, and occurs
spontaneously without coughing or back patting. Vomitus may also be black from the color of meconium.
Bowel sounds will begin to increase in number as the bowel increases peristaltic action in an effort to push
stool past the point of obstruction. Waves of peristalsis may be observable across the abdomen. The infant
may reveal that abdominal pain is developing by crying—hard, forceful, indignant crying—and by pulling the
legs up against the abdomen. Lastly, the child’s respiratory rate will increase as the intestine fills, the
diaphragm is pushed up harder and harder against the lungs, and lung capacity decreases. An abdominal Xray, sonogram, MRI, or barium enema will reveal no air below the level of obstruction in the intestine or
isolate the level of the obstruction.
Therapeutic Management
As soon as a bowel obstruction is confirmed, an orogastric or nasogastric tube is inserted and then attached to
low suction or left open to the air to prevent further gastrointestinal distention (see Chapter 37). Always use
low intermittent suction with decompression tubes in neonates because pressure greater than this can irritate
and ulcerate their sensitive stomach lining.
IV therapy is begun to restore fluid and electrolyte balance; immediate surgery is scheduled to relieve the
obstruction before pressure on the bowel causes death of the involved intestinal lining (Sundaram,
Hoffenberg, Kramer, et al., 2012).
Repair of the obstruction (with the exception of meconium plug syndrome) can usually be accomplished by
laparoscopy, although full abdominal surgery may be necessary. The area of stenosis or atresia is removed,
and the bowel is anastomosed. If the repair is anatomically difficult or the infant has other anomalies that
interfere with overall health, a temporary colostomy may have to be constructed, and the infant discharged to
home care with follow-up surgery rescheduled at 3 to 6 months of age (see Chapter 37 for care of a child with
a colostomy). If a large portion of the bowel has to be removed, this can have an impact on nutrient
absorption (called short bowel syndrome) as the child grows older.
Meconium Plug Syndrome
A meconium plug is an extremely hard portion of meconium that has completely blocked the intestinal lumen,
causing bowel obstruction. The cause is unknown but probably reflects normal variations of meconium
consistency. Meconium plugs usually form in the lower end of the bowel because this meconium formed early
in intrauterine life and has the best chance to become dry and obstructive. The condition is associated with
Hirschsprung disease, cystic fibrosis, hypothyroidism, and magnesium sulfate administration to halt preterm
labor (Cuenca, Ali, Kays, et al., 2012).
Assessment
Because the obstruction is low in the intestinal tract, signs of obstruction such as abdominal distention and
vomiting may not occur for at least 24 hours. Typically, the infant will be identified first as an infant who has
had no meconium passage in the first 24 hours postbirth. A gentle rectal examination may reveal the presence
of hardened stool, although the plug may be too high up in the bowel to be palpated. An X-ray or sonogram
may reveal distended air-filled loops of bowel up to the point of obstruction. A barium enema not only reveals
the level of obstruction but also may be therapeutic in loosening the plug.
Therapeutic Management
A subset of patients experiences spontaneous resolution of the meconium plug without any treatment. Of the
70% of patients requiring treatment, contrast or saline barium enema was used, with 97% success (Cuenca et
al., 2012). The administration of a small (about 5 ml) saline enema (never use tap water in newborns, infants,
and young children because it can lead to water intoxication) may cause enough peristalsis to expel the plug.
Instillation of acetylcysteine (Mucomyst) rectally may be prescribed to soften stool. Gastrografin, a highly
osmotic radiographic substance, administered as an enema, is yet another solution. Because it is
hyperosmotic, the substance pulls fluid into the bowel, allowing the stool to soften and the plug to pass.
Assess that the infant is well hydrated before and after the procedure because an infant can become
hypovolemic from the effect of such a strong-acting medium.
Once the thickened portion of meconium has been passed, the infant should have no further difficulty and,
over the next several hours, may pass a great amount of stool. Postprocedure prognosis is very good. Observe
the infant for further passage of meconium (which should occur at least once daily) over the next 3 days,
however, to be certain additional plugs do not exist farther up in the bowel. If an infant is going to be
discharged before this time, instruct parents on the importance of observing for meconium and also about
phoning their primary care provider should the infant have no further bowel movements while at home.
Occasionally, a neonate passes a small plug of hardened meconium—hard enough it would have caused an
obstruction except it is so small—in the first 1 or 2 days of life. Be certain to record and report such a finding
because the infant will need close observation for continued defecation, the same as for the infant who
actually had an obstruction, to be certain there is not a larger and truly obstructing plug higher in the bowel.
Assess the family history of a newborn who has a meconium plug for cystic fibrosis, a recessively inherited
disorder (see Chapter 40), aganglionic megacolon (Hirschsprung disease), or a polygenic inherited disorder
(see Chapter 45) because, if there is a family history, the infant will need observation for these disorders.
Hypothyroidism can also present with constipation or hardened stool in newborns along with signs such as a
large protruding tongue, lethargy, and subnormal body temperature. Both hypothyroidism and cystic fibrosis
screening are done along with phenylketonuria screening in newborns. Be certain this blood test is obtained in
any newborn with a meconium plug (Levy, 2010). Infants born at home, especially, may not have had this
done.
Meconium Ileus
Meconium ileus (obstruction of the intestinal lumen by hardened meconium) is a specific phenomenon that
occurs almost exclusively in infants with cystic fibrosis, resulting from the abnormal pancreatic enzyme
function seen with cystic fibrosis (see Chapter 40) and reflects extreme meconium plugging (Tappero &
Honeyfield, 2015). The usual symptoms of bowel obstruction occur: no meconium passage, abdominal
distention, and vomiting of bile-stained fluid. Meconium ileus is the only bowel obstruction that can present
with abdominal distention at birth (Tappero & Honeyfield, 2015). Unlike simple meconium plugging, the
obstruction point may be too high in the intestine for enemas to reduce it; instead, the bowel must be incised
and the hardened meconium removed by laparotomy. Meconium ileus is so strongly associated with cystic
fibrosis, the infant needs close follow-up by an interprofessional cystic fibrosis team in the following months.
Diaphragmatic Hernias
A diaphragmatic hernia is a weakness in the musculature that permits a portion of the abdominal organs, such
as the stomach or intestine, to protrude through the chest wall. This can cause a collapse of the left lung due
to cardiac displacement on the right side of the chest. It occurs in 1 to 5 per 10,000 live births, with slight
increased frequency in boys and a decreased frequency in Blacks (Oluyomi-Obi, Kuret, Puligandla, et al., 2017).
The defect is caused because, early in intrauterine life, the chest and abdominal cavity are one; at
approximately week 8 of intrauterine growth, the diaphragm forms and divides them. If the diaphragm does
not form completely, the intestines can herniate through the diaphragm opening into the chest cavity as a
diaphragmatic hernia (Fig. 27.13).
Assessment
Diaphragmatic hernia is frequently detected in utero by routine sonogram (Oluyomi-Obi et al., 2017). If not, it
is apparent at birth when the newborn has extreme difficulty establishing effective respirations. It can also be
indicated by a scaphoid (sunken) abdomen caused by the displacement of abdominal contents into the chest
(Tappero & Honeyfield, 2015). Surgeons have tried fetal surgery to correct or lessen the lung compromise
from this diagnosis, but multiple randomized trials comparing prenatal surgical intervention to postnatal
intervention show no benefit to prenatal intervention (Hedrick, 2013).
At birth, breath sounds are usually absent on the affected side of the chest cavity because at least one of the
lobes of the lungs on that side cannot expand completely (and may not have fully formed). The infant may be
cyanotic with intercostal or subcostal retractions. The abdomen generally appears sunken because it is not as
filled with intestine as usual. These infants have a potential for developing persistent pulmonary hypertension
because blood cannot perfuse readily through the unexpanded lung. This can lead to right-to-left shunting
through the foramen ovale in the heart and also causes the ductus arteriosus to remain patent. One condition,
then, has led to another until heart involvement complicates an already serious lung picture. The mechanics of
right-to-left heart shunts are further discussed in Chapter 41.
Therapeutic Management
Although surgical repair may be done as an imminent surgical procedure, it is usually delayed until
cardiorespiratory status has been stabilized as much as possible and the baby can be transported to a highacuity nursery for care. Surgery includes repair of the diaphragm and replacement of the herniated intestine
and organs back into the abdomen, possibly requiring both thoracic and abdominal incisions. If the disorder of
the diaphragm is large, an insoluble polymer (Teflon) patch may be used to reconstruct a better diaphragm
shape.
The repair can be complicated if there is not enough room in the abdomen for the intestine to be returned. In
these infants, the abdominal incision may not be closed. The intestine is covered by silicone elastomer
(Silastic) and left to be closed at a later date after the abdomen has grown, the same as gastroschisis surgery.
If the lung that was compressed in utero is truly hypoplastic and so cannot function, it will be removed at the
time of surgery. If it is developed but just deflated, over the next week after surgery, the lung will gradually
expand and begin to function. Chest tubes may not be used to avoid increased respiratory work and
overdistention of the compromised lung. The mortality rate of children with diaphragmatic hernia is about
40%, with death often occurring because of associated anomalies of the heart, lung, and intestine and because
of premature birth (Kadir & Lilja, 2017).
An Imperforate Anus
An imperforate anus (Fig. 27.14) is a stricture or the absence of the anus (Gourlay, 2013). In week 7 of
intrauterine life, the upper bowel elongates to pouch and combine with a pouch invaginating from the
perineum. These two sections of bowel meet, the membranes between them are absorbed, and the bowel is
then patent to the outside. If this motion toward each other does not occur or if the membrane between the
two surfaces does not dissolve, an imperforate anus occurs. The disorder can be relatively minor, requiring
just surgical incision of the persistent membrane, or much more severe, involving sections of the bowel that
are many inches apart with no anus. There may be an accompanying fistula to the bladder in boys and to the
vagina in girls (retrovaginal fistula), further complicating a surgical repair. The problem occurs in
approximately 1 in 5,000 live births, more commonly in boys than in girls. It may occur as an additional
complication of spinal cord disorders because both the external anal canal and the spinal cord arise from the
same germ tissue layer.
Assessment
The condition may be detected by a prenatal sonogram. It is discovered at birth when inspection of a
newborn’s anal region reveals no anus, a membrane filled with black meconium protrudes from the anus, or if
it is impossible to insert a rubber catheter into the rectum. A “wink” reflex (touching the skin near the rectum
should make the anus contract) cannot be elicited if sensory nerve endings in the rectum are not intact. Even
with all these methods, some instances of the stricture will not be detected at birth because the anus appears
as usual, and the stricture exists so far inside that it can’t be seen. By 24 hours, no stool will be passed, and
abdominal distention will become evident. An X-ray or sonogram will reveal the disorder if the infant is held in
a slightly head-down position to allow swallowed air to rise to the end of the blind pouch of the bowel. This
method is also helpful to estimate the distance the intestine is separated from the perineum or the extent of
the correction that will be necessary.
Because newborns are discharged at 2 or 3 days or even a few hours after birth, it’s important that follow-up
care by parents includes an assessment of whether the infant is defecating. If not, they may be asked to
collect a urine specimen so it can be examined for the presence of meconium to help determine whether the
infant has a rectal–bladder fistula. Placing a urine collector bag over the vagina in girls may reveal a
meconium-stained discharge or that a rectovaginal fistula is present.
Therapeutic Management
The degree of difficulty in repairing an imperforate …
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