CONGENITAL ANOMALIES

Submitted to, Submitted by

Mrs. Sharon Suryamol K S
Asst. Professor 2nd Year MSc Nursing
Govt. College of Nursing Govt. College of Nursing
Kottayam Kottayam

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 CONGENITAL ANOMALIES
INTRODUCTION
Those defects and diseases which are substantially determined before or during birth and
recognized in early life are known as congenital abnormalities, some defects require emergency
or elective surgical management where as some others require no emergency management and
they are classified as minor defects. Prevention of congenital malformations should focus on
environmental factors rather than attempting to improve heredity towards successful
reproductive outcome.

CONGENITAL ANOMALY
A congenital anomaly (congenital abnormality, congenital malformation, birth defect) is a
condition which is present at the time of birth which varies from the standard presentation.

Definition
According to WHO the term congenital anomalies include all biochemical, structural and
functional disorders present at birth and the congenital malformations should be confined to
structural defects only present, at birth.
CONCEPTS OF CONGENITAL ANOMALIES
Congenital abnormalities are those defects and diseases which are substantially determined
before or during birth and recognizable in early life. Some disorders are detected at birth, eg cleft
lip and cleft palate. Some are obvious in early life like congenital dislocation of hip which is
detected until walking commence and some may become apparent until much later in life, e.g.,
patent ductus arteriosus, which is diagnosed usually in school age or even later. Some disorders
may remain unrecognized. Some defected are classified as major which may require surgical
interventions, either as emergency or as elective. Some are classified as minor that have no
functional implications, like skin tags in front of the ear.
Congenital malformations may be regarded as one form of reproductive failure. The favourable
environmental and genetic factors result in normal re-production and the unfavourable factors
lead to sterility, abortion, stillbirth, preterm births of neonatal death. Congenital anomalies
represent a relative reproductive success when compared to sterility or abortion. Prevention of
congenital defects must be achieved by special attention to environmental factors rather than by
attempting to improve heredity towards successful reproductive outcome.
INCIDENCE
The global incidence of congenital disorders is estimated about 30 to 70 per 1000 live birth
(1989). Approximately half of these infants have fatal outcome or lifelong chronic diseases.
Actual numbers vary widely between countries. The most common congenital defects are
congenital hearts diseases and central nervous system malformations.

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In India, the incidence of congenital defects is about 2.5 to 4 precent among children. It is
considered as third most frequent cause of prenatal mortality in India. Congenital anomalies are
also considered as one of the important causes of physical and mental handicapped conditions
among survivors. Most common type of birth defects is CNS abnormalities, approximately 22
percent of all defects. In northern part of India, neural tube defects are most common, whereas in
the rest of India, musculoskeletal disorders are commonly found.
RISK FACTORS
Some factors are considered to be significantly associated with incidence of congenital
anomalies.
 Advanced maternal age
Elderly mother has risk of birth of baby with Down's syndrome or other congenital anomalies
 Consanguinity
Baby born out of consanguineous marriages (among blood relations), eg, marriage with first
cousin or uncle-niece, are at risk of congenital defects like mental retardation.
 Maternal malnutrition especially folic acid deficiency can lead to CNS defects and iodine
deficiency a can lead to mental retardation of other congenital anomalies.
ETIOLOGY OF CONGENITAL ANOMALIES
The causes of most of the congenital anomalies are not fully understood. Majority of the causes
are unknown of due to complex interaction between genetic and environmental factors and is
considered as multifactorial. Approximately 65 percent cases may be due to multifactorial,
whereas about 25 percent case are due to genetic factors and about 10 percent cases are due to
environmental factors.

Genetic factors
Congenital anomalies are inherited through the genes in the ovum or sperm. The anomalies may
be related to chromosomal abnormalities, single gene disorders or polygenic inheritance. Single
gene disorders may be either autosomal or X-linked inheritance, which may be dominant or
recessive traits. There are about 3000 known genetic disorders.
Chromosomal abnormalities occur either in the form of numerical or structural alterations and
found time to time in human beings. They arise in various ways, i.e., non-disjunction,
translocation, deletion, duplication, inversion, isochromosomes and mosaicism. The most
common chromosomal abnormality related to autosomes is Down's syndrome or Trisomy 21.
Single Gene Disorders
Single gene disorders are found as autosomal dominant inheritance, autosomal recessive
inheritance and X-linked inheritance. The principal characteristics of the autosomal and X linked
inherited disorders are as follows;

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a. Autosomal-dominant traits
In this condition, every affected child has at least one affected parent (except mutations).
unaffected individual needs only be heterozygous for the given allele. Male and female offspring
are equally affected (50-50). There is a risk of 50 percent involvement of each sibling of an
affected individual, if the parent is affected. There are affected individuals in several generations.
b. Autosomal-Recessive Traits
In this disease, each parent of an affected individual must carry atleast one mutant allele (normal
parents are carriers). Every affected individual is homozygous for the given allele. Individual
possessing a single mutant allele do not show the trait. Either sex may be affected and there is a
25 percent risk of involvement of the sibling of an affected individual. The disease tends to be
rare but more severe than dominantly inherited traits. X-linked inheritance of sex-linked
inheritance X-linked disorders are inherited abnormalities in which the abnormal genes are
carried on the sex (X) chromosomes. The know two types are, X-linked recessive and X-linked
dominant: The Y-chromosome is known to carry only one genetic trait, i.e. hairy ears. Therefore,
all of the known sex-chromosome disorders involve one of the X-chromosomes
c. Polygenic or multifactorial inheritance
Each individual carries a variable number of genes which, if combined in a certain way, can
cause physical defects in offspring.
A polygenically determined abnormality is the result of a combination of these multiple genes
that is one specific gene is totally responsible for the defect. The intrauterine and postnatal
environment can be influential in this form of inheritance, and may be responsible for the
polygenetic disorder. Multifactorial inheritance is a combination of polygenic and environmental
factors.
Some examples of multifactorial inheritance are pyloric stenosis, cleft lip and/or palate, isolated
heart disease, anencephaly, meningomyelocele, diabetes, idiopathic epilepsy, asthma,
schizophrenia, mental retardation, colon cancer, coronary artery disease, etc If the parents are
unaffected, the recurrence risk of the specific defect is usually given in the range of 3 to 5
percent. If one parent is affected the risk is more. The more siblings affected the higher the risk
or recurrence a future offspring, even as high as 11 to 21 percent.

Environmental factors
Various environmental factors are responsible for occurrence of congenital anomalies.
Teratogenic agents of the environment adversely affect the normal cellular development of the
embryo or fetus causing birth defects. The common environmental factors are:
A. Intrauterine infections especially by STORCH (Syphilis, Toxoplasmosis, Rubella
Cytomegalovirus and Herpes Virus).

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B. Drugs intake by the mother during pregnancy like steroid hormones, stilbestrol,
anticonvulsants, folate antagonists, cocaine, lithium, thalidomide, etc.
C. X-ray exposure during pregnancy.
D. Maternal diseases like diabetes, cardiac failure, malnutrition, folic acid deficiency, Iodine
deficiency disorders, endocrine abnormalities, etc.
E. Abnormal intrauterine environment like bicornuate uterus, septed uterus, polyhydramnios,
oligohydramnios, foetal hypoxia, etc.
F. Maternal addiction with alcohol, tobacco or smoking (active or passive).
G. Environmental pollution, especially air pollution.
Effects of the teratogens depend upon the severity of exposure, the gestational age of fetus at the
time of exposure and the maternal and fetal immune response to the teratogenic agents. The fetus
is potentially susceptible to some teratogenic effect even after the completion of organogenesis.
The ultimate effect may be death, malformations, growth retardation of fetus of functional
disorders.

COMMON CONGENITAL ANOMALIES

V- Vertebral defect
A- Anorectal anomalies
C-Cardiac defect
T-Trachea oesophageal fistula
E-Oesophageal Artesia
R-Renal problems
L-Limb defects
There are thousands of known congenital anomalies affecting various system of the body. Most
common problems are related to central nervous system
MAJOR CONGENITAL ANOMALIES
 Cleft lip
 Cleft palate
 Congenital heart disease
 Neural tube defects
 Brain anomalies
 Omphalocele
 Hepatosplenomegaly
 Asymmetry of face
 Generalized dysmorphism

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 Specific skin lesions

MINOR CONGENITAL ANOMALIES

 Flat occiput
 Prominent occiput
 Window's pek
 Triple hair whorl
 Flat bridged nose
 Ear lobe crease
 Ear lobe notched
 Forward displacement or protruding ears
 Cleft uvula
 Webbed neck
 Extra nipples
 Single umbilical artery
 Umbilical hernia
 Taperd fingers
 Overlapping digits
 Broad thumb or great toes
 Increased space between toes
 Syndactyly
 Polydactyly
DIAGNOSTIC APPROACHES
An accurate and early diagnosis is of primary importance. It depends upon the available facilities
for diagnostic tests. It is now possible to identify the healthy carriers of a number of genetic
disorders like haemophilia, thalassemia, inborn errors of metabolism, etc. There are thousands of
known genetic diseases and hundreds of established patterns of malformation syndromes, so it
may be difficult to diagnose all conditions accurately. But various prenatal and postnatal
investigations help to detect the congenital abnormities for necessary care.

Prenatal Diagnosis
Advanced medical technology helps in the detection of congenital anomalies in intrauterine
period by the following diagnostic measures:
a. Amniocentesis in early pregnancy, about 14 to 16 weeks, helps in prenatal diagnosis of
chromosomal abnormalities (Down's syndrome) and many inborn errors of metabolism
(galactosemia).
b. Chorionic villus sampling for cytogenetic study to detect chromosomal aberration especially
in advanced maternal age.

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c. Estimation of maternal serum alpha-fetoprotein and chorionic gonadotropin to screen out
neural tube defect and trisomy
d. Ultrasonography for fetal profile.
e. Fetoscopy with special fetoscope or endoscope.
f. Amniography with radio opaque dye to diagnose soft tissue abnormalities.
g. Fetal blood sampling and skin scraping test, protein assay and DNA diagnosis
h. Radiography
i. Antenatal screening for maternal diseases, metabolic and endocrinal functions by regular
examination, laboratory investigations and details family history. Prenatal diagnosis of
congenital anomalies helps the parents to decide for the medical termination of pregnancy of to
allow the birth of a malformed baby of some time for intrauterine corrective surgical
interventions.

Postnatal diagnosis
Postnatal diagnosis is easy by various approaches. Details maternal and family history along with
thorough physical examination of the new born baby helps in diagnosis. There are numbers of
screening tests for early diagnosis of genetic abnormalities like sex chromosome abnormalities,
congenital dislocation of hip, PKU, congenital hypothyroidism, sickle cell disease, cystic
fibrosis, Duchene muscular dystrophy, congenital adrenal hyperplasia, G6PD deficiency, etc.
Biochemical assay, cytogenetic study, blood test, hormonal assay, radiography, ultrasonography,
etc. are useful to detect different anomalies for early management.
NERVOUS SYSTEM
NEURAL TUBE DEFECTS
Neural tube defects are the congenital malformations of the CNS resulting from a defective
closure of the neural tube during early embryogenesis between 3d and 4th week of intrauterine
life. It involves the defects in the skull, vertebral column, the spinal cord and other portion of
CNS. It occurs in about 1 to 5 per 1000 live births. Risk in second sibling is high. The defect is
usually obvious at birth and varies in severity from spina bifida occulta to anencephaly.
ETIOLOGY
The exact cause of neural tube defect is not known, but the triggering factors are maternal
radiation exposures, drugs (valproic acid), exposure to chemicals, malnutrition especially folic
acid deficiency and genetic determinant. These factors alone or in one or the other combination,
adversely affect the normal development of the neural tube, thee causing the defect.
TYPES OF NEURAL TUBE DEFECTS

Spina Bifida

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Spina bifida is one of the commonest anomalies of neural tube development seen in infants. It is
the congenital defect of the spinal column due to failure of the fusion of vertebral arches with or
without protrusion of the meninges and dysplasia of the spinal cord. It is the malformation of the
spine in which the posterior portion of the lamina of the vertebra fails to close. It can be only a
small deformed lamina separated by a midline gap or may be a complete absence of lamina.
Spina bifida can be broadly divided into two groups, spina bifida occulta and spina bifida cystic
Spina bifida cystic is commonly found as meningocele and meningomyelocele

Spina Bifida Occulta

It is the frequent and most benign neural tube defect. It is most posterior arch and laminae of the
vertebrae, usually L5 and S1. There is no protrusion of the of the meninges. But the dysplasia of
the spinal cord is a prominent feature. Most of the cases are asymptomatic. The symptomatic
children usually present after 6 to 8 years of age with any of the following (a) progressive
deformity of the foot, (b) changes in micturition pattern, (c) alteration in the gait and (d) trophic
ulcers on the toes and feet. Other significant anomalies of the spinal cord (syringomyelia,
diastematomyelia, tethered cord etc) may be found in association of the condition. Progressive
neurological deficits require surgical correction of the defect. Laminectomy is done and the
intraspinal lesion is excised. Operation can be done even before neurological deficit appears in
selected cases. CT scan and MRI help to confirm the diagnosis before operation.

Meningocele
It is hernia protrusion of the meninges through a midline defect in the posterior vertebral arch. It
forms a fluctuating cystic swelling filled with CSF and covered by thin transparent membrane or
with skin. It transilluminates easily. It is generally found in the lower back, i.e. lumbo-sacral
region. It may also be found in the thoracic region and in the skull (cranial meningocele). The
spinal cord and nerve roots are usually normal. There is no dysplasia of the spinal cord and the
child may find asymptomatic. It is relatively uncommon lesion (4 to 5 %). The symptomatic
child may present weakness of the legs or lack of sphincter control. Associated anomalies like
hydrocephalus, tethered cord may be present. As the skin or membrane covering is thin, CSF
leakage may present and there is chance of infections Surgical closure of the sac should be done
as early as possible to prevent infections. Prognosis is generally good unless hydrocephalus and
neurological deficits are developed.

Meningomyelocele (Myelomeningocele)
It is a midline cystic of meninges with spinal tissue and CSF, which herniates through a defect in
the posterior vertebral arch. It is one of the commonest lesions (90 to 95%) and be present
anywhere on the midline in the back, but lumbosacral area is the most common site. There is
dysplasia of the spinal cord and this is always accompanied by neurological deficit. These can be
found as two types (a) myelocele (open type) and (b) myelomeningocele (open type), the
commonest cystic lesion containing neural tissue which may be dysplastic spinal cord or nerve
fibres. The child with myelomeningocele may present with flaccid paralysis, absence of

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sensation and drop reflex. Postural abnormalities like club foot, subluxation of hips may be
present.

ANENCEPHALY
Anencephaly is a congenital absence of cranial vault with the cerebral hemisphere completely
missing or reduced to small masses. Various congenital anomalies can be associated with this
condition, like congenital heart disease, cleft palate, etc. This condition is incompatible with life,
death usually occurs with a week or two of birth.
ENCEPHALOCELE
It is a sac like protrusion of meanings brain substance (cerebral cortex, cerebellum. or part of
brainstem) herniating through a congenital bony defect in the skull.
It is commonly found in the midline and in the occipital or parietal area. It may also find on
frontal bone in the orbit or in the nose. The size may vary from small to as big as size of head.
The survived child may develop hydrocephalus, visual problems, seizures, microcephaly and
mental retardation. Associated congenital anomalies are usually present, Le cleft lip and palate,
abnormal genitalia, congenital nephrosis. Prenatal diagnosis can be done by estimation of alpha-
fetoprotein and USG. In postnatal period, the conditions is evident at birth X-ray skull and CT
scan help to detect them with associated anomalies.

HYDROCEPHALUS
Hydrocephalus is the abnormal accumulation of cerebrospinal fluid (CSF) in the intracranial
spaces. It occurs due to imbalance between production or absorption of CSF or due to
obstruction of the CSF pathways. It results in the dilatation of the cerebral ventricles and
enlargement of head.
ETIOLOGY
Hydrocephalus may occur due to congenital or acquired causes.
TYPES
Congenital Hydrocephalus.
It occurs due to the following's conditions Intrauterine infections mainly in rubella,
toxoplasmosis, cytomegalovirus, tumour obstructing the CSF flow, Intracranial haemorrhage.
Congenital malformations like aqueduct stenosis.
Arnold-chiari malformation (displacement of the brain-stem and cerebellum through foramen
magnum), Dandy-Walker anomaly (congenital septum or membrane blocking the outlet of 4th
ventricle). Malformations of arachnoid villi.

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Acquired Hydrocephalus
It occurs usually following the conditions like,
Inflammation-Meningitis, Encephalitis.
Trauma - Birth injury, head injury, intracranial haemorrhage.
Neoplasm - Space occupying lesions like tuberculoma, subdural hematoma or abscess, gliomas,
ependymoma, astrocytoma, choroid plexus papilloma, pseudotumor cerebri.
PATHOLOGY
The ventricular system becomes greatly distended and dilated. Increased intraventricular
pressure leads to thinning of cerebral cortex and cranial bones. Ependymal lining of ventricles is
disrupted resulting in periventricular ooze. Subependymal edema occurs and white matter is
compressed. Downward bulging of third ventricle compresses the optic nerves and hypophysis
cerebri with dilation of Sella tunica Choroid plexus is usually atrophied to some degree. Cortical
atrophy may also occur.
CLINICAL MANIFESTATIONS
Congenital hydrocephalus starts in fetal life and presents at birth or within first few months of
like. The features include excessive enlargement of head, delayed closure of anterior fontanel,
tense and bulging fontanel with open sutures. Presence of signs of increased intracranial pressure
and alteration of muscle tons (spasticity) if the extremities are common presenting features.
There is delayed in head holding of the infant.
Later, with gradual increase in size of the head, the child presents with protruding forehead,
shiny scalp with prominent scalp veins. The eye brows and eyelids may be drawn upwards gaze
resulting the sun-set' sign of eyes. The cracked-pot (MacEwen's) sign may be elicited I by
percussion of head. Transillumination is positive. Mental function and neurological
manifestations are usually wary with the causative and associated factors. The children may have
normal intelligence. Hypocephalus occurring late in childhood, may not present with large head.
The features may be found as increased intracranial pressure (ICP) with papilledema. Spasticity,
ataxia, urinary incontinence and progressive deterioration of mental activities.
DIAGNOSTIC EVALUATION
Physical examination and neurological assessment along with serial measuring of head
circumference is helpful to diagnose the condition. Increase in head circumference in first 3
months of life, more than 1 cm every 15 days and persistent widening of squamo-parietal sutures
should arouse suspicion of hydrocephalus. Positive transillumination of infant head, typical
cracked-pot sound (Macewan's sign) of the skull bone, ophthalmoscopy are important diagnostic
measures
MRI, CT scan, cranial ultrasonography and X-ray skull also can be performed to diagnose the
underlying pathology and involvement of the intracranial structures.

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MANAGEMENT
Management of hydrocephalus depends upon specific cause, associated malformations, clinical
course and severity of the condition Surgery may not be indicated, if the hydrocephalus gets
spontaneous arrest. Surgical management is indicated in obstructive hydrocephalus, in rapid
enlargement of head, visual or in life threatening increase ICP Ventriculostomy and choroid
plexectomy have been performed with variable results. Surgical shunts are the treatment of
choice at the present time. Intracranial or extracranial shunt is done to bypass the obstruction and
to divert the CSF from the ventricular system to another compartment. The most commonly
performed extracranial shunt is ventriculo-peritoneal shunt (V-P shunt). Other approaches are
ventriculostrial shunt, ventriculopleural shunt or ventriculogallbladder shunt.
NURSING DIAGNOSIS
The important nursing diagnoses are,
 Altered cerebral tissue perfusion related to increased ICP.
 Altered nutrition, less than body requirement related to reduced oral intake
 Risk for impaired skin integrity related to enlarged head.
 Anxiety related to the abnormal condition and surgical interventions.
 Risk for infection related to introduction of infecting organism through the shunt.
 Risk for fluid volume deficit related to CSF drainage. Ineffective family coping related to life
threatening problem of infant.
COMPLICATIONS
Hydrocephalus can be complicated with seizures, herniation of brain, persistent increased ICP,
developmental delay, infections, neurological deficits, motor and intellectual handicaps, visual
problems (squint, optic atrophy, field defect) aggressive and delinquent behaviour etc.
Ventriculoarterial (V-A) shunt may be complicated with endocarditis, bacteremia,
thromboembolism, ventriculitis, corpulmonale etc. shunt dependency also may develop.

THE KIDNEY AND UNRINARY TRACT

Congenital malformations of the kidneys and urinary tract are commonly found in the paediatric
surgery units. These problems usually required surgical correction. Some of them are producing
no clinical symptoms, but approximately about 25 percent cases of chronic renal failure are due
to congenital anomalies.
RENAL AGENESIS
Renal agenesis is the absence of kidney due to failure of ureteric bud formation. mesenchymal
blastema differentiation of final mesenchymal condensation it may be unilateral or or bilateral
and associated with various congenital anomalies. Bilateral renal associated malformations is
known as Pother's syndrome.
RENAL HYPOPLASIA AND DYSPLASIA

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Renal hypoplasia occurs due to reduction of renal mass affecting the nephron. It may be
unilateral or bilateral. It is sometimes difficult to differentiate from renal dysplasia which is
characterized by disorganization of renal parenchyma with immature nephron and ductal
elements resulting in a large or small kidney. Renal hypoplasia may be simple, segmental or
oligo-meganephric type. Renal dysplasia may be multicystic and sometimes hypoplastic or
aplastic. It can be segmental or total and may be associated with urinary tract obstruction.

HORSE-SHOE KIDNEY
Horse-shoe kidney develops when lower poles of the kidneys are fused in the midline due fusion
of ureteric buds during fetal development. These kidneys are more vulnerable to develop wilm's
tumour than in general.
DUPLEX KIDNEY
 With 2 ureters and renal pelvis
 Results from incomplete division of the metanephric diverticulum or ureteric bud.
POLYCYSTIC KIDENYS
Polycystic kidney is one of the commonest congenital anomalies as inherited autosomal disease.
It is a complex syndrome resulting from progressive dilatation of specific portion of the nephron
which may arise during the development and the completion of development of nephron due to
adverse hereditary metabolic environment.
OBSTRUCTIVE LESIONS OF THE URINARY TRACT
Obstructive uropathy is mainly caused by congenital abnormalities like pelvic junction
obstruction and posterior urethral valves which may lead to irreversible renal damage. Other
congenital causes of obstructive lesions are duplex renal system, neuromuscular bladder
dysfunction, meatal stenosis, phimosis, etc. Acquired conditions like renal calculi, trauma,
tumour, tuberculosis also may produce the obstruction.

PELVI-URETERIC JUNCTION STENOSIS

Pelvi-ureteric junction (PUJ) obstruction is commonly found as unilateral or bilateral stenosis of
PUJ causing hydronephrosis. It can be associated with ectopic and horse-shoe kidney. PUJ
stenosis may present as a flank mass without any symptoms of with UTI and upper abdominal
pain.
HYDRONEPHROSIS
Hydronephrosis is the dilatation of renal pelvis which may be found as unilateral or bilateral. It
may be due to obstruction of urine flow in the distal urinary tract or reflow of urine up the
ipsilateral ureter or due to bladder neck obstruction or urethral obstruction.
POSTERIOR URETHRAL VALVE (PUV)

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Posterior urethral valves almost invariably occur in the male child and most frequent cause of
distal urinary tract obstruction. The child present with dribbling of urine, abnormal urine stream,
palpable bladder, recurrent urinary tract infections (UTIS), vomiting and failure to thrive. In
severe and prolonged obstruction, renal dysplasia and anuria may develop.

MEATAL STENOSIS
Meatal stenosis may occur as congenital abnormality. It may be found due to meatal ulcer and
caring, following infection or circumcision and improper hygiene. It can cause lower urinary
tract obstruction. Meatal dilatation or meatoplasty may be needed to relieve the obstruction.
EPISPADIAS
Epispadias is the congenital abnormal urethral opening on the dorsal aspect of the penis. Urethra
is displaced dorsally due abnormal development of the infraumbilical wall and upper wall of the
urethra. It is usually associated with exstrophy of bladder and ambiguous genitalia. Rarely it may
find in female infants. epispadias in male child
A. Anterior epispadias with normal continence.
a. Glandular
b. Balanltic or penile.
B. Posterior epispadias associated with incomplete bladder neck and incontinence of urine
a Penopubic
b. Sub symphyseal
The male infants with epispadias are having short and broad penis with dorsal curvature In
females, cleft extends along the roof or entire urethra, involving the bladder neck Urethra is short
and patulous.
Female epispadias can be classified as:
A. Bifidelitoris with no incontinence of urine
B. Sub symphyseal with incontinence of urine In both male and female, pubic bones are usually
found separated, along with epispadias
MANAGEMENT
Management of epispadias is done by the surgical correction usually in three stages. First stage
operation is done in about 1.5 to 2 years of age for penile lengthening, elongation of urethral
strip and chordee correction. Second stage operation is done at least 6 months after first stage for
urethral reconstruction. Third stage operation is done about 3 to 4 years of age bladder neck

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reconstruction and correction of VUR. Cystoplasty can be done to enhance the capacity after 2 to
3 years of 3 stage operation.
HYPOSPADIAS
Hypospadias is the congenital abnormal urethral opening on the ventral aspect (under surface) of
the penis. It is one of the commonest malformations of male children.

Classification
Hypospadias can be classified depending upon the site of the urethral meatus.
A. Anterior hypospadias (65-70%). It may be found as glandular or coronal or on distal penile
shaft.
B. Middle (10-15%) penile shaft hypospadias
C. Posterior hypospadias (20%) It may found on proximal penile shaft or as penoscrotal, scrotal
or perineal type.
Problems Related to hypospadias
A child with hypospadias may have following problems
a. Presence of painful downward curvature of the penis during erection as chordee
b. chordee, there is deflected stream of urine and the child wets his thigh during urination.
c. Inability to void urine while standing, in case of penoscrotal, scrotal and perineal hypospadias
it also may found with the penis in the normal elevated position.
d. There can be meatal stenosis, fistula, urethral stricture of stenosis or diverticulum.
MANAGEMENT
Management of hypospadias is done by surgical reconstruction to obtain straight penis at
erection, to form urethral tube and urethral meatus at the tip of glans penis

GASTRO INTESTINAL CONGENITAL MALFORMATIONS

CLEFT PLATE AND CLEFT LIP
Clefts occur due to the failure of union in the development of the face and mouth. Defects may
occur in the lip or palate alone or may be combined. Bilateral clefts are sometimes found.
CAUSES
1. Failure of embryonic development - cause not.
2. Hereditary factor.
3. May be related to mutant genes, chromosomal abnormalities, teratogens.
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Associated problems
1. Eating
a) Suction cannot be created for effective sucking
b) Food returns through the nose
2. Nasal speech
3. Lack of normal dental function and appearance Feeding is the main problem. An artificial
palate e.g., orthodontic palate is used for feeding.
COMPLAICATIONS
a) Pierre Robin syndrome
b) Intellectual deficits
c) Recurring otitis media
d) Faulty social adjustment related to Poor-self-concept and abnormal speech Plastic surgery is
required to correct the defects. Cleft lip is repaired after 3 months of age
PREOPERATIVE PREPARATION
Treat the infant as a normal one. There is difficulty in feeding So, a soft nipple with a large hole
should be used. In extreme cases dropper feeding or gavage feeding is required. It' we use
dropper put it it on the back of the tongue. The hands of infant are retrained by a clove hich
restraints or elbow restraints to avoid irritation by contact on the lips with the hands. The
clothing and bleeding time of blood should be tested. Keep the mouth clean and lips lubricated.
Give water after feeds to clean the mouth. The mother and nurse should watch the child for signs
of aspiration, respiratory distress and gastrointestinal disturbance. Protect the child from
infection
POSTOPERATIVE CARE
The infant will require close observation to keep the airway open. Observe the tissues of the
mouth, tongue, nostrils for swelling and if present, report to the doctor. A laryngoscope suction
apparatus, endotracheal tube should be ready for use if necessary. Cleanse the suture line with
the prescribed solutions frequently with great care. The lip should be patted, not wiped. For
cleansing, hydrogen peroxide solution can be used. Mineral oil may be applied following the
gentle cleansing to prevent crusting. If a crust forms, a scar is to result. If a suture is pulled or
sloughs out, the suture line will not be even.
Meet all physical needs like personal hygiene, nutritional needs, elimination needs and give
psychological care. The child should be kept comfortable warm and dry. He should not cry
excessively use a logan bar to avoid stress on the suture line. Allow the mother to stay with the
child. Give only clear fluids for several days. If a medicine dropper is used, it is inserted in the

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corner of the mouth so as not to touch the area about the suture line, He is fed slowly and in a
sitting position. He is bubbled frequently both for relief of air which he has swallowed and for its
general comforting effect Water is given to cleanse the mouth of milk after the feeding. Give
water between feedings.
If the band is restrained for improving circulation, alternatively the hand should be released
under supervision. To provided emotional satisfactions, the child should be cuddled. The infant
should never be placed up on his abdomen or even up on his side if there is likelihood that he
may roll over on his face. In the lateral position he is likely to aspirate mucus or milk. Change
the position frequently to prevent hypostatic pneumonia. The adhesive tapes of logan bar should
be kept clean. After two weeks, when the wound heals, bottle or breastfeeding is allowed. The
mother should express her milk when the child is not
Health teaching on discharge
1. Advise the parents not to reject or overprotect the child.
2. Parents should be taught how to feed the child and the general care of the child.
3. Dropper feeding without touching the palate is taught.
4. Advise the parents to bring the child for frequent medical check-up

ANORECTAL MALFORMATIONS
Anorectal Malformations (ARMs) are developmental deformities of the lower end of the
alimentary tract.i.e., the anorectal canal. The exact cause of these malformations is not known. It
occurs due to arrest in embryonic development of the anus, lower rectum and urogenital tract at
the 8th week of embryonic life.
CLINICAL MANIFESTATIONS
The important presenting features are abnormally formed or no anal opening and absence of
meconium or presence of fistula with passage of stool through the fistula. In female baby the
fistula may present between rectum and vagina or perineum. In male baby the fistula is
commonly found between rectum and urinary tract or perineum. Presence of meconium in urine
may be found in some children. Progressive abdominal distension and vomiting may present.
Rectal tube cannot be inserted into the rectum during examination.
IMPERFORATE ANAL MEMBRANCE
Infant fails to pass meconium. Greenish bulging membrance is seen on examination. Bowel and
sphincter return to normal after excision.
ANAL STENOSIS
It accounts for 10 percent of all ARMs. The baby may pass ribbon-like stools with difficulty as
the anal opening is very small.
ANAL AGENESIS

16
It presents with only anal dimple. Usually, fistulas are found to the perineum or urethra in male
and perineum or vulva in female. Intestinal obstruction develops, if there is absence of any
fistula.
RECTAL AGENESIS
This condition accounts for 75 percent of all ARMs. It presents with fistula. In male baby. fistula
may communicate with posterior urethra and in female with upper vagina. Associated manor
congenital malformations are common.

RECTOPERINEAL FISTULA
It is found as small orifice in the perineum, usually anterior to the center of the external
sphincter. In male baby it is found close to the scrotum and in female the vulva
RECTOVAGINAL FISTULA
It presents with a communication between rectum and vagina and stool passed through the
vagina.
DIAGNOSIS
Physical examination of the neonates is the most important diagnostic measure of ARMS. USG
helps to locate the rectal pouch. X-ray with inverted infant (upside down position),i.e.
invertogram or Wangensteen-rice X-ray is useful to locate rectal pouch which can be performed
only after the infant is 24 hours of age.
MANAGEMENT
The reconstructive surgery is done to correct or repair the congenital malformations. It depends
upon the type of anomalies and sex of the infant. In case of low ARMS, rectal cutback anoplasty
or Y V plasty is done for male infants and dilatation of fistula with - definitive repair or perineal
anoplasty is performed for female infants.
In case of high ARMS, initial colostomy is done in the neonatal period followed by definitive
reconstructive surgery as posterior sagittal anorectoplasty (PSARP) at the age of 10 to 12 months
or when the infant is having 7 to 9 kg of body weight. Colostomy closure is done after 10 to 12
weeks of successful definitive surgery.
NURSING MANAGEMENT
Basic pre-operative and post-operative care should be provided as for abdominal surgery Special
interventions in pre-operative period should include maintenance of warmth, fluid and electrolyte
balance and general stability of the infant. Measurement of abdominal girth is important before
surgery. Special care to be provided for colostomy.
COMPLICATIONS

17
The common complications of ARMS are urinary tract infection, intestinal obstruction, faecal
impaction, colostomy related problem, recurrence of fistula, anal stenosis and post operative
complications.
PROGNOSIS
Prognosis of ARMS depends upon type of anomalies, presence of associated malformations,
operative skill, presence of neuromuscular fecal control and general health of the infant.

IMPERFORATE ANUS
The term imperforate anus is used to describe all congenital abnormalities of the anorectal canal
or in the location of the anus within the perineum. Usually seen immediately after birth or within
several hours.
CAUSES
1. An arrest is embryologic development of the anus, lower rectum,and urogenital tract at
the 8th week of embryonic life. 2. Closely associated with other congenital deviations, including:
a. congenital heart diseases
b. Esophageal atresia
c. Spinal malformations.
d. Hydronephrosis
e. Low birth weight..
TYPES
There are four types of anorectal defects
1. Stenosis of the anus
2. Anal membrane atresia
3. Anal agenesis
4. Rectal atresia
CLINICAL MANIFESTATIONS
1. No anal opening
2. Thermometer or small finger cannot be inserted into recturn

18
3. Absence of meconium stool
4. Green tinged urine if fistula is present (high, male)
5. Progressive abdominal distention
6. Fistula is likely to be present
a) Female-occurs between rectum and vagina or perineum
b) Male-occurs between rectum and urinary tract, scrotum, or perineum.

DIAGNOSIS
When we are taking the rectal temperature, no meconium is seen in the thermometer and catheter
cannot be passed through. X-ray will confirm the type of anomaly.
COMPLICATIONS
1. Rectal stenosis or prolapse, usually confined to mucosa
2. Separation of anastomosis
3. Urethral injury or stricture
4. Urinary retention and infection
5. Recurrent urinary fistulas
6. Fecal impaction or incontinence
clothing and bleeding time of blood should be tested. Keep the mouth clean and lips lubricated.
Give water after feeds to clean the mouth. The mother and nurse should watch the child for signs
of aspiration, respiratory distress and gastrointestinal disturbance. Protect the child from
infection.
Health teaching on discharge
1. Advise the parents not to reject or overprotect the child.
2. Parents should be taught how to feed the child and the general care of the child.
3. Dropper feeding without touching the palate is taught.
4. Advise the parents to bring the child for frequent medical check-up
TREATMENT
1. Low female
a. Decompression of bowel with catcher irrigations

19
b. Dilatation of fistula for 8- 12 months thereafter
c. Definitive repair
2. Low-male
a. Rectal cutback anoplasty or Y-V plasty)
b. Local dilatation of fistula
3. High-male
a. Colostomy for decompression
b. Definitive pull-through surgery-deferred until about 1 year of age or when child
attain 6.75-9 kg. (15-20 lbs.) 4. High-female

Preoperative care
1. Once the diagnosis is confirmed, gastric suction is used.
2. Withhold oral feeds. Note any vomiting, colour and amount.
3. Give intravenous fluids to maintain fluid and electrolyte balance.
4. In high type of imperforate anus a colostomy is needed before surgical correction.
5. Care of colostomy is important after surgery.
6. In case of stenosis, dilation and correction is done. In some case anoplasty is done.
7. Meticulous skin care is very essential.
8. Nasogastric tube may be passed to decompress the stomach.
9. Observe the patient carefully for any signs of distress and report it to physician. Check vital
signs frequently and maintain temperature stability.

Postoperative care
The infant will require close observation to keep the airway open. Observe the tissues of the
mouth, tongue, nostrils for swelling and if present, report to the doctor. A laryngoscope suction
apparatus, endotracheal tube should be ready for use if necessary. Cleanse the suture line with
the prescribed solutions frequently with great care. The lip should be patted, not wiped. For
cleansing, hydrogen peroxide solution can be used. Mineral oil may be applied following the
gentle cleansing to prevent crusting. If a crust forms, a scar is to result. If a suture is pulled or
sloughs out, the suture line will not be even.
Meet all physical needs like personal hygiene, nutritional needs, elimination needs and give
psychological care. The child should be kept comfortable warm and dry. He should not cry
excessively use a logan bar to avoid stress on the suture line. Allow the mother to stay with the

20
child. Give only clear fluids for several days. If a medicine dropper is used, it is inserted in the
corner of the mouth so as not to touch the area about the suture line, He is fed slowly and in a
sitting position. He is bubbled frequently both for relief of air which he has swallowed and for its
general comforting effect Water is given to cleanse the mouth of milk after the feeding. Give
water between feedings. If the band is restrained for improving circulation, alternatively the hand
should be released under supervision. To provided emotional satisfactions, the child should be
cuddled. The infant should never be placed up on his abdomen or even up on his side if there is
likelihood that he may roll over on his face. In the lateral position he is likely to aspirate mucus
or milk. Change the position frequently to prevent hypostatic pneumonia. The adhesive tapes of
logan bar should be kept clean.

DIAPHRAGMATIC HERNIA OR HIATUS HERNIA

Hiatus hernia is the protrusion of the abdominal organ through the defect in the diaphragm into
the thoracic cavity.
SIGNS AND SYMPTOMS
1. Severe respiratory difficulty such as tachypnoea, dyspnoea, retractions and cyanosis.
2. Reduced chest movements on the affected side.
3. Excessive crying of the child.
4. Peristaltic sound are heard in the thoracic cavity.
5. Shock and hypoxia.
Diagnosis is confirmed by chest and abdominal X-ray examination.
NURSING MANAGEMENT
1. At birth, resuscitation of the child with positive pressure breathing is needed
2. Do suction of the secretions, maintain the airway and give oxygen inhalations.
PREOPERATIVE CARE
1. The child is placed on the affected side as in semi-fowler's position to help in the expansion of
the lung and to keep the abdominal viscera in the abdomen.
2. Keep the infant as quick as possible.
3. Ryles tube aspiration is done to decompress the stomach and to reduce respiratory difficulty.
4. Prophylactic antibiotics are given to prevent infection.
5. Intravenous fluids are given to maintain nutrition.

21
6. The child is prepared for emergency surgery.
POSTOPERATIVE CARE
1. Care of the gastrostomy and chest drainage is same as that for oesophageal atresia surgery.
2. Care and observation of the incisional site is important since wound dehiscence is a major
problem in children.
3. Keep the baby quiet to prevent pressure on the suture line by crying
4. Record the TPR ½ hrly and maintain normothermia.
5. Give reassurance and physcological support to parent.
6. Nasopharyngeal suctioning, frequent change of position, and chest essential to prevent
atelectasis to help in the expansion of the lungs.
7. Motivate the mother for the child care
OMPHALOCELE
An omphalocele is the hemiation of intestines into the umbilical cord.
NURSING MANAGEMENT
[Link] defect is covered with sterile saline dressings.
[Link] should be kept moist.
[Link] pressure is allowed on the abdomen.
4. Ryles tube aspiration is done to decompress the stomach.
5. Surgical treatment is by skin flap technique. In this, the viscera is replaced to the abdomen and
the abdominal wall is closed.
6. Postoperatively, the infant may be fed by peripheral hyperalimentation and then by
gastrostomy.

CONGENITAL PYLORIC STENOSIS

Pyloric stenosis is an abstraction at the pyloric sphincter caused by hypertrophy of the circular
muscle with narrowing of its lumen.
SIGNS AND SYMPTOMS
1. Severe and peristalsis, projectile vomiting.
2. Vigorous regurgitation.
3. Hypertrophy and dilatation of the stomach

22
4. Palpable mass in right upper quadrant of abdomen.
5. loss of weight
6. Dehydration.
7. Oliguria
8. Constipation.
9. Gastritis
DIAGNOSIS
1. Palpation of pyloric mass in conjunction with persistent, projectile vomiting.
2. Tests for metabolic alkalosis- due to loss of hydrochloric acid and potassium from vomiting
3. Urine becomes alkaline and concentrated.
4. Blood haemoglobin elevated.
5. X-ray examination with barium.
TREATMENT
1. Initial treatment
a. Rehydrate and correct electrolytes
b. Correct alkalosis
2. Surgical: pyloromyotomy (Fredet-Ramstedt procedure)
a. Hypertrophy of the pyloric muscle regresses to normal size but about 12 weeks
postoperatively
b. Gastroesophageal reflux may be a complication of surgery.

Preoperative preparation
1. Observation and recording of vital signs.
2. Observation of vomiting and stool.
3. Observation, recording and reporting of dehydration.
4. Sucking of fingers and hyperperistalsis is observed
5. Frequent suctioning of the mouth and pharynx and maintenance of respiration
6. Collection of specimens for investigations and assisting with diagnostic procedures.
7. Withhold oral fluids and administer intravenous fluids.

23
8. Lavaging of the stomach with isotonic saline.
9. If oral feeds are given, bubble the baby frequently.
10. Change position 2hourly.
11. Daily record the weight.
12. In case of dehydration, give intravenous replacement therapy and maintain fluid and
electrolyte balance.

Postoperative care
1. Provision of adequate fluids and nutrition to maintain fluid electrolyte balance.
2. Oral feedings are started after 6 hours of surgery. Glucose and electrolyte water orally with
slow advance to full and regular diet.
3. Feed slowly and bubble freely
4. Continue to elevate the infants head and shoulders after feeding for 45-60 minutes for several
feedings after surgery. Place on right side to aid gastric emptying.
5. Regurgitation may continue for a short period after surgery.
6. Record TPR 1/hrly. Observe for vomiting, abdominal distension, cyanosis, restlessness, pallor.
If abdominal distension is present.
7. Advise parents to bring the child for follow-up care.

INTESTINAL OBSTRUCTION
Intestinal obstruction occurs in neonate 1/1500 births.
CAUSES
I. Atresia and stenosis especially in ileum, duodenum, jejunum and colon.
2. Meconium ileus.
3. Megacolon
4. Malrotation
5. Intra-abdominal hernias
6. Paralytic ileus
7. Intussusception tumour
8. Polyp
9. Strangulation

24
SIGNS AND SYMPTOMS
It varies according to the degree and site of obstruction
 Fever
 Increased W.B.C. count
 Toxicity and shock
 Vomiting
 Dehydration
 Reduced bowel sounds
 Abdominal distension
Diagnosis is confirmed by barium enema and abdominal X-ray.

Nursing management
The main anticipated dangers are aspirations, respiratory embarrassment and abdominal
distension.
1. Continuous nasogastric suction is essential.
2. Measure the abdominal girth.
3. Intravenous infusions are given to correct dehydration.
4. For meconium ileus, no operative treatment is required. For other types, surgery required.
5. The preoperative care of these infants includes maintaining fluid and electrolyte balance,
blood transfusions, correction of acidosis with sodium bicarbonate and prophylactic
administration of antibiotics. The post operative care is same as that any other abdominal
surgery.

CONGENITAL MEGACOLON HIRSCHSPRUNG'S DISEASE

Congenital megacolon is caused by a congenital absence of autonomic parasympathetic ganglion
cells in the submucosal and intramuscular neural plexuses of the wall of the intestine. It is the
commonest cause of intestinal obstruction in neonates.
CAUSES
1. Failure of migration of the cell of the embryonic neural crest into the wall of the intestine.
2. Failure of the plexus to progress in a craniocaudal direction within the intestinal wall.
SIGNS AND SYMPTOMS
1. No meconium is passed
2. Abdominal distension

25
3. Bile-stained vomiting
4. Weight loss, dehydration
5. Alternate episodes of constipation and diarrhoea
6. Lack of appetite
7. Failure to thrive in older children and chronic constipation
8. Abdominal distension.
9. Stool appears ribbon like, fluid like, or in pellet form
DIAGNOSIS
Diagnosis is confirmed by rectal examinations, barium enema, manometry.
1. Rectal examination: exhibits absence of fecal material
2. Roentgen examination with barium enema
3. Rectal biopsy: absence or less no of ganglion nerve cells
4. Anorectal manometry: records the reflex response of sphincter
5. Ultrasonogram
MEDICAL AND SURGICAL MANAGEMENT
In case of the mild condition, isotonic saline enemas, stool softness and a low residue diet is
given. In severe cases, surgical removal of the ganglionic bowel is done to improve the
functioning of the internal sphincter. A temporary loop colostomy is done prior to the corrective
surgery. Surgical treatment is
1. Swenson's procedure
2. Duhamel's procedure
NURSING MANAGEMENT
In the newborn, the nurse observes for the passage of meconium, abdominal distension and
vomiting. In a older child, observation is for anorexia, undernutrition, irritability, distended
abdomen and constipation. Once the diagnosis is confirmed the nurse should teach the parents
about the management and also about the need of surgery. A low residue diet is advised for the
child.

Preoperative care
1. The parents should be instructed about the surgery and about the need of colostomy.
2. Vital sighs ate taken and recorded.
3. Abdominal girth is measured daily.
26
4. Child is kept in a semi fowlers position to help in lung expansion,
5. Withhold oral feeds to prevent abdominal distension:
6. Daily rectal tube to relieve distension.
7. Daily rectal wash is given with normal saline.
8. Prophylactic antibiotics are given to prevent infection.
9. Observe for complications due to intestinal obstruction, pain, shock and vomiting.
10. To prevent dehydration, give intravenous fluids.

Postoperative care
1. Assessment of level of consciousness
2. Recording of vital signs, 1/2hrly until it stabilizes
3. Observations of the dressings for soakage and bleeding
4. Place the child in a comfortable position as per order of surgeon
5. Nil orally, only intravenous fluids are given unless ordered
6. Oral feeds are given only after the bowel sounds are heard.
7. Care of the nose and mouth is given
8. Pulmonary infection is prevented by deep breathing, coughing and turning exercises
9. Would infection is prevented by proper hand washing and by keeping strict aseptic technique
10. Observe the stoma of the colostomy for infection and follow aseptic technique while caring
for colostomy

INTUSSUSCEPTION
Intussusception is the invagination or telescoping of a portion of the intestine into an adjacent,
more distal section of the intestine.

Causes
1. May be due to increased mobility of intestine and hyperperistalsis present in young children.
2. Possible contributing causes in older child
 Meckel's diverticulum
 Polyps, cysts in the bowel
 Malrotation of intestines
 Acute enteritis
 Abdominal injury

27
  Abdominal surgery
 Intestinal intubation
CLINICAL MANIFESTATIONS
(A) Age
1. Incidence is rare in first month of life.
2. 4-10 months is most common age of onset (50%-70%) in well-nourished, otherwise, healthy
child
3. 1-2 years of age- frequency of occurrence is high.
(B) Sudden onset
1. Paroxysmal abdominal pain
2. Currant jelly-like stools
a) Blood and mucus present in stool
b) One or more stools with this characteristic
c) Presence of bloody mucus on finger following rectal examination
d) Hemoccult positive
3. Vomiting
4. Increasing absence of stools.
5. Increasing abdominal distention and tenderness
6. Dehydration and fever
7. Shock like state
DIAGNOSIS
1. general condition and appearance of child history
2. x ray examination
3. ultrasonogram

Treatment
1. Hydrostatic reduction of telescoped bowel with barium enema used during first 48hrs after
onset.
2. Surgical reduction of intussusceptions, resection if bowel is gangrenous.

Preoperative care

28
1. Assist in maintaining or restoring hydration and electrolyte balance.
2. Prevent vomiting and aspiration.
3. Be aware of the patient's condition by frequent observations.
4. Prepare the patient for surgery when he is shock like or febrile.
5. Offer support to the parents during this time of crisis and fear.

Postoperative care
1. Careful monitoring of vital signs and general condition especially abdominal tenderness,
bowel sounds, lethargy, and tolerance to fluids.
2. Be alert for complications after surgery
 Fever
 Diarrhea
 Shock
 Dehydration
 Toxicity
3. Assist in maintaining stomach decompression until first stool is passed.
4. Oral fluid is begun after first stool is passed-about 4-5 days after surgery
5. Start with glucose and water.

CONGENITAL ANOMALIES OF MUSCULOSKELETAL SYSTEM

DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH)
DDH describes those conditions involving the abnormal development of the proximal femur
and/or acetabulum. It may be associated with other congenital anomalies. Left hip in more
commonly affected, but bilateral involvement occurs in more than 50 percent of cases. Girls are
affected 8 times more than boys. DDH was previously known as congenital dislocation of hip
(CDH).
TYPES
[Link]
[Link]
[Link]
ETIOLOGY

29
DDH is caused by genetic and environmental factors. It may develop following breech delivery
or other difficult deliveries, when head of the femur may get dislocated upward and backward
which may cause development of a false acetabulum.
PATHOPHYSIOLOGY
The structures of hip joint, i.e., acetabulum, femoral head and capsule may not develop properly.
These lead to partial or complete dislocation of femoral head from the shallow acetabular cavity.
The degree of DDH may include as follows:
Dysplasia - This condition is found as shallow acetabulum with upward slant of acetabular roof.
Subluxation - In this condition acetabular surface of the femoral head is in contact with shallow
dysplastic acetabular surface but the head slides laterally and superiorly.
Dislocation - Here articular cartilage of completely displaced femoral head does not contact with
acetabular articular cartilage.

CLINICAL MANIFESTATIONS
The physical findings during neonatal examination or afterward include asymmetry of the thigh,
presence of gluteal and knee folds, diminished spontaneous movements, shortening of the
affected leg, inability to abduct the hip fully and posterior bulging of femoral head. The affected
leg appears short and externally rotated.
Positive Ortolani sigh (forced abduction of hip causes a clicking sound), positive
Trendelenburg's sign (downward left of pelvis on affected side) and positive Barlow's test
including hip instability are important. If not treated, the child starts walking delayed with
presence of pain in the joint, waddling gait, and lordosis. Subluxation of the hip joint may be
detected at birth or may manifest several months later.
DIAGNOSTIC TEST
 Physical examination
 Ortolanis maneuver and Barlows maneuver
 Allis sign
 Ultra sound and Xray
BARLOWS TEST
If the hip is dislocated that is, if the hip can be popped out of socket with this maneuver -the test
is considered positive. The Ortolani maneuver is then used, to confirm the positive finding (i.e.,
that the hip actually dislocated).
GALEAZZI TEST

30
The Galeazzi test, also known as the Allis sign, is used to assess for hip dislocation, primarily in
order to test for developmental dysplasia of the hip. It is performed by flexing an infant's knees
when they are lying down so that the feet touch the surface and the ankles touch the buttocks.
MANAGEMENT
 Casting
 Skin traction
 Pavlik harness:This is a specially designed harness to gently position your baby's hips in a
well aligned and secure position.
 Surgery
 Closed reduction
 Open reduction
NURSING MANAGEMENT
 Teach parents regarding application of pavlik maneuver
 Explain about feeding and bathing
 Skin care
 Assess the warmth and colour of feet
 Evaluate the child for any complications,
 Joint swelling consistent for at least 6 weeks, decrease in motor skills such as the ability
to hold a fork or pencil
 Consistent fever
 Chronic fatigue
 Malaise or feelings of being unwell

CLUB FOOT
DEFINITION
Clubfoot is a complex deformity of foot result of complicated interrelationship between bone,
ligaments and muscles.
CAUSES
 Hereditary-defective gene
 Excessive pressure of amniotic fluid on fetus causes excessive pressure of uterine wall on
fetus.
 Mechanical cause (Uterine compression)
 Arrested fetal development
 Circulatory failure to calf and foot muscles Irradiation(maternal) during pregnancy.
CLASSIFICATION
Talipes Equinovarus: 95% all cases

31
In this condition, the foot is fixed in plantar flexion and deviates medially that is the heal is
elevated. If the condition is not corrected, the child walks on the toes and the outer boarder of the
foot.
Talipes Calcancovalgus:
In this the foot is dorsiflexed and deviates laterally that is the heel is turned outward from the
midline of the body and the anteriar part of the foot is elevated on the outer border. If not
corrected the child walks on the outwardly turned heel and the inner border of the foot. These
conditions may be unilateral or bilateral Other less commonly observed types are:
Talipes varus: Due to the heel's being turned inward from the midline of the leg. Only the outer
portion of the sole rests on the floor
Talipes valgus: Due to the heels being turned outward from the midline of the leg. Only the inner
side of the sole rests on the floor
Talipes Equinovalgus: Due to the heels being elevated and turned outward from the midline of
the body.
Talipes calcancovarus: Due to the heels being turned towards the midline of the body and
anterior part of the foot being elevated. Only the heels rests on the floor.

TERMS
Talipes: Foot and ankle
Varus: Bending inward
Valgus: Bending outward

Nursing Management
 The short-term goals in the care of infant; who has clubfoot are to correct the deformity
and to maintain the body part in as normal a position as possible.
 The long-term goal is to prevent a recurrence of the deformity.
 Manipulation or exercise program to be done several times a day is gaining the
cooperation of the parents
Care of an Infant in a Denis Browne Splint
 A Denis Browne splint may be used for infants who have clubfoot.
 This appliance is made of two footplates attached to a crossbar.
 When the splint is fitted to the feet, varying position of angulation of feet occur

CONCLUSION

32
 Congenital disorders are one of the main causes of the global burden of disease, and low-
and middle-income countries are disproportionately affected. These areas are also less likely to
have facilities to treat reversible conditions such as clubfoot, leading to more pronounced and
long-lasting effects. Some congenital disorders can be treated with surgical and non-surgical
options, such as cleft lip and palate, clubfoot and hernias. Others, including heart defects, neural
tube defects, and down syndrome, can cause lifelong impacts.

REFERENCE
1. PSN Menon. IAP Textbook of Pediatrics. 4 th edition. Volume 1. Jaypee Brothers
Publications. Tamilnadu.
2. Gupta. PG Textbook of Pediatrics. Volume 1. 1st edition. Jaypee Publications. New Delhi.
2015.
3. M Rennie. Rennie and Robertson’s Textbook of Neonatology. 5th edition. Churchil
Livingstone publisher. 2012.
4. Meharban Singh. Essential Pediatrics for Nurses. 4th edition. CBS Publishers. New
Delhi. 2017.
5. Pal. Textbook of Pediatric Nursing. 1st edition. Paras Medical Publisher. 2016.

33