Pr. Dr. Ayman Reda
Children are not small adults.
They suffer from different disorders and their physical and
psychological responses are different.
Their capacity for adaptation is greater but they must endure any
consequences of disease and its management for longer.
In contrast to adults they rarely have comorbidity from degenerative
diseases or lifestyle problems but they can suffer the unique
consequences of congenital malformations.
Children must be treated within the context of their families
ANATOMY AND PHYSIOLOGY
Anatomical differences between adults and children are important in
surgery. Infants and small children have a wider abdomen, a
broader costal margin and a shallower pelvis.
Thus, the edge of the liver is more easily palpable below the costal
margin and the urinary bladder is an intra-abdominal organ. The ribs
are more horizontal and respiratory function is more dependent on
diaphragmatic movement. The umbilicus is relatively low lying.
In the small child, transverse supraumbilical incisions are preferred to
vertical midline ones for laparotomy.
Abdominal scars grow with the child and may migrate – a gastrostomy
sited in the epigastrium of the infant may end up as a scar over the
Special features that must be considered in
children when preparing for surgery
Thermoregulation Warm fluids, warm theatre, insulate child
Hypoglycaemia Maintain glucose level
Clotting Give intramuscular vitamin K preoperatively to neonates
Fluid and electrolyte Allow for higher sodium and fluid balance needs
Less postoperativeCatabolism Relatively lower postoperative energy requirements
Gastro-oesophageal reflux Use a nasogastric tube to prevent aspiration
Atypical presentations of infection High index of suspicion
Psychology Trained staff, day surgery for minor operations
Special features of surgical
technique in children
■ Gentle tissue handling
■ Bipolar diathermy is preferred to unipolar during dissection
■ Abdominal incisions can be closed with delayed absorbable
■ Bowel can be anastomosed with interrupted single-layer
■ Skin can be closed with absorbable subcuticular sutures
Esophageal Atresia and
• Esophageal atresia (EA) is a congenital
interruption or discontinuity of the esophagus
resulting in esophageal obstruction.
• Tracheoesophageal fistula (TEF) is an abnormal
communication (fistula) between the esophagus
EA may be present with or without a TEF.
Alternatively, a TEF can occur without EA.
• There is association of anomalies in patients
with EA/TEF that must be considered under the
acronym VACTERL (vertebral, anorectal,
cardiac, tracheal, esophageal, renal,limb).
• The diagnosis of EA should be entertained in an infant with
excessive salivation along with coughing or choking during the first
oral feeding. A maternal history of polyhydramnios is often present.
The inability to pass a nasogastric tube into the stomach of the
neonate is a cardinal feature for the diagnosis of EA. Inability to
pass a nasogastric tube in an infant with absent radiographic
evidence for gastrointestinal gas is virtually diagnostic of an isolated
EA without TEF
• On the other hand, if gas is present in the gastrointestinal tract
below the diaphragm, an associated TEF is confirmed. These
simple rules provide the correct diagnosis in most cases.
Occasionally, a small amount of isotonic contrast may be given by
mouth to demonstrate the level of the proximal EA pouch and/or the
presence of a TEF, but this is rarely necessary. In fact, the risk of
aspiration with studies of this type is generally high
• The immediate care of an infant with EA/TEF
includes IV fluid ,continuous suction of the
proximal EA pouch and manipulation of the
endotracheal tube distal to the TEF may
minimize the leak and permit adequate
ventilation. Further, placement of an occlusive
balloon (Fogarty) catheter into the fistula via a
bronchoscope may be useful.
• Finally, urgent thoracotomy with direct ligation of
the fistula after rapid preoperative preparation
Hypertrophic Pyloric Stenosis
• It is one of the most common gastrointestinal disorders
during early infancy, with an incidence of 1:3000 to 4000
• This condition is most common between the ages of 2
and 8 weeks. In HPS, hypertrophy of the circular muscle
of the pylorus results in constriction and obstruction of
the gastric outlet.
• Gastric outlet obstruction leads to nonbilious, projectile
emesis, loss of hydrochloric acid with the development of
hypochloremic, metabolic alkalosis, and ultimate
dehydration. Visible gastric peristalsis may be seen as a
wave of contraction from the left upper quadrant to the
epigastrium. The infants usually feed vigorously between
episodes of vomiting. Palpation of the pyloric tumor (also
called the olive) in the epigastrium or right upper
quadrant by a skilled examiner is pathognomonic.
• Ultrasound is diagnostic .
Hypertrophic Pyloric Stenosis
• The treatment of this
condition is by surgical
mechanical distraction of
the pyloric ring (by a
• Prior to surgery,it is
important that the infant is
hydrated with intravenous
fluids to establish a
normal urine output. It is
important that the
alkalosis is slowly
• Postoperatively, infants
are usually allowed to
resume enteral feedings.
• In contrast with more distal intestinal
atresias, duodenal atresia (DA) is
believed to occur as a result of failure of
vacuolization of the duodenum from it
solid cord stage. DA is associated with
several conditions, including prematurity,
Down syndrome, maternal
polyhydramnios, malrotation, annular
pancreas, and biliary atresia (BA).
• The classic plain abdominal radiograph of
DA is termed the double-bubble sign (air-
filled stomach and duodenal bulb).
• The treatment of DA is by surgical
bypass of the duodenal obstruction by
• Intrauterine focal mesenteric vascular
accident. The clinical presentation is
typically dependent on the level of
• The treatment of JIA is to re-establish
Anomalies of Intestinal
• Midgut volvulus is a true surgical
emergency since delay in operative
correction is associated with a high risk of
intestinal necrosis and subsequent death.
The sudden appearance of bilious emesis
in a newborn is the classic presentation.
• The meconium syndromes of infancy represent a complex group of
gastrointestinal conditions associated with CF( cystic fibrosis)
• Meconium Plug is a frequent cause of neonatal intestinal
obstruction and associated with multiple conditions including
Hirschsprung’s disease, maternal diabetes, hypothyroidism, and CF.
Typically, affected infants are often preterm and present with signs
and symptoms of distal intestinal obstruction. Plain abdominal
radiographs reveal multiple dilated loops of intestine. The diagnostic
and therapeutic procedure of choice is a water-soluble contrast
enema. This often results in the passage of a plug of meconium and
relief of the obstruction. The operative management of simple
meconium ileus is required when the obstruction cannot be relieved
with contrast enema.
• Complicated Meconium IleusMeconium ileus is considered
complicated when perforation of the intestine has taken place.
• Intussusception is the telescoping of one portion of the intestine into
the other and is the most common cause of intestinal obstruction in
• In most pediatric intussusceptions, the cause is unknown, the
location is at the ileocecal junction, and there is no identifiable
pathologic lead point. Invariably, there is marked swelling of the
lymphoid tissue within the region of the ileocecal valve.
• The incidence of a pathologic lead point is up to 12% in most
pediatric series and increases directly with age. The most common
lead point for intussusception is a Meckel’s diverticulum; however,
other causes must be considered including polyps, the appendix,
intestinal neoplasm, submucosal hemorrhage associated with
Henoch-Schönlein purpura, foreign body, ectopic pancreatic or
gastric tissue, and intestinal duplication.
• Intussusception classically produces severe, cramping abdominal
pain in an otherwise healthy child. The child often draws his or her
legs up during the pain episodes and is usually quiet during the
intervening periods. After some time, the child becomes lethargic.
Vomiting is almost universal. Although frequent bowel movements
may occur with the onset of pain, the progression of the obstruction
results in bowel ischemia with passage of dark blood clots mixed
with mucus, commonly referred to as “currant jelly” stool. An
abdominal mass may be palpated.
• In about half of cases, the diagnosis of intussusception can be
suspected on plain abdominal radiographs. Suggestive radiographic
abnormalities include the presence of a mass, sparse gas within the
colon, or complete distal small bowel obstruction. In cases where
there is a low index of suspicion for intussusception based on
clinical findings, an abdominal ultrasound may be the initial
diagnostic test. Ultrasonography can be diagnostic.
• When the clinical index of suspicion for intussusception is high,
hydrostatic reduction by contrast agent or air enema is the
diagnostic and therapeutic procedure of choice.
• Contraindications to this study include the presence of peritonitis or
• Further, an intussusception that is located entirely within the small
intestine is unlikely to be reached by enema and more likely to have
an associated lead point. Hydrostatic reduction using barium has
been the mainstay of therapy; however, more recently, the use of air
enema has become more widespread.
• Successful reduction is accomplished in more than 80% of cases
and is confirmed by resolution of the mass, along with reflux of air
into the proximal ileum.
• To avoid radiation exposure altogether, intussusception reduction by
saline enema under ultrasound surveillance may be employed.
• Recurrence rates after hydrostatic reduction are about 11% and
usually occur within the first 24 hours. Recurrence is usually
managed by another attempt at hydrostatic reduction. A third
recurrence is usually an indication for operative management.
Air enema reduction of an
Remember the following
■ Loose stools may be present
■ Tenderness and guarding in the right iliac fossa is characteristic
■ Exclude referred pain from right lower lobe pneumonia
■ Take special care in diagnosing appendicitis in the preschool child
■ Surgery is the treatment but only after fluid resuscitation and
• Hirschsprung’s Disease is characterized pathologically by absent
ganglion cells in the myenteric (Auerbach’s) and submucosal
(Meissner’s) plexus. This neurogenic abnormality is associated with
muscular spasm of the distal colon and internal anal sphincter
resulting in a functional obstruction. Hence, the abnormal bowel is
the contracted, distal segment, whereas the normal bowel is the
proximal, dilated portion.
• The area between the dilated and contracted segments is referred to
as the transition zone. In this area, ganglion cells begin to appear,
but in reduced numbers.
• The aganglionosis always involves the distal rectum and extends
proximally for variable distances. The rectosigmoid is affected in
about 75% of cases, splenic flexure or transverse colon in 17%, and
the entire colon with variable extension into the small bowel in 8%.
• The risk for Hirschsprung’s disease is greater if there is a positive
family history and in patients with Down syndrome.
Presentation and complication
• In most, infants are symptomatic within the first 24 hours of life with
progressive abdominal distention and bilious emesis. Failure to
pass meconium in the first 24 hours is highly significant and a
cardinal feature of this condition. In some infants, diarrhea may
develop due to the presence of enterocolitis.
• The diagnosis of Hirschsprung’s disease may also be overlooked for
prolonged periods. In these cases, older children may present with a
history of poor feeding, chronic abdominal distention, and a history
of significant constipation. Since constipation is a frequent
problem among normal children, referral for surgical biopsy to
exclude Hirschsprung’s disease is relatively frequent.
• Enterocolitis is the most common cause of death in patients with
uncorrected Hirschsprung’s disease and may present with diarrhea
alternating with periods of obstipation, abdominal distention, fevers,
hematochezia, and peritonitis.
• The initial diagnostic step in a newborn with radiographic evidence for a
distal bowel obstruction is a barium enema. Prior to this study, rectal
examination and enemas should be avoided so as not to interfere with the
identification of a transition zone.
In a normal barium enema study, the rectum is wider than the sigmoid
In patients with Hirschsprung’s disease, spasm of the distal rectum usually
results in a smaller caliber when compared with the more proximal sigmoid
Failure to completely evacuate the instilled contrast material after 24 hours
would also be consistent with Hirschsprung’s disease and may provide
additional diagnostic yield.
• Anorectal manometry may also suggest the diagnosis of Hirschsprung’s
disease. The classic finding is failure of the internal sphincter to relax when
the rectum is distended with a balloon.This is more often useful in an older
patient and is seldom used in neonates.
• A rectal biopsy is the gold standard for the diagnosis of Hirschsprung’s
disease. In the newborn period, this is done at the bedside with minimal
morbidity using a special suction rectal biopsy instrument. It is important to
obtain the sample at least 2 cm above the dentate line so as to avoid
sampling the normal transition from ganglionated bowel to the paucity or
absence of ganglia in the region of the internal sphincter.
Traditionally, in mangement of intestinal obstruction caused by this a
leveling procedure is done, followed by proximal diversion.
• A definitive procedure is performed later and involves variations of
pull through procedures among three main procedures.
• In the Swenson procedure, the aganglionic bowel is removed down to the level of the internal sphincters and a
coloanal anastomosis is performed on the perineum.
• In the Duhamel procedure, the aganglionic rectal stump is left in place and the ganglionated, normal colon is
pulled behind this stump. A GIA stapler is then inserted through the anus with one arm within the normal,
ganglionated bowel posteriorly and the other in the aganglionic rectum anteriorly. Firing of the stapler therefore
results in formation of a neorectum that empties normally, due to the posterior patch of ganglionated bowel.
• Finally, the Soave technique involves an endorectal mucosal dissection within the aganglionic distal rectum. The
normally ganglionated colon is then pulled through the remnant muscular cuff and a coloanal anastomosis is
• More recently, the Soave procedure has been performed in the newborn period as a primary procedure and
without an initial colostomy.
• The spectrum of anorectal malformations ranges from simple anal
stenosis to the persistence of a cloaca.
• By 6 weeks’ gestation, the urorectal septum moves caudally to
divide the cloaca into the anterior urogenital sinus and posterior
• Failure of this septum to form results in a fistula between the bowel
and urinary tract (in boys) or the vagina (in girls).
• Complete or partial failure of the anal membrane to resorb results in
an anal membrane or stenosis.
• Breakdown of the cloacal membrane anywhere along its course
results in the external anal opening being anterior to the external
sphincter (i.e., anteriorly displaced anus).
• An anatomic classification of anorectal anomalies is based on the
level at which the blind-ending rectal pouch ends in relationship to
the levator ani musculature . Historically, the level of the end of the
rectal pouch was determined by obtaining a lateral pelvic radiograph
(i.e., invertogram) after the infant is held upside down for several
minutes to allow air to pass into the rectal pouch.
Clinical picture and investigation
• Inspection of the perineum alone determines the pouch
level in 80% of boys and 90% of girls. Clinically, if an
anocutaneous fistula is seen anywhere on the perineal
skin of a boy or external to the hymen of a girl, a low
lesion can be assumed, which allows a primary perineal
repair procedure to be performed, without the need for a
• Most all other lesions are high or intermediate, and they
require proximal diversion by a sigmoid colostomy. This
is followed by a definitive repair procedure at a later
date. If required, the level of the rectal pouch can be
detailed more definitively by ultrasonography or MRI.
Abdominal Wall Defects
• During normal development of the human embryo, the midgut herniates
outward through the umbilical ring and continues to grow. By the 11th week
of gestation, the midgut returns back into the abdominal cavity and
undergoes normal rotation and fixation, along with closure of the umbilical
ring. If the intestine fails to return, the infant is born with abdominal contents
protruding directly through the umbilical ring and is termed an omphalocele .
Most commonly, a sac is still covering the bowel, thus protecting it from the
surrounding amniotic fluid. Occasionally, the sac may be torn at some point
in utero, thus creating confusion with the other major type of abdominal wall
defect termed gastroschisis. In contrast with omphalocele, the defect seen
with gastroschisis is always on the right side of the umbilical ring with an
intact umbilical cord, and there is never a sac covering the abdominal
contents. The major morbidity and mortality with either anomaly are not as
much with surgical repair of the abdominal defect as they are with the
associated abnormalities. In the absence of other major anomalies, the
long-term survival is excellent.
• The treatment of an omphalocele consists of a nasogastric or orogastric
tube decompression for prevention of visceral distention due to swallowed
air. An intravenous line should be secured for administration of fluids and
broad-spectrum antibiotics. The sac should be covered with a sterile, moist
dressing and the infant transported to a tertiary care pediatric surgery
• Prior to operative repair, the infant should be evaluated for potential
chromosomal and developmental anomalies by a careful physical
examination, plain chest radiograph, echocardiography if the physical
examination suggests underlying congenital heart disease, and renal
ultrasonography. Since the viscera are covered by a sac, operative repair
of the defect may be delayed so as to allow thorough evaluation of the
• Several options exist for the surgical management of an omphalocele and
are largely dictated by the size of the defect. In most cases, the contents
within the sac are reduced back into the abdomen, the sac is excised with
care to individually ligate the umbilical vessels, and the fascia and skin are
• Fascial closure may be facilitated by stretching the anterior abdominal wall
as well as milking out the contents of the bowel proximally and distally.
• In giant omphaloceles, the degree of visceroabdominal disproportion
prevents primary closure and the operative management becomes
more challenging. Construction of a Silastic silo allows for gradual
reduction of the viscera into the abdominal cavity over several days.
Monitoring of intraabdominal pressure during reduction may prevent
the development of an abdominal compartment syndrome. Once the
abdominal contents are returned to the abdomen, the infant is taken
back to the operating room for formal fascia and/or skin closure.
• Occasionally, closure of the fascia may be impossible. In these
cases, the skin is closed and a large hernia is accepted. This is
repaired after 1 or 2 years. When the skin cannot be closed over the
defect, several options exist, including the topical application of an
antimicrobial solution to the outside of the sac such as silver nitrate
or silver sulfadiazine. Over time, this results in granulation tissue
and subsequent epithelialization of the sac. A repair of the large
hernia is then performed a few years after this.
in the pediatric age group
• Repair of an inguinal hernia (IH) represents one of the most frequent
surgical procedures performed in the pediatric age group. Virtually
all IH in children are indirect and congenital in origin.
• Most IH present as a bulge in the region of the external ring
extending downward for varying distances to the scrotum or labia.
Often, the hernia is detected by a pediatrician during a routine
physical examination or observed by the parents. Inguinal pain may
also be a presenting complaint.
• Incarceration and possible strangulation are the most feared
consequences of IH and occur more frequently in premature infants.
Because of the risk for these complications, all IH in children should
IH repair in premature infants
• The timing for IH repair in premature infants is controversial.
Early repair may be associated with a higher risk for injury to
the cord structures, greater recurrence rate, and anesthetic-
related apnea. These factors must be weighed against the
higher risk for incarceration and strangulation, the potential for
losing the patient during follow-up, and the development of a
larger IH with loss of domain in the abdominal cavity. Taking
these factors into account, most pediatric surgeons perform
herniorrhaphy before the neonate is discharged to home from
• If the infant has already been discharged home, most pediatric
surgeons wait until the infant is older than 60 weeks
postconception (gestational age + postnatal age). After this
age, the risk for postoperative apnea is diminished.
• In patients with incarcerated IH containing bowel,
attempts should be made to reduce the hernia, unless
there is clinical evidence of peritonitis.
• This may require intravenous sedation and careful
• If the reduction is successful, the child is admitted and
observed for 24 to 48 hours. The IH repair should be
done after the period of observation to allow for tissue
edema to subside.
• On the other hand, if the IH cannot be reduced, the child
should be promptly taken to the operating room for
Biliary Atresia BA
• BA is characterized by progressive (not static) obliteration of the
extrahepatic and intrahepatic bile ducts. The cause is presently unknown.
Patients who are not offered surgical treatment uniformly develop biliary
cirrhosis, portal hypertension, and death by 2 years of age.
• Pathologically, the biliary tracts contain inflammatory and fibrous cells
surrounding minuscule ducts that are probably remnants of the original
ductal system. Bile duct proliferation, severe cholestasis with plugging, and
inflammatory cell infiltrate are the pathologic hallmarks of this disease. This
histology is usually distinct from the giant cell transformation and
hepatocellular necrosis that are characteristic of neonatal hepatitis, the
other major cause of direct hyperbilirubinemia in the newborn.
• A serum direct bilirubin level higher than 2.0 mg/dL or greater than 15% of
the total bilirubin level defines cholestasis and is distinctly abnormal, and
further evaluation is mandatory. Delay in diagnosis of BA is associated with
a worse prognosis. Thus, the initial opportunity for success in the
management of this disease relies on the early recognition of abnormal
• In addition to a careful history and physical examination, blood and
urine should be obtained for bacterial and viral cultures, reducing
substances in the urine to rule out galactosemia, serum IgM titers for
syphilis, cytomegalovirus, herpes, and hepatitis B, serum α1 -
antitrypsin level and phenotype, serum thyroxine level, and a sweat
chloride test done to exclude CF
• Ultrasonography of the liver and gallbladder is important in the
evaluation of the infant with cholestasis. In BA, the gallbladder is
typically shrunken or absent, and the extrahepatic bile ducts cannot
• The next diagnostic step is to perform a percutaneous liver biopsy if
the hepatic synthetic function is normal. This is well tolerated under
local anesthesia, and the diagnostic accuracy is in the range of 90%.
• If the needle biopsy and/or the abdominal ultrasound are consistent
with BA, exploratory laparotomy (laparoscopy) is then performed
• The initial goal at surgery is to confirm the diagnosis. This requires
the demonstration of the fibrotic biliary remnant and definition of
absent proximal and distal bile duct patency by
• The classic technique for correction of BA is the Kasai
hepatoportoenterostomy. In this procedure, the distal bile duct is
transected and dissected proximally up to the level of the liver
capsule, whereby it is excised, along with the gallbladder remnant .
A Roux-en-Yhepaticojejunostomy is then constructed by
anastomosis of the jejunal Roux-limb to the fibrous plate above the
• A cystic enlargement of the common
bile duct is referred to as a
Type I cysts represent 80% to 90% of
cases and are simply cystic dilations of
the common bile duct.
Type II cysts are represented as a
diverticulum arising from the common
Type III cysts are also referred to as
choledochoceles and are isolated to
the intrapancreatic portion of the
common bile duct and frequently
involve the ampulla.
Type IV cysts are second in frequency
and represent dilation of both
intrahepatic and extrahepatic bile
In type V cysts, only the intrahepatic ducts
• The pathophysiology of choledochal
cysts remains poorly understood.
Clinical picture and investigation
• Although choledochal cysts can produce symptoms in any age group, most
become clinically evident within the 1st decade of life. The triad of a right
upper quadrant mass, abdominal pain, and jaundice is highly suggestive of
• In some patients, pancreatitis may bepresent.
• In older children and adults, the presentation may be more insidious and
include choledocholithiasis, cholangitis, and cirrhosis with progression to
• Malignant degeneration is also found in up to 16% of adults with
• In addition to routine measurement of serum bilirubin, alkaline phosphatase,
and amylase levels, the most useful diagnostic test for choledochal cysts is
ultrasonography. Once dilation of the extrahepatic biliary ducts is
demonstrated, no further testing is usually necessary in children.
• Although seldom necessary, preoperative endoscopic retrograde
cholecystopancreatography may provide additional information regarding
the pancreaticobiliary ductal anatomy to guide intraoperative decision
• Total cyst excision with Roux-en-Y hepaticojejunostomy is the definitive
procedure for management of types I and II choledochal cysts.
• In cases whereby there is significant inflammation, it may be impossible to
safely dissect the entire cyst way from the anterior surface of the portal vein.
In these circumstances, the internal lining of the cyst can be excised,
leaving the external portion of the cyst wall intact.
• Type III cysts are typically approached by opening the duodenum,
resecting the cyst wall with care to reconstruct and marsupialize the
remnant pancreaticobiliary ducts to the duodenal mucosa.
• In type IV cysts, the bile duct excision is coupled with a lateral hilar
dissection to perform a jejunal anastomosis to the lowermost intrahepatic
• If the intrahepatic cysts are confined to a single lobe or segment, hepatic
resection may be indicated.
• The treatment of type V cysts involving both lobes is usually palliative with
transhepatic or U tubes until liver transplantation can be performed.
• The postoperative outcomes following excision of choledochal cysts are
• Neuroblastoma (NBL), the most common abdominal malignancy in
• These tumors are of neural crest origin and, as a result, may arise
anywhere along the sympathetic ganglia or within the adrenal
• Although these tumors may occur at any site from the brain to the
pelvis, 75% originate within the abdomen or pelvis, and half of these
occur within the adrenal medulla. Twenty percent of NBLs originate
within the posterior mediastinum, and 5% are within the neck.
• The median age at diagnosis is 2 years.
• Approximately 25% of patients present with a solitary mass that may
be cured by surgical therapy, whereas most present with extensive
locoregional or metastatic disease.
• The presenting symptoms of NBL are dependent on several factors, including the site
of the primary tumor, the presence of metastatic disease, the age of the patient, as
well as the metabolic activity of the tumor.
• The most common presentation is a fixed, lobular mass extending from the flank
toward the midline of the abdomen. Although the abdominal mass may be noted in an
otherwise asymptomatic child, patients may complain of abdominal pain,distention,
weight loss, or anorexia. Bowel or bladder dysfunction may arise from direct
compression of these structures by the tumor.
• Cervical tumors may be discovered as a palpable or visible mass or be associated
with stridor or dysphagia.
• Posterior mediastinal masses are usually detected by plain chest radiographs in a
child with Horner’s syndrome, dyspnea, or pneumonia.
• Further, the tumor may extend into the neural foramina and cause symptoms of
spinal cord compression.
• Marrow replacement by tumor may result in anemia and weakness.
• Numerous paraneoplastic syndromes can occur in conjunction with NBL. Cerebellar
ataxia, involuntary movements, and nystagmus are the hallmark of the “dancing eyes
and feet” syndrome. Excess secretion of vasoactive intestinal polypeptide may
stimulate an intractable watery diarrhea. Hypertension may be significant, owing to
excessive catecholamine production by the tumor.
• A spot urine should be tested for the catecholamine metabolites
homovanillic and vanillylmandelic acid.
• A serum lactate dehydrogenase level higher than 1500 IU/mL, serum ferritin
level higher than 142 ng/mL, and neuron-specific enolase levels higher than
100 ng/mL correlate with advanced disease and reduced survival.
• CT and/or MRI are the preferred modalities for characterizing the location
and extent of the NBL. This tumor frequently infiltrates through vascular
structures . As such, many tumors that cross the midline are generally not
• A CT scan of the chest should be done to exclude pulmonary metastasis,
and a bone scan should be done to identify potential bone metastasis.
• In addition, radiolabeled metaiodobenzyl guanidine (MIBG) is one of the
single best studies to document the presence of metastatic disease.
• Finally, a bone marrow aspirate .
• Current therapy for NBL is multimodal:
incorporating surgery, chemotherapy,
radiation, and occasionally immunotherapy.
• Teratomas are tumors that contain elements derived from more than
one of the three embryonic germ layers. In addition, teratomas must
contain tissue that is foreign to the anatomic site in which they
occur. Teratomas can occur anywhere in the body and present as
cystic, solid, or mixed lesions. When they occur during infancy and
early childhood, they are most commonly extragonadal. In contrast,
in older children teratomas most frequently involve the gonads.
• Teratomas occur most frequently in the neonatal period and the
sacrococcygeal region is the most common site. Sacrococcygeal
teratoma (SCT) is four times more common in females and is most
often an obvious external presacral mass.
• 80% are left-sided
• Symptoms and signs include dyspnea, chest
retractions, decreased breath sounds on
• Prenatal ultrasound is accurate in 40–90% of
cases, showing herniation of abdominal contents
in thorax ,chest film, arterial blood gas
measurements, echocardiogram; ultrasound for
neural tube defects.
• The posterolateral location of this hernia is known as
Bochdalek’s hernia and distinguished from the
congenital hernia of the anteromedial, retrosternal
• diaphragm, which is known as Morgagni’s hernia.
• Primary repair or mesh repair once respiratory status
has been optimized
Failure of complete urethral tubularisation in the male fetus results in
hypospadias, a common congenital anomaly affecting about one in
every 200–300 boys. In most cases the urethra opens just proximal to
the glans penis but in severe cases the meatus may be on the penile
shaft or in the perineum.
The dorsal foreskin is hooded and there is a variable degree of chordee (a
ventral curvature of the penis most apparent on erection) .
Glanular hypospadias may be a solely cosmetic concern but more
proximal varieties interfere with micturition and erection.
In severe forms of hypospadias, additional genitourinary anomalies and
intersex disorders should be excluded.
Surgical correction of distal hypospadias is frequently undertaken before 2
years of age, often as a single-stage operation.
Proximal varieties may require complex staged procedures.
Surgery aims to achieve a terminal urethral meatus so that the boy can
stand to micturate with a normal stream, a straight erection and a
penis that looks normal.
Ritual circumcision must be avoided in
infants with hypospadias because the
foreskin is often required for later