Thyroglossal Duct Cyst

One of the most common lesions in the midline of the neck is the thyroglossal
.duct cyst, which most commonly presents in preschool-age children
Thyroglossal remnants are involved with the embryogenesis of the thyroid
gland, tongue, and hyoid bone and produce midline masses extending from
the base of the tongue (foramen cecum) to the pyramidal lobe of the thyroid
.gland. Complete failure of thyroid migration results in a lingual thyroid
Ultrasound or radionuclide imaging may therefore be useful to identify the
.presence of a normal thyroid gland within the neck
Thyroglossal duct cysts may be located in the midline of the neck anywhere
from the base of the tongue to the thyroid gland. Most, however, are found
.at or just below the hyoid bone
The classic treatment is Sistrunk operation( complete excision of the cyst in
continuity with its tract, the central portion of the hyoid bone, and the tissue
above the hyoid bone extending to the base of the tongue) .Failure to remove
these tissues results in a high risk of recurrence, or fistula
 Esophageal Atresia and
Tracheoesophageal Fistula

• 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
and trachea.
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).
 Diagnosis
• 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
 Management
• The immediate care of an infant with EA/TEF
includes 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
(exclude VACTERL)
Hypertrophic Pyloric Stenosis
• It is one of the most common gastrointestinal disorders during early infancy, with an
incidence of 1:3000 to 4000 live births.
• 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 .
• The treatment of this condition is by surgical mechanical distraction of the pyloric ring
(by a pyloromyotomy).
• 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 underlying metabolic alkalosis
is slowly corrected.
• Postoperatively, infants are usually allowed to resume enteral feedings.
 Duodenal Atresia
• 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
duodenoduodenostomy.
 Jejunoileal Atresia
• Intrauterine focal mesenteric vascular
accident. The clinical presentation is
typically dependent on the level of
obstruction.
• The treatment of JIA is to re-establish
intestinal continuity.
 Anomalies of Intestinal
Rotation/Fixation
• 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.
 Meconium Syndromes
• 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. Abdominal distention
is a prominent feature. 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

• Intussusception is the telescoping of one portion of the intestine into

the other and is the most common cause of intestinal obstruction in
early childhood.
• 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.
 Clinically
• 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.
 Management
• 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 hemodynamic
instability.
• 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.
 Hirschsprung’s Disease
• 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.
 Investigation
• 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 colon. In patients
with Hirschsprung’s disease, spasm of the distal rectum usually results in a smaller
caliber when compared with the more proximal sigmoid colon. 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. An important
goal of this study is to exclude other causes of constipation in the newborn such as
meconium plug, small left colon syndrome, and atresia.
• 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. The advantage of this method is that it can be done in an outpatient
setting, without the need for general anesthesia.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.
 Treatment
• Traditionally, 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
performed.
• More recently, the Soave procedure has been performed in the newborn
period as a primary procedure and without an initial colostomy.
 Anorectal malformations
• 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
anorectal canal.
• 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
stoma.
• 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
• Defects of the anterior abdominal wall are a relatively frequent anomaly
managed by pediatric surgeons. 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.
 Treatment
• 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
facility.
• 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 infant.
• 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
closed.
• 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 morechallenging. 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 beimpossible. 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.
 Inguinal Hernia

• 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. The
variable persistence of the embryonic processus vaginalis offers a
spectrum of abnormalities including a scrotal hernia, communicating
hydrocele, a hydrocele of the cord, or a simple hydrocele
• 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
be repaired.
• 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 the nursery.
• 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 monitoring. 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 inguinal exploration. If an
intestinal resection is required, it can usually be done through the opened
hernia sac prior to IH repair.
 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
direct hyperbilirubinemia.
 Investigation
• 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
be visualized.
• 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%.
 Treatment
• If the needle biopsy and/or the abdominal ultrasound are consistent
with BA, exploratory laparotomy is then performed expeditiously.
• 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
cholecystocholangiography.
• 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
portal vein.
 Choledochal Cyst
• A cystic enlargement of the common bile duct is referred to as a
choledochal cyst.
• Types
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 bile duct.
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 ducts.
In type V cysts, only the intrahepatic ducts are dilated.
• 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
the diagnosis.
• 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
portal hypertension.
• Malignant degeneration is also found in up to 16% of adults with
choledochal cysts.
• 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
making.
 Treatment
• 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
cysts.
• 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
excellent
 Neuroblastoma
• Neuroblastoma (NBL), the most common abdominal malignancy in
children.
• These tumors are of neural crest origin and, as a result, may arise
anywhere along the sympathetic ganglia or within the adrenal
medulla.
• 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.
 Clinical picture
• 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.
 Management
• 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
resectable. 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.
 Teratoma

• 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.
 Diaphragmatic Hernia,
Congenital
• 80% are left-sided
• Symptoms and signs include dyspnea, chest
retractions, decreased breath sounds on affected side
• 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