SURGICAL CONDITIONS Umbilical cord

1. The cord contains the two umbilical arteries, the vein, the rudimentary allantois, the remnant of the omphalomesenteric duct, and a gelatinous substance called Wharton jelly.

2. The sheath of the umbilical cord is derived from the amnion.3. The muscular umbilical arteries contract readily, but the vein does not. The vein retains a

fairly large lumen after birth4. The blood vessels are functionally closed, but anatomically patent for 10–20 days. The

arteries become the lateral umbilical ligaments; the vein, the ligamentum teres; and the ductus venosus, the ligamentum venosum.

5. During this interval, the umbilical vessels are potential portals of entry for infection. The umbilical cord usually sloughs within 2 wk.

6. Delayed separation of the cord, after more than 1 mo, has been associated with neutrophil chemotactic defects and overwhelming bacterial infection

Single umbilical artery:1. A single umbilical artery is present in about 5–10/1,000 births; the frequency is about 35–

70/1,000 in twin births. 2. Approximately 30% of infants with a single umbilical artery have congenital abnormalities;

usually more than one; many such infants are stillborn or die shortly after birth. 3. Trisomy 18 is one of the more frequent abnormalities. 4. The number of arteries present should be recorded as an aid to the early suspicion and

identification of abnormalities in such infants. 5. For infants with a single umbilical artery, many recommend renal ultrasonography

Congenital omphalocele:1. An omphalocele is a herniation or protrusion of the abdominal contents into the base of the

umbilical cord. 2. In contrast to the more common umbilical hernia, the sac is covered with peritoneum

without overlying skin. The size of the sac that lies outside the abdominal cavity depends on its contents.

3. Herniation of intestines into the cord occurs in about 1/5,000 births and herniation of liver and intestines in 1/10,000 births.

4. The abdominal cavity is proportionately small. 5. Immediate surgical repair, before infection has taken place and before the tissues have been

damaged by drying (saline-soaked sterile dressings should be applied immediately) or by rupture of the sac, is essential for survival. Mersilene mesh or similar synthetic material may be used to cover the viscera if the sac has ruptured or if excessive mobilization of the skin would be necessary to cover the mass and its intact sac.

6. The risk of associated congenital anomalies/syndromes, including Beckwith-Wiedemann syndrome (omphalocele, macrosomia, hypoglycemia), trisomies 13 and 18, and cardiac anomalies, is increased in patients with omphalocele

Granuloma:1. The umbilical cord usually dries and separates within 6–8 days after birth. The raw surface

becomes covered by a thin layer of skin; scar tissue forms, and the wound is usually healed within 12–15 days.

2. The presence of saprophytic organisms delays separation of the cord and increases the possibility of invasion by pathogenic organisms.

3. Mild infection or incomplete epithelialization may result in a moist granulating area at the base of the cord with a slight mucoid or mucopurulent discharge. Good results are usually obtained by cleansing with alcohol several times daily.

4. Persistence of granulation tissue at the base of the umbilicus is common. The tissue is soft, 3–10 mm in size, vascular and granular, and dull red or pink, and it may have a seropurulent secretion.

5. Treatment is cauterization with silver nitrate, repeated at intervals of several days until the base is dry.

6. Umbilical granuloma must be differentiated from umbilical polyp, a rare anomaly resulting from persistence of all or part of the omphalomesenteric duct or the urachus. The tissue of the polyp is firm and resistant, is bright red, and has a mucoid secretion. If the polyp is communicating with the ileum or bladder, small amounts of fecal material or urine may be discharged intermittently. Histologically, the polyp consists of intestinal or urinary tract mucosa. Treatment is surgical excision of the entire omphalomesenteric or urachal remnant.

Umb.sepsis (omphalitis)

1. Although aseptic delivery and routine cord care (daily application of triple dye to the umbilical stump and surrounding skin) decrease the risk of umbilical infection, the necrotic tissue of the umbilical cord is an excellent medium for bacterial growth.

2. Omphalitis may remain localized or may spread to the abdominal wall, the peritoneum, the umbilical or portal vessels, or the liver.

3. Infants with abdominal wall cellulitis or those with necrotizing fasciitis have a high incidence of associated bacteremia. Portal vein phlebitis may develop and result in the later onset of extrahepatic portal hypertension.

4. The general manifestations may be minimal (periumbilical erythema), even when septicemia or hepatitis has resulted.

5. Treatment:a. antibiotic therapy effective against Staphylococcus aureus and Escherichia coli and,b. if abscess formation has occurred, surgical incision and drainage. c. Necrotizing fasciitis is often polymicrobial and has a high mortality. d. Changes in newborn bathing practices that replace antiseptics with nonantiseptic

pH-balanced soap may be associated with increased risk of omphalitis.Umbilical hernia

1. Often associated with diastasis recti, an umbilical hernia is due to imperfect closure or weakness of the umbilical ring.

2. Predisposing factors include:a. Black race and b. Low birthweight.

3. The hernia appears as a soft swelling covered by skin that protrudes during crying, coughing, or straining and can be reduced easily through the fibrous ring at the umbilicus.

4. The hernia consists of omentum or portions of the small intestine. The size of the defect varies from <1 cm in diameter to as much as 5 cm, but large ones are rare.

5. Treatment:

a. Most umbilical hernias that appear before the age of 6 mo disappear spontaneously by 1 yr of age.

b. Even large hernias (5–6 cm in all dimensions) have been known to disappear spontaneously by 5–6 yr of age.

c. Strangulation is extremely rare. d. It is generally agreed that “strapping” is ineffective. e. Surgery is not advised unless the hernia persists to the age of 4–5 yr, causes

symptoms, becomes strangulated, or becomes progressively larger after the age of 1–2 yr.

f. Defects exceeding 2 cm are less likely to close spontaneously.

ERB’S PALSY

Erb-Duchenne paralysis:1. The injury is limited to the 5th and 6th cervical nerves. 2. The infant loses the power to abduct the arm from the shoulder, rotate the arm exter

nally, and supinate the forearm. 3. The characteristic position consists of adduction and internal rotation of the arm with

pronation of the forearm. 4. Power to extend the forearm is retained, but the biceps reflex is absent; 5. The Moro reflex is absent on the affected side. 6. The outer aspect of the arm may have some sensory impairment. 7. Power in the forearm and hand grasp is preserved unless the lower part of the plexus is

also injured; the presence of hand grasp is a favorable prognostic sign. 8. When the injury includes the phrenic nerve, alteration in diaphragmatic excursion may

be observed fluoroscopically. Klumpke paralysis:

1. It is a rare form of brachial palsy; injury to the 7th and 8th cervical nerves and the 1st thoracic nerve produces a paralyzed hand and ipsilateral ptosis and miosis (Horner syndrome) if the sympathetic fibers of the 1st thoracic root are also injured. Mild cases may not be detected immediately after birth.

2. Full recovery occurs in most patients, the prognosis depending on whether the nerve was merely injured or was lacerated. If the paralysis was due to edema and hemorrhage about the nerve fibers, function should return within a few months; if due to laceration, permanent damage may result.

3. Involvement of the deltoid is usually the most serious problem and may result in shoulder drop secondary to muscle atrophy.

4. In general, paralysis of the upper part of the arm has a better prognosis than paralysis of the lower part

Treatment:a. In upper arm paralysis, the arm should be abducted 90 degrees with external

rotation at the shoulder, full supination of the forearm, and slight extension at the wrist with the palm turned toward the face. This position may be achieved with a brace or splint during the 1st 1–2 wk.

b. In lower arm or hand paralysis, the wrist should be splinted in a neutral position and padding placed in the fist.

c. Gentle massage and range-of-motion exercises may be started by 7–10 days of age. Infants should be closely monitored with active and passive corrective exercises.

d. If the paralysis persists without improvement for 3–6 mo, neuroplasty, neurolysis, end-to-end anastomosis, and nerve grafting offer hope for partial recovery.

Vascular Ring:

I. Definition. Vascular ring denotes a variety of anomalies of the aortic arch and its branches that create a "ring" of vessels around the trachea and esophagus.

II. Pathophysiology. Partial obstruction of the trachea or the esophagus, or both, may result from extrinsic compression by theencircling ring of vessels.

I. Clinical presentation. Dysphagia or stridor (respiratory insufficiency), or both, are the modes of presentation.

II. Diagnosis is by barium swallow, which identifies extrinsic compression of the esophagus in the region of the aortic arch.

III. Management consists of surgical division of a portion of the constricting ring of vessels. The specific surgical plan must be tailored to the particular type of aortic arch anomaly present.

Esophageal Atresia

I. Definition. The esophagus ends blindly ~10-12 cm from the nares. In 85% of cases, the distal esophagus communicates with the posterior trachea (distal tracheoesophageal fistula [TEF]).

II. Pathophysiology. i. Prime morbidity is pulmonary. Complete esophageal obstruction results in

"excess salivation" and aspiration of pharyngeal contents. ii. TEF allows the crying newborn to greatly distend the stomach with air;

iii. Atelectasis iv. Aspiartion of gastric secretion directly produces chemical pneumonitis, which

may be complicated by bacterial pneumonia.III. Clinical presentation.

i. The pregnancy may have been complicated by polyhydramnios. ii. After delivery, the infant typically is unable to swallow saliva, which drains from

the corners of the mouth and requires frequent suctioning. iii. Attempts at feeding will result in prompt regurgitation, coughing, choking, and

cyanosis.IV. Diagnosis:

i. It is established by attempting to pass a nasogastric tube and meeting resistance at 10-12 cm from the nares followed by chest x-ray film for confirmation. Chest x-ray film will show the tube to end or coil in the region of the thoracic inlet. The x-ray film should also be examined for possible skeletal anomalies, pulmonary infiltrates, cardiac size and shape, and abdominal bowel gas patterns.

ii. The absence of gas in the gastrointestinal (GI) tract implies esophageal atresia without TEF (10% of cases), surgical management of which will probably differ from that of the more common esophageal atresia with TEF.

iii. Careful contrast x-ray study of the proximal esophageal pouch can also be performed to delineate the precise length of the proximal pouch and to rule out the rare proximal TEF.

V. Managementi. Frequent suctioning and by placing the baby in a relatively upright (45°) position to

lessen the likelihood of reflux of gastric contents up the distal esophagus into the trachea.

ii. Broad-spectrum antibiotics should be administered.iii. Surgical therapy. The steps and timing of surgical therapy must be individualized.

Some surgeons perform preliminary gastrostomy to decompress the stomach and provide additional protection against reflux. Ligation of the TEF and esophageal anastomosis are the essential steps in total surgical correction.

Hirschsprung's disease (congenital aganglionic megacolon).

I. Clinical presentation: infants have distended abdomens, fail to pass meconium, and vomit bilious material.

II. Diagnosis1. Abdominal x-ray studies show multiple dilated loops of intestine; the site of obstruction

(distal small bowel vs colon) cannot be determined on plain x-ray films.2. Contrast radiologic studies. The preferred diagnostic test is contrast enema. It may

identify colonic atresia, outline microcolon (which may signify complete distal small bowel obstruction), or suggest a transition zone (which may signify Hirschsprung's disease). The procedure can identify and treat meconium plug-hypoplastic left colon syndrome. If the test is normal, ileal atresia, meconium ileus, and Hirschsprung's disease are possibilities.

3. Sweat test. A sweat test may be needed to document cystic fibrosis in cases of meconium ileus (unlikely to be helpful in the first few weeks of life).

4. Mucosal rectal biopsy for histologic detection of ganglion cells is the safest and most widely available screening test for Hirschsprung's disease. However, laparotomy is often necessary to determine the exact nature of the problem in infants with normal results of barium enema.

III. Management1. Nonoperative management is "curative" in cases of meconium plug and hypoplastic left

colon.i. Passage of time and colonic stimulation by digital examination and rectal enemas

promote return of effective peristalsis.ii. In infants who achieve apparently normal bowel function, one must rule out

Hirschsprung's disease by mucosal rectal biopsy; a small percentage of patients with meconium plug will prove to have aganglionosis.

iii. uncomplicated meconium ileus, if identified, can often be treated by nonoperative means. Repeated enemas with Hypaque or acetylcysteine (Mucomyst) may disimpact the inspissated meconium in the terminal ileum and relieve the obstruction.

2. Surgical therapy. Surgical intervention is required for atresia of the ileum or colon, for complicated meconium ileus, and when the diagnosis is in doubt. Hirschsprung's disease is usually treated in the neonatal period by colostomy through ganglionic bowel. Some

surgeons are performing "one-stage" pull-through procedures, without preliminary colostomy.

Imperforate Anus

I. Definition. Imperforate anus is the lack of an anal opening of proper location or size. There are two types: high and low.

a. High imperforate anus. The rectum ends above the puborectalis sling, the main muscleresponsible for maintaining fecal continence. There is no fistula to the perineum. In males, there may be a rectourinary fistula, and in females, a rectovaginal fistula.

b. Low imperforate anus. The rectum has traversed the puborectalis sling in the correct position. Variants include anal stenosis, imperforate anus with perineal fistula, and imperforate anus without fistula.

II. Diagnosis:a. By inspection of any perineal opening. b. X-ray studies of the lumbosacral spine and urinary tract because there is a high

incidence of dysmorphism in these areas.III. Management.

a. Surgical therapy in the neonate consists of colostomy for high anomalies andperineal anoplasty or dilation of fistula for low lesions.

Choanal Atresia

I. Definition. Choanal atresia is a congenital blockage of the posterior nares caused by persistence of a bony septum (90%) or a soft tissue membrane (10%).

II. Pathophysiology. Choanal atresia, which is complete and bilateral, is one of the causes of respiratory distress immediately after delivery. The effects of upper airway obstruction are compounded because neonates are obligate nose-breathers and will not "think" to breathe through the mouth.

III. Clinical presentation. Respiratory distress resulting from partial or total upper airway obstruction is the mode of presentation.

IV. Diagnosis is based on an inability to pass a catheter into the nasopharynx via either side of the nose.

V. Management. Simply making the infant cry and thereby breathe through the mouth will temporarily improve breathing. Insertion of an oral airway will maintain the ability to breathe until the atresia is surgically corrected.

Pierre Robin Syndrome

I. Definition. This anomaly consists of mandibular hypoplasia in association with cleft palate.II. Pathophysiology. Airway obstruction is produced by posterior displacement of the tongue

associated with the small size of the mandible.III. Clinical presentation. Severity of symptoms varies, but most infants manifest a high degree of

partial upper airway obstruction.IV. Management

1. Infants with mild involvement can be cared for in the prone position and fed through a special Breck nipple. Adjustment to the airway compromise will occur over days or weeks.

2. More severe cases require nasopharyngeal tubes or surgical procedures to hold the tongue in an anterior position. Tracheostomy is generally a last resort.

Congenital Lobar Emphysema

1) Definition. Lobar emphysema is a term used to denote hyperexpansion of the air spaces of a segment or lobe of the lung.

2) Pathophysiology. Inspired air is trapped in an enclosed space. As the cyst of entrapped air enlarges, the normal lung is increasingly compressed. Cystic problems are more common in the upper lobes.

3) Clinical presentation. Small cysts may cause few or no symptoms and are readily seen on x-ray film. Giant cysts may cause significant respiratory distress, with mediastinal shift and compromise of the contralateral lung.

4) Diagnosis. Usually, the cysts are easily seen on plain chest x-ray films. However, the radiologic findings may be confused with those of tension pneumothorax.

5) Management. Therapeutic options include observation for small asymptomatic cysts, repositioning of the endotracheal tube to selectively ventilate the uninvolved lung for 6-12 h, bronchoscopy for endobronchial lavage, and operative resection of the cyst with or without the segment or lobe from which it arises.

Cleft Lip and Palate:

1. Introduction:1. Cleft of the lip appears because of hypoplasia of the mesenchymal layer, resulting in

a failure of the medial nasal and maxillary processes to join. 2. Cleft of the palate appears to represent failure of the palatal shelves to approximate

or fuse.2. INCIDENCE AND EPIDEMIOLOGY:

1. The incidence of cleft lip with or without cleft palate is ≈1/750 births; the incidence of cleft palate alone is ≈1/2,500 births.

2. Clefts of the lip are more common in males. 3. Possible causes include maternal drug exposure, a syndrome-malformation complex,

or genetic factors. 4. Associated congenital malformations: chromosomal aneuploidy, holoprosencephaly

3. CLINICAL MANIFESTATIONS: 1. Clefts may be unilateral (more often on the left side) or bilateral and may involve the

alveolar ridge. Deformed, supernumerary, or absent teeth are associated findings.2. Isolated cleft palate occurs in the midline and may involve only the uvula or may

extend into or through the soft and hard palates. 3. When associated with cleft lip, the defect may involve the midline of the soft palate

and extend into the hard palate on one or both sides 4. SEQUELAE OF CLEFT LIP AND PALATE.

1. Recurrent otitis media and hearing loss are frequent with cleft palate. 2. Displacement of the maxillary arches and malposition of the teeth 3. Speech defects

5. TREATMENT. 1. Feeding:

1) construction of a plastic obturator to assist in feedings

2) use of soft artificial nipples with large opening and a squeezable bottle 2. Surgical closure:

1) cleft lip:1) is usually performed by 3 mo of age, when the infant has shown

satisfactory weight gain and is 2) Should be free of any oral, respiratory, or systemic infection. 3) Modification of the Millard rotation-advancement technique is the

most commonly used technique; 4) The initial repair may be revised at 4 or 5 yr of age.

2) Closure of the palate is usually done before 1 yr of age to enhance normal speech development.

POSTERIOR URETHRAL VALVES

1. The most common cause of severe obstructive uropathy in children is posterior urethral valves, affecting 1 in 8,000 boys.

2. The urethral valves are tissue leaflets fanning distally from the prostatic urethra to the external urinary sphincter. A slit-like opening usually separates the leaflets.

3. Valves are of unclear embryologic origin and cause varying degrees of obstruction. 4. Vesicoureteral reflux occurs in 50% of patients, and distal ureteral obstruction may result

from a chronically distended bladder or bladder muscle hypertrophy. 5. The renal changes range from mild hydronephrosis to severe renal dysplasia; their severity

probably depends on the severity of the obstruction and its time of onset during fetal development.

6. There may be oligohydramnios and pulmonary hypoplasia7. Affected boys with posterior urethral valves are discovered prenatally when maternal

ultrasonography reveals bilateral hydronephrosis, a distended bladder, and, if the obstruction is severe, oligohydramnios.

8. Prenatally diagnosed posterior urethral valves, particularly when discovered in the second trimester, carry a poorer prognosis than those detected after birth.

9. In the male neonate, posterior urethral valves are suspected when there is a palpably distended bladder and the urinary stream is weak. If the obstruction is severe and goes unrecognized during the neonatal period, infants may present later in life with failure to thrive due to uremia or sepsis caused by infection in the obstructed urinary tract.

10. With lesser degrees of obstruction, children present later in life with difficulty in achieving diurnal urinary continence or with UTI. The diagnosis is established with a Voiding Cysto Urethogram or by perineal ultrasonography

11. Treatment consists of transurethral ablation of the valve leaflets, which is performed endoscopically under general anesthesia. If the urethra is too small for transurethral ablation, temporary vesicostomy is preferred, in which the dome of the bladder is exteriorized on the lower abdominal wall. When the child is older, the valves may be ablated and the vesicostomy closed

Diaphragmatic Hernia

1) Definition: a communication between the abdominal and thoracic cavities with or without abdominal contents in the thorax.

2) Anatomy:

a. The term congenital diaphragmatic hernia (CDH) typically refers to the Bochdalek form.

b. The Bochdalek hernia accounts for up to 90% of the hernias seen in the newborn period, with 80–90% occurring on the left side.

c. The Morgagni hernia accounts for 2–6% of congenital diaphragmatic defects. d. The size of the defect is highly variable, ranging from a small hole to complete

agenesis of this area of the diaphragm. 3) Embryology, pathology:

1. CDH may be due to defective formation of the pleuroperitoneal membrane. 2. When the abdominal contents return to the abdomen from the umbilical sac at 10 wk

gestation, herniation of the abdominal contents may occur3. In CDH, a major limiting factor for survival is the associated pulmonary hypoplasia

Aetiology:1. The incidence of CDH is 1/5,000 live births, with females affected twice as often as males. 2. Defects are more common on the left (85%) and are occasionally (<5%) bilateral. 3. Pulmonary hypoplasia and malrotation of the intestine are part of the lesion, not associated

anomalies. 4. Most cases of CDH are sporadic, but familial cases have been reported. 5. Associated anomalies include central nervous system lesions, esophageal atresia,

omphalocele, and cardiovascular lesions. 6. CDH is recognized as part of several chromosomal syndromes: trisomy 21, trisomy 13,

trisomy 18, Fryn, Brachmann–de Lange, Pallister-Killian, and Turner.Clinical presentation:

1. Respiratory distress may occur immediately or there may be a “honeymoon” period of up to 48 hr when the baby is relatively stable. Early respiratory distress within 6 hr of life is thought to be a poor prognostic sign.

2. The clinical signs of respiratory distress are characterized by tachypnea, grunting, use of accessory muscles, and cyanosis.

3. Children with CDH will also have a scaphoid abdomen and increased chest wall diameter. 4. Bowel sounds may also be heard in the chest with decreased breath sounds bilaterally. 5. The point of maximal cardiac impulse may be displaced away from the side of the hernia 6. Unrecognized diaphragmatic hernia is a rare cause of sudden death in infants and toddlers

Investigations:1. CDH can be diagnosed on prenatal ultrasound (between 16 and 24 wk) in over 50% of cases.

Findings on ultrasound may include polyhydramnios, chest mass, mediastinal shift, gastric bubble or a liver in the thoracic cavity, and fetal hydrops.

2. Chest radiograph: Bowel loops are seen chest cavity with empty abdomen and mediastinal shift

D.D:1. pulmonary sequestration, 2. cystic adenomatoid malformation

Treatment:1. Initial management:

a. Aggressive respiratory support including rapid endotracheal intubation b. Gentle ventilation with permissive hypercapnea reduces lung injury and mortality.

c. Babies with CDH may be surfactant deficient. 2. Surgical repair:

a. Most centers will wait at least 48 hr after stabilization and resolution of the pulmonary hypertension.

b. A subcostal approach is the most frequently used; primary repair using native tissue or a Gore-Tex patch

c. Both laparoscopic and thoracoscopic repairs for stable infants.d. Overall survival of live-born infants is 67%

3. Longterm complications:a. Pulmonary function changes, b. Neurodevelopmental delays, c. growth retardationd. Bronchopulmonary dysplasiae. Gastroesophageal reflux disease (GERD)f. Pectus excavatum and scoliosisg. Defects due to oxygen toxicity

Congenital Hypertrophic Pyloric Stenosis

Introduction:1. Hypertrophic pyloric stenosis occurs in 1per 1,000 infants. 2. Males (especially first-borns) are affected approximately four times mre commonly. 3. Pyloric stenosis develops in approximately 20% of the male and 10% of the female

descendants of a mother who had pyloric stenosis. 4. The incidence of pyloric stenosis is increased in infants with type B and O blood groups. 5. Pyloric stenosis is associated with other congenital defects, including tracheoesophageal

fistula and hypoplasia or agenesis of the inferior labial frenulum.Etiology:

1. The cause of pyloric stenosis is unknown and probably develops only after birth. Abnormal muscle innervation, elevated serum levels of prostaglandins, reduced levels of pyloric nitric oxide synthase and infant hypergastrinemia have been implicated.

2. Pyloric stenosis has been associated with eosinophilic gastroenteritis, Apert syndrome, Zellweger syndrome, trisomy 18, Smith-Lemli-Opitz syndrome, and Cornelia de Lange syndrome.

3. A variable association has been found with the use of erythromycin in neonates Clinical Manifestation:

1. Nonbilious vomiting is the initial symptom of pyloric stenosis. 2. The vomiting usually starts after 3 wk of age; projectile occurring immediately after a

feeding; after vomiting, the infant is hungry and wants to feed again. 3. Progressive loss of fluid, hydrogen ion, and chloride leads to hypochloremic metabolic

alkalosis. 4. There may be a total body potassium deficit. 5. Jaundice associated with a decreased level of glucuronyl transferase is seen in ≈5% .

Diagnosis:1. The diagnosis has traditionally been established by palpating the pyloric mass.

2. The mass is firm, movable, ≈2 cm in length, olive shaped, hard, best palpated from the left side, and located above and to the right of the umbilicus in the mid epigastrium beneath the liver edge.

3. After feeding, there may be a visible gastric peristaltic wave that progresses across the abdomen. After the infant vomits, the abdominal musculature is more relaxed and the “olive” easier to palpate.

4. Ultrasound examination confirms the diagnosis in the majority of cases and allows an earlier diagnosis. Findings include pyloric thickness >4 mm or an overall pyloric length >14 mm.

5. contrast studies: elongated pyloric channel, a bulge of the pyloric muscle into the antrum (shoulder sign), and parallel streaks of barium seen in the narrowed channel, producing a “double tract sign”

D.D:1. Infants of inadequate maternal-infant bonding 2. gastroesophageal reflux, 3. Adrenal insufficiency 4. Inborn errors of metabolism 5. gastroenteritis, 6. pyloric membrane or pyloric duplication 7. Duodenal stenosis

Treatment:1. Rehydration:

a. Correction fluid, acid-base, and electrolyte losses. b. Bicarbonate concentration should be <30 mEq/dL, which implies that the alkalosis

has been corrected to prevent postoperative apnea2. Surgery:

a. The surgical procedure of choice is pyloromyotomy. The traditional Ramstedt procedure is performed through a short transverse skin incision. The underlying pyloric mass is split without cutting the mucosa, and the incision is closed. Laparoscopic technique is equally successful.

3. Conservative:a. Conservative medical therapy consisting of small frequent feedings and atropine has

been successful in small groups of patients. b. Endoscopic balloon dilation has been successful in infants with persistent vomiting

secondary to incomplete pyloromyotomyIntussusception

Introduction:1. Intussusception is the most frequent cause of intestinal obstruction in the first 2 years of life. 2. It is three times more common in males than in females.

Etiology:1. In 85% of cases the cause is not apparent. Small bowel polyp, Meckel diverticulum,

omphalomesenteric remnant, duplication, Henoch-Schönlein purpura, lymphoma, lipoma, parasites, foreign bodies, and viral enteritis with hypertrophy of Peyer patches all have been implicated as causes.

2. Rotavirus vaccines were once implicated

3. Intussusception of the small intestine occurs in patients with celiac disease (CD) and cystic fibrosis related to the bulk of stool in the terminal ileum.

4. In children older than 6 years, lymphoma is the most common cause. Pathophysiology:

1. The intussusception starts just proximal to the ileocecal valve and extends for varying distances into the colon.

2. The terminal ileum telescopes into the colon. 3. Swelling, hemorrhage, incarceration, arterial and venous compromise, and necrosis of the

intussuscepted ileum may occur, as well as intestinal perforation and peritonitis.Clinical Findings

1. Recurring paroxysms of abdominal pain with screaming and drawing up of the knees. 2. Vomiting and diarrhea followed by bloody stools 3. The abdomen is tender and distended. 4. A sausage-shaped mass may be palpated in the upper mid abdomen.

Treatment1. Initially barium enema and air enema are both diagnostic and therapeutic. 2. Air insufflation of the colon under fluoroscopic guidance is a safe alternative to barium

enema that has excellent diagnostic sensitivity and specificity without the risk of contaminating the abdominal cavity with barium.

3. Care is required in performing either air or barium enema because ischemic damage to the colon secondary to vascular compromise increases the risk of perforation.

Surgery:1. Surgery is required in extremely ill patients, in patients with evidence of bowel perforation,

or in those in whom hydrostatic or pneumatic reduction has been unsuccessful. Prognosis

1. The mortality rate with treatment is 1–2%. 2. hydrostatic or pneumatic reduction may recur within 24 hours in 3–4% of patients. 3. Patients older than 5 years are more likely to have persistent symptoms, an underlying lead

point and recurrent intussusception after reduction

Dental Caries

Etiology:1. The development of dental caries depends on interrelationships between the tooth surface,

dietary carbohydrates, and specific oral bacteria. 2. Organic acids produced by bacterial fermentation of dietary carbohydrates reduce the pH of

dental plaque adjacent to the tooth to a point at which demineralization occurs. 3. The initial carious lesion appears as an opaque white spot on the enamel; and with

progressive loss of tooth mineral, cavitation occurs.4. A group of microorganisms, mutans streptococci, are associated with the development of

dental caries. These bacteria have the ability to adhere to enamel, produce abundant acid, and survive at low pH. Once the enamel surface cavitates, other oral bacteria (lactobacilli) colonize the tooth, produce acid, and foster further tooth demineralization.

Epidemiology:

1. The incidence of dental caries has decreased in developed countries in the past 30 yr but remains highly prevalent among low-income children and children from developing countries.

Clinical manifestations:1. Larger lesions present as cavitations of the occlusal surface. 2. Caries lesions of the exposed smooth (buccal and lingual) surfaces are generally found only

in children with rampant caries3. If left untreated, dental caries invade the dental pulp, leading to an inflammation of the pulp

(pulpitis) and significant pain. 4. Pulpitis can progress to dental abscess. 5. Infection of a primary tooth may disrupt normal development of permanent tooth. 6. In a small percentage of cases, this process may lead to sepsis and facial space infection.

Treatment:1. If caries involves the dental pulp, a partial removal of the pulp (pulpotomy) or complete

removal of the pulp (pulpectomy) may be required. 2. If a tooth requires extraction, a space maintainer may be indicated to prevent migration of

teeth, which subsequently leads to malposition of permanent successor teeth3. Oral antibiotics are indicated for dental infections associated with cellulitis, facial swelling.

Penicillin is the antibiotic of choice, except in patients with a history of allergy to this agent; clindamycin and erythromycin are suitable alternatives

Prevention:1. optimizing the fluoride content of communal water supplies to 1 ppm. Children who reside

in areas with fluoride-deficient water supplies and are at risk for caries will benefit from dietary fluoride supplements.

2. Daily brushing, especially with fluoridated toothpaste, will help prevent dental caries. 3. Decreasing frequent sugar ingestion prevents dental caries. 4. Plastic dental sealants have been shown to be effective in the prevention of caries on the pit

and fissure of the primary and permanent molars. Sealants are most effective when placed soon after teeth erupt.

Acute otitis media

Classification of otitis: 1. Acute otitis media- an acute infection2. Otitis media with effusion- an acute inflammation: nonsuppurative or secretory otitis media 3. Chronic suppurative otitis media4. Recurrent otitis media: 3 or more episodes of AOM in 6 months or 4 episodes in 1 year.

Epidemiology1. Peak incidence in the first two years of life (esp. 6-12 months)2. Boys more affected girls3. 90% of children will have at least one infection by age 6.

Acute Otitis Media- definition1. History of acute onset (<48 hours) of signs and symptoms, 2. The presence of Middle Ear Effusion as indicated by:

A. Bulging of the tympanic membrane B. Limited or absent mobility of the tympanic membrane C. Air-fluid level behind the tympanic membrane

Predisposing Factors1. Frequent upper respiratory infections, 2. Exposure to parental cigarette smoke, 3. Unfavorable eustachian tube function, 4. Immunocompromised children ; 5. Down syndrome6. Bottle Feeding7. Cleft palate8. Genetic Susceptibility9. Allergies.10. Day-care facilities11. Use of a pacifier

Pathogenesis:Viral infectionà eustachian tube dysfunction à middle-ear space do not drain normallyà a

vacuum develops in the middle ear à pull serous fluid from the middle ear lining cells à OME à pathogenic respiratory bacteria reflux into the middle-ear à formation of an abscess in the middle earà AOM à resolve spontaneously within a few days, middle-ear effusions can persist for weeks Microbiology of Acute Otitis Media:

1. Viral: 40% : respiratory syncytial virus, rhinovirus, coronavirus, parainfluenza, adenovirus, enterovirus and influenza virus

2. S pneumoniae: 25% to 50% 3. H influenzae: 15% to 30%, and 4. M catarrhalis: 3% to 20%.

5. Group A β-hemolytic streptococcus and Staphylococcus aureus are much less common

Clinical manifestations:1. Generalized discomfort, and unexplained crying or irritability. 2. Ear pain, often manifested by irritability, a change in sleeping or eating habits, and,

occasionally, holding or tugging at the ear3. Pulling at the ear, however, has a low sensitivity and specificity. 4. Fever also may be present and, rarely, rupture of the tympanic membrane with

purulent otorrhea. 5. Dysequilibrium also can be associated with OME

DIAGNOSIS1. acute onset of a new fever and ear discomfort2. Presence of MEE; 3. Signs and symptoms of middle-ear inflammation, including erythema of the tympanic

mbrane or otalgia 4. Tympanic membrane in OM:

• Erythema is non specific• Bulging• Amber, pale yellow, or (rarely) bluish color • Restricted movement • Membrane may be retracted

Differential Diagnoses of Acute Otalgia

Common1. Foreign body in the external ear canal2. Otitis externa 3. CSOM

Less Common1. Accidental trauma (e.g., perforation of TM)2. Cholesteatoma

Rare1. Mastoiditis2. Herpes zoster oticus (Ramsey Hunt syndrome)

MANAGEMENT OF ACUTE OTITIS MEDIA Guidelines

1. The diagnosis of AOM should be clear2. < 6 mo; immuonocompromised require immediate antibiotics3. Observation of a patient for 48 to 72 hours for low-risk patients as allowance for viral

etiology - Watchful Waiting4. Antibiotic with the narrowest spectrum 5. High-dose amoxicillin (90 mg /kg) to get middle ear concentration for 10 days is the

first-line choice 6. Second-line agents: second- or third-generation cephalosporins or amoxicillin plus

clavulanate 7. Azithromycin for penicillin sensitive patients

Pain Management1. acetaminophen or ibuprofen, accompanied by a topical anesthetic drop if the TM is

not perforated2. Auralgan (benzocaine and antipyrine) ear drops found to be superior3. No proven benefit of antihistamine or decongestant preparations & steroids

Complications of AOM 1. Permanent conductive hearing loss 2. Impaired speech and language development 3. Mastoiditis, 4. Intracranial extension of infection 5. Facial nerve paralysis6. Labyrinthitis 7. Infectious dermatitis 8. CSOM

INTRACRANIAL COMPLICATIONS of AOM1. Lateral sinus thrombosis.2. Meningitis, 3. Epidural abscess, 4. Subdural abscess, 5. Focal encephalitis, 6. Brain abscess, 7. Otitic hydrocephalus (pseudotumor cerebri) due to obstruction by thrombus of

intracranial venous drainage into the neck

PREVENTION 1. Breast-milk feeding; 2. Avoidance, of exposure to individuals with respiratory infection; 3. Avoidance of environmental tobacco smoke; 4. Pneumococcal vaccination.

OTITIS MEDIA WITH EFFUSION (OME):

1. Asymptomatic presence of middle-ear effusion 2. Often associated with conductive hearing loss 3. Can persist for months.

Etiology:1. Post AO, Cytokines and other inflammatory mediators.2. An alternative theory suggests eustachian tube obstruction, negative middle-ear

pressure, and transudation of fluid.3. OME may resolve without specific intervention within a few months

Management:1. Trial course of antibiotics sometimes resolve OME 2. Ventilating tubes: if

1. the effusion has persisted for 4 months 2. bilateral hearing impairment of 20 dB or greater

3. Laser-assisted myringotomy 4. Adenoidectomy

Complications of OME1. Cholesteatoma, 2. Adhesive otitis, 3. Retraction pockets,4. Membrane atrophy, and 5. Persistent membrane perforation.

Chronic Suppurative Otitis Media

Chronic suppurative otitis media (CSOM) is a perforated tympanic membrane with persistent drainage from the middle ear. Definition:CSOM is defined as chronic otorrhea (ie, lasting >6-12 wk) through a perforated tympanic membrane.Chronic suppuration can occur with or without cholesteatoma, and the clinical history of both conditions can be very similar. Predisposing factors:1. Patients with craniofacial anomalies are special populations at risk for CSOM.2. Cleft palate, Down syndrome, cri du chat syndrome, choanal atresia, cleft lip, and microcephaly

are at risk for CSOM, presumably from altered eustachian tube anatomy and function.Common bacteria

1. Pseudomonas aeruginosa, 2. Staphylococcus aureus, 3. Proteusspecies,

4. Klebsiella pneumoniae, 5. diphtheroids 6. anaerobes

Clinical features:1. Patients present with a draining ear of some duration and a premorbid history of recurrent

acute otitis media, traumatic perforation, or the placement of ventilation tubes. 2. Typically, they deny pain or discomfort. 3. A common presenting symptom is hearing loss in the affected ear. 4. Reports of fever, vertigo, and pain should raise concern about intratemporal or intracranial

complications. 5. A history of persistent CSOM after appropriate medical treatment should alert the physician to

consider cholesteatoma.Complications of DiseaseIntratemporal and intracranial.Intratemporal complications include:

1. petrositis, 2. facial paralysis, and 3. labyrinthitis.

Intracranial complications include:1. lateral sinus thrombophlebitis, 2. meningitis, and 3. intracranial abscess.

Sequelae include:1. Hearing loss, 2. Acquired cholesteatoma, and 3. Tympanosclerosis.

Management:1. Patients with chronic suppurative otitis media (CSOM) respond more frequently to topical

therapy than to systemic therapy. Successful topical therapy consists of 3 important components: selection of an appropriate antibiotic drop, regular aggressive aural toilet, and control of granulation tissue

2. Systemic therapy should be reserved for cases of CSOM that fail to respond to topical therapy. Prior to instituting systemic therapy, a culture should be obtained for sensitivity.

3. Ciprofloxacin remains the most effective of the quinolonesfor 14 days for pseudomonads. Aminoglycosides and cephelexins are also useful.

I.Subdural effusion

I. Collections of fluid in the subdural space develop in 10-30% of patients with meningitis and are asymptomatic in 85-90% of patients.

II. Subdural effusions are especially common in infants. III. Symptomatic subdural effusions may result in:

1. A bulging fontanel, 2. Diastasis of sutures,

3. Enlarging head circumference, 4. Emesis, 5. Seizures, 6. Fever, and 7. Abnormal cranial transillumination.

IV. CT or MRI scanning confirms the presence of a subdural effusion. V. In the presence of increased ICP or a depressed level of consciousness, symptomatic

subdural effusion should be treated by aspiration through the open fontanel. Fever alone is not an indication for aspiration

PEDIATRIC CANCERSNEUROBLASTOMA

Introduction:1. Neuroblastoma (NB) is an embryonal cancer of the peripheral sympathetic nervous system 2. The clinical spectrum of NB varies, ranging from spontaneous regression in some types to

very aggressive tumors in others. Pathogenesis:

1. Mutation, prenatally and perinatally, that may be caused by environmental and genetic factors.

2. Maternal and paternal occupational chemical exposures, work in farming, and work related to electronics.

Clinical:1. Tumor may develop at any site of sympathetic nervous system tissue. 2. Most cases arise in the abdomen, either in the adrenal gland or in retroperitoneal

sympathetic ganglia. 3. Usually a firm, nodular mass that is palpable in the flank or midline is causing abdominal

discomfort. Investigations:

1. On plain radiography or CT the mass often contains calcification and hemorrhage. Wilms tumor, another common flank mass in a young child, usually does not calcify.

2. NB originates from cervical, thoracic, or pelvic ganglia in 30% of cases.3. NB usually is discovered as a mass or multiple masses on plain radiographs, CT, or MRI. 4. Tumor markers, including homovanillic acid (HVA) and vanillylmandelic acid (VMA) in urine,

are elevated in 95% of cases and help to confirm the diagnosis. 5. A pathologic diagnosis is established from tumor tissue obtained by biopsy.

Treatment:1. The usual treatment for low-risk NB is surgery for stages 1 and 2 and observation for stage

4S. 2. Children with spinal cord compression at diagnosis also may require urgent treatment with

chemotherapy, surgery, or radiation to avoid neurologic damage. 3. Stage 4S has a very favorable prognosis, with nearly 100% survival with supportive care only,

because the tumor regresses spontaneously.

Wilms tumor (nephroblastoma)

1. Wilms tumor, also known as nephroblastoma, is a complex mixed embryonal neoplasm of the kidney composed of three elements: blastema, epithelia, and stroma.

2. Most cases are sporadic, although 1–2% of patients have a family history (autosomal dominant)

Syndromes associated with Wils tumor:1. WAGR syndrome: is a contiguous gene deletion syndrome that consists of Wilms tumor,

aniridia, genitourinary abnormalities (cryptorchidism, streak ovaries, bicornate uterus, ambiguous genitalia), and mental retardation.

2. Denys-Drash syndrome is characterized by male pseudohermaphrodism, early-onset renal failure characterized by mesangial sclerosis, and an increased risk of Wilms tumor.

3. Beckwith-Wiedemann syndrome is characterized by hemihypertrophy, macroglossia, and visceromegaly, with a 3–5% risk of developing Wilms tumor.

Clinical:1. Two broad categories of Wilms tumor have been recognized: favorable and unfavorable. 2. Wilms tumor usually presents as an abdominal mass. It generally is discovered fortuitously,

and it is not uncommon for the parent to notice it while bathing the infant.3. It also may be identified during a well child examination. 4. These renal masses vary in size, usually are smooth and firm, and occasionally may cross the

midline. 5. Some patients may present with abdominal pain and vomiting, and hematuria is seen in 12–

25% of patients. 6. Hypertension also has been described and probably is due to renal ischemia.

Management:1. Management of Wilms tumor include the use of preoperative chemotherapy and radiation

therapy 2. Surgical extirpation of the tumor should be performed

Differential diagnosis of tumors:

TUMOR AGE CLINICAL SIGNS LABORATORY FINDINGS

Wilms Preschool Unilateral flank mass, aniridia, hemihypertrophy

Hematuria;bone scintigraphy (clear cell sarcoma)

Neuroblastoma Preschool GI/GU obstruction, raccoon eyes, myoclonus-opsoclonus, diarrhea, skin nodules (infants)

Increased VMA;increased HVA;increased ferritin; stippled calcification in mass.Bone marrow positive

Non-Hodgkin lymphoma >1 yr Intussusception in >2-yr-old Increased urate; bone marrow positive

Hepatoblastoma Birth–3 yr Large, firm liver Increased AFP

Hepatoma School age, teens

Large, firm liver; hepatitis B, cirrhosis

Increased AFP

BRAIN TUMORS

Primary central nervous system (CNS) tumors are a heterogeneous group of diseases that are, collectively, the second most frequent malignancy in childhood and adolescence.

Distribution of Childhood Brain Tumors Based on Histology

HISTOLOGY DISTRIBUTION (% OF TOTAL)

Medulloblastoma/primitive neuroectodermal tumor 20

HISTOLOGY DISTRIBUTION (% OF TOTAL)

Juvenile pilocytic astrocytoma 20

Low-grade astrocytoma 15

High-grade astrocytoma 7

Ependymoma 7

Craniopharyngioma 7

Unclassified primary tumors 7

Choroid plexus tumors, germ cell tumors, oligodendroglioma, meningioma, mixed tumors, pineal parenchymal tumors

1–2 (each histologic group)

The clinical presentation:1. Depends on the tumor location, the tumor type, and the age of the child. 2. Signs and symptoms are related to:

a. Obstruction of cerebrospinal fluid b. Increased intracranial pressure (icp) c. Focal brain dysfunction.

3. Supratentorial (cortical) lesions:a. Subtle changes in personality, mentation, and speech b. signs of increased ICP such as vomiting, lethargy, and irritability, as well as the later

finding of macrocephaly. c. focal disorders such as motor weaknesses, sensory changes, speech disorders,

seizures, and reflex abnormalities. d. Infants with supratentorial tumors may present with hand preference.

4. Infr tentorial tumors:a. The classic triad of headache, nausea and vomiting, and papilledema is associated

with midline or infratentorial tumors. b. Disorders of equilibrium, gait, and coordination occur with infratentorial tumors. c. Torticollis may result in cerebellar tonsil herniation. d. Blurred vision, diplopia, and nystagmus also are associated with infratentorial

tumors.e. Tumors of the brainstem region may be associated with gaze palsy, multiple cranial

nerve palsies, and upper motor neuron deficits (e.g., hemiparesis, hyperreflexia, clonus).

5. Optic pathway tumors:a. Present with visual disturbances such as decreased visual acuity, Marcus Gunn

pupil (afferent papillary defect), nystagmus, and/or visual field defects. 6. Suprasellar region tumors and third ventricular region tumors:

a. May present initially with neuroendocrine deficits such as diabetes insipidus, galactorrhea, precocious puberty, delayed puberty, and hypothyroidism.

b. The diencephalic syndrome presents with failure to thrive, emaciation, increased appetite, and euphoric affect, and occurs in infants and young children with tumors in these regions.

7. Parinaud syndrome presents with pineal region tumors and is manifested by paresis of upward gaze, pupillary dilation reactive to accommodation but not to light, nystagmus to convergence or retraction, and eyelid retraction.

8. Spinal cord tumors and spinal cord dissemination of brain tumors may manifest as long nerve tract motor and/or sensory deficits, bowel and bladder deficits, and back or radicular pain.

9. The signs and symptoms of meningeal metastatic disease from brain tumors or leukemia are similar to those of infratentorial tumors.

DIAGNOSIS.1. The evaluation of a patient suspected of having a brain tumor is an emergency. 2. Initial evaluation should include a complete history, physical (including ophthalmic)

examination, and neurologic assessment with neuroimaging. 3. For primary brain tumors, MRI is the neuroimaging standard. Tumors in the

pituitary/suprasellar region, optic path, and infratentorium are better delineated with MRI than with CT.

4. Patients with tumors of the midline and the pituitary/suprasellar/optic chiasmal region should undergo evaluation for neuroendocrine dysfunction.

5. Formal ophthalmologic examination is beneficial in patients with optic path region tumors to document the impact of the disease on oculomotor function, visual acuity, and fields of vision.

6. The suprasellar region and pineal region are preferential sites for germ cell tumors. 7. Both serum and CSF measurements of β-human chorionic gonadotropin and α-fetoprotein

can assist in the diagnosis of germ cell tumors. 8. In tumors with a propensity for spreading to the leptomeninges, such as

medulloblastoma/PNET, ependymoma, and germ cell tumors, lumbar puncture and cytologic analysis of the CSF is indicated;

Lumbar puncture is contraindicated in individuals with newly diagnosed hydrocephalus secondary to CSF flow obstruction or in individuals with infratentorial tumors. Lumbar puncture in these

individuals may lead to brain herniation, resulting in neurologic compromise and death. Therefore, in children with newly diagnosed intracranial tumors and signs of increased ICP, the lumbar puncture

usually is delayed until surgery or shunt placement.