Pediatric ABSITE Questions

Pediatrics

Neuroblastoma Third Most common Pediatric malignancy tumor of neural crest cells - found in adrenal medulla Mets to skin = blueberry muffin lesions Dx - vanillymandelic acid and homovanillic acid urine levels Treatment - resection with chemotherapy however some can spontaneously regress!   Nephroblastoma (Wilms Tumor) Second most common pediatric tumor Associated with Beckwith-Wiedemann (macrogloassia, hypoglycemia, gigantism, visceromegaly) and WAGR (wilms, aniridia, genitourinary abnormalities) Treatment = nephrectomy Thyroglossal Duct Cyst midline anterior neck mass that moves up and down with tongue movement remnant of base of tongue to pyramidal lobe of thyroid Treatment = Sistrunk procedure (with removal of central portion of hyoid bone) Branchial cleft cyst 1st - connect with external auditory canal, associated with facial nerve 2nd - MOST COMMON - through carotid bifucation into tonsillar pillar 3rd - lateral neck   Cystic Hygroma obstructed lymph vessels lead to mass usually in neck and can cause obstruction Can often get infected treatment - urgent aspiration if airway compromise then serial resections Esophageal Atresia (EA)/ Tracheoesophageal Fistula (TE) Type A: EA without TE Type B: EA with TE fistula b/w proximal esophogeal stump and trachea Type C: EA with TE fistula b/w distal esophageal stump and trachea (MOST COMMON) Type D: EA with both proximal and distal TE Type E: TE without EA (aka “H” type) Diagnosis: prenatal polyhydraminous EA: inability to pass NG tube. TE: air in distal GI tract Most (30 – 50 percent) have associated abnormalities, 20% cardiac malforation MUST get echo to see if aortic arch is on the right and not left to plan which thoractomy to do Tx: right thoracotomy and end to end anastomosis and closure of trachea Complications: REFLUX - sometimes requires an anti-reflux procedure  Pulmonary Sequestration intralobar (pulmonary venous drainage, older child), extralobar (systemic drainage, infants) Congential lobar emphysema failure of cartialge developement leads to hyperinflation Bronchoscopy will worsen condition Urgent resection (lobectomy) may be needed to avoid pulmonary or cardiac compression Congential Cystic adenoid malformation cyst in airway leading to recurrent infection or respiratory compromise Bronchiogenic cyst extrapulmonary cysts that compress structures - resect cyst Pyloric Stenosis Nonbilious projectile vomiting, generally in children ages 4 to 8 weeks HYPOKALEMIC, HYPOCHLOREMIC, METABOLIC ALKALOSIS with PARADOXICAL ACIDURIA     Renal Tubules resorbes sodium for volume loss in exchange for potassium     As potassium levels decrease secondary to gastric content, potassium is resorbed in exchange for hydrogen and thus urine becomes acidic Diagnosis: >4mm thick or >14mm long Treatment: Resuscitate; pyloromyotomy   Prune-Belly Syndrome Abdominal wall defect associated with dilated urinary tract and bilateral undescended testes that will require bilateral orchipexy Treatment - abdominal wall reconstruction Duodenal Atresia Failure of recanalization Diagnosis: Double bubble sign on x-ray; GI series shows duodenum past midline Treatment: Duodenoduodenostomy versus duodenotomy (for webs / windsock deformity - Do not resect for risk of injury of ampulla and pancreatic duct Associated with Down’s Syndrome   Intestinal Atresia (Jejunal / Ileal)Intrauterine vascular accident; not failure of recanalization Type I: intraluminal diaphragm or web Type II: Fibrous cord connecting two ends; mesentery intact Type IIIa: Discontinuous bowel; V-shaped mesenteric defect Type IIIb: apple core deformity Type IV: multiple atretic segments Treatment: Excision; multiple lesions in approximately 10 percent Biliary Atresia Diagnosis: HIDA scan doesn't show filling into the intestine Treatment: Roux-en-Y hepaticojejunostomy (Kasai procedure); often requires OLT Choledochal Cysts Classification: Type I: solitary extrahepatic fusiform cyst (most common) Type II: diverticulum of common bile duct Type III: choledochocele Type IV: Multiple intra- and extrahepatic cysts Type V: Caroli’s Disease (diffuse intrahepatic cysts) Diagnosis: U/S, ERCP, PTC Treatment: Hepaticojejunostomy (type I); diverticulectomy (type II); excision / sphincteroplasty (type III); OLT (types IV & V)   Malrotation any child under 2 with bilious vomitng = UPPER GI EMERGENTLY Treatment: Ladds Procedure (counterclockwise detorsion, lysis bands from cecum to abdominal wall and bands between duodenum and terminal ileum, broadening the mesentery, appendectomy, placement of cecum to left lower quadrant and duodenum to right lower quadrant) Hirschsprungs disease failure of neural crest nerve cells to migrate with abscense of Auerbach's plexus 75% rectosigmoid, 15% past splenic flexure, 5% total colon Dx = suction rectal biopsy 2 cm above dentate line Tx = swenson (pull through with complete removal or afunctional colon) Meckel’s Diverticulum Remnant of omphalomesenteric duct on antimesenteric side of bowel most common lead point for intussusception and most common pediatric lower GI bleeding cause Rule of two’s: 2 percent, two feet of ileocecal valve, two types of tissue (gastric, pancreatic), 2 percent symptomatic if incidentially found at abdominal operation - only resect if narrow base Intussusception colicky abdominal pain, bilious emesis, currant jelly stools, abdominal mass on physical exam Treatment: Hydrostatic or pneumatic reduction (80% successful); surgery for failure / recurrence Max Pressure for air contrast enema = 120mmHg, Max column height for barium enema = 1 meter Surgery (gentle pressure rather than pulling the bowel), recurrence is 5-10% after surgery, usually within 24 hours   Meconium Ileus obstruction due to thick meconium “soap-bubble” sign = meconium mixed with air Treatment = water-soluble contrast enema Hirschsprung DiseasePresentation: Bilious emesis, abdominal distension, constipation, failure to pass meconium suction rectal biopsy (presence of ganglion cells rules out diagnosis) - shows absence of Auerbach plexus Treatment: 1. colostomy; 2. ileorectal anastomosis (various types); 3. colostomy takedown Necrotizing Enterocolitis (NEC) Indications for Surgery - Free air, portal venous gas, abdominal wall changes, peritonitis, worsening acidosis/thrombocytopenia, but NOT Pneumatosis    Bell Stage I - suspicious (NPO, TPN, ABX)     Bell Stage II - definite NEC - close observation     Bell Stage III - advanced NEC (absolute indications for surgery) = Surgery Imperforate Anus high (stump above puborectalis) - diverting colostomy and delayed anastomosis -  low: posterior saggital anorectoplasty (PSARP) (Pena Procedure)     High lesions usually form fistulas either to prostatic urethra or vagina low (stump below puborectalis)     Low lesions usually with a fistula to perineum (scrotal raphe or vaginal fourchette)   Sacrococcygeal Teratoma most common teratoma in the neonate and can grow so large that they lead to high output failure, hydrops, and fetal demise treatment - resection   Gastroschisis (bad bowel, good baby”) without peritoneal sac. Rarely associated with other abnormalities. Treatment: primary abdominal wall closure, TPN; Silastic silo if unreducible Omphalocele (“good bowel, bad baby”) bowel hernia with peritoneal covering. Commonly associated with other abnormalities Treatment: reduction and abdominal wall closure Silastic silo to facilitate reduction, primary abdominal wall closure once reducible   Umbilical Hernia most close spontaneously; repair for defect greater than 2 cm, age > 5 years Pentalogy of Cantrell Omphalocele, Anterior diaphragmatic hernia, Sternal cleft, Ectopia cordis, VSD or left ventricle diverticulum Congenital Diaphragmatic Hernia Bochdalek (back and to the left) - most common, usually symptomatic so usually wait 1-3 days for stabilization possibly on ECHO then repair Morgagni - anterior - usually not symptomatic so usually noted in adulthood Inguinal Pathology Hernia: patent process vaginalis; repair open versus laparoscopically more common on right, increased risk with prematurity Hydrocele: fluid collection within scrotum; either communicating or non-communicating, depending on patency of process vaginalis. Non-communicating hydroceles tend to resolve spontaneously. Treatment via surgical evagination with repair of patent process vaginalis Testicular Torsion  acute scrotal pain and high riding testicle   Bilateral exploration through scrotal incision, Pexy both testicles, resect if necrotic Undesceded Testicle Repair at 2 years old orchiopexy doesn't decrease malignancy risk but increases chance of fertility and allows for earlier identification for cancer if in scrotum