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64 Cards in this Set
- Front
- Back
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what is the treatment for biliary atresia?
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Biliary atresia:
-need Kasai procedure before 3 mo = hepatoportoenterostomy |
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what syndrome? exomphalos-macroglossia-gigantism
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Beckwith-Wiedemann:
-overgrowth disorder present at birth characterized by an increased risk of childhood cancer (Wilms, hepatoblastoma) and certain features. -macroglossia (large tongue), macrosomia (birth weight and length >90th percentile), midline abdominal wall defects (omphalocele, umbilical hernia, diastasis recti), ear creases or ear pits, and neonatal hypoglycemia |
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baby presents c/ jaundice >2 wks after birth.
-Liver bx: periportal fibrosis, bile plugging -Tx is __ -> prognosis is _ |
Biliary atresia:
-need Kasai procedure before 3 mo (to avoid liver damage) = hepatoportoenterostomy. 1/3 improve, 1/3 require liver tx, 1/3 die -may be related to in utero viral infection |
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Branchial cleft cysts:
-which one?: angle of mandible, may connect c/ ext auditory canal. avoid facial nerve during dissection |
Branchial cleft cysts:
-1st: angle of mandible, may connect c/ ext auditory canal. avoid facial nerve during dissection -2nd: anterior border of SM. goes through carotid bifurcation into tonsillar pillar -3rd: lateral neck -tx for all = resection |
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Branchial cleft cysts:
-which one?: anterior border of SM. goes through carotid bifurcation into tonsillar pillar |
Branchial cleft cysts:
-1st: angle of mandible, may connect c/ ext auditory canal. avoid facial nerve during dissection -2nd: anterior border of SM. goes through carotid bifurcation into tonsillar pillar -3rd: lateral neck -tx for all = resection |
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Branchial cleft cysts:
-Which one?: lateral neck |
Branchial cleft cysts:
-1st: angle of mandible, may connect c/ ext auditory canal. avoid facial nerve during dissection -2nd: anterior border of SM. goes through carotid bifurcation into tonsillar pillar -3rd: lateral neck -tx for all = resection |
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1st sign of CHF in kids = _
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1st sign of CHF in kids = HEPATOMEGALY
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A 9-month-old girl with a history of intermittent jaundice has a type I choledochal cyst. The most appropriate management is
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Type 1 choledochal cyst = fusiform dilation of entire CBD, mildly dilated common hepatic duct, normal intrahepatic ducts
-85%of choledochal cysts -Tx: cholecystectomy and excision of the cyst with Roux-en-Y hepaticojejunostomy |
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Choledochal cyst:
_% risk of cancer _% risk of pancreatitis |
Choledochal cysts:
-must excise b/c 25% risk of cancer, 30% risk of pancreatitis -2/2 reflux of pancreatic enzymes in utero -TYPE 1 (90%): whole CBD involved => excise + hepaticojejunostomy -Type 2 (3%): diverticulum => diverticulectomy -Type 3: choledochocele involving sphincter => choledochojej OR excise + sphincteroplasty -TYPE 4 (10%): intra- and extrahepatic cysts (Caroli dz) => transplant -Type 5: intrahepatic cysts |
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Type _ choledochal cyst:
>90% of choledochal cysts Tx is _ |
Choledochal cysts:
-must excise b/c 25% risk of cancer, 30% risk of pancreatitis -2/2 reflux of pancreatic enzymes in utero -TYPE 1 (90%): whole CBD involved => excise + hepaticojejunostomy -Type 2 (3%): diverticulum => diverticulectomy -Type 3: choledochocele involving sphincter => choledochojej OR excise + sphincteroplasty -TYPE 4 (10%): intra- and extrahepatic cysts (Caroli dz) => transplant -Type 5: intrahepatic cysts |
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Type _ choledochal cyst = #2 most common = 10% of cases
-Also known as Caroli dz -Tx is _ |
Choledochal cysts:
-must excise b/c 25% risk of cancer, 30% risk of pancreatitis -2/2 reflux of pancreatic enzymes in utero -TYPE 1 (90%): whole CBD involved => excise + hepaticojejunostomy -Type 2 (3%): diverticulum => diverticulectomy -Type 3: choledochocele involving sphincter => choledochojej OR excise + sphincteroplasty -TYPE 4 (10%): intra- and extrahepatic cysts (Caroli dz) => transplant -Type 5: intrahepatic cysts |
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what is therapy for type 1 choledochal cyst?
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Choledochal cysts:
-must excise b/c 25% risk of cancer, 30% risk of pancreatitis -2/2 reflux of pancreatic enzymes in utero -TYPE 1 (90%): whole CBD involved => excise + hepaticojejunostomy -Type 2 (3%): diverticulum => diverticulectomy -Type 3: choledochocele involving sphincter => choledochojej OR excise + sphincteroplasty -TYPE 4 (10%): intra- and extrahepatic cysts (Caroli dz) => transplant -Type 5: intrahepatic cysts |
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What is this baby's problem?
Resp distress. CXR shows unilateral upper/middle lobe radiolucency -Tx is _ |
Congenital lobar emphysema (cartilage fails to develop in bronchus => air trapping c/ expiration)
-massive hyperinflation of a single lobe, usally upper/middle -1/3 have resp distress @ birth -5% present after 6 mo old. 2:1 M;F -CXR: radiolucency of affected lobe, compression of other lobe -If severely symptomatic, requires lobectomy. excellent prognosis |
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baby in resp distress, hypoxic. Bowel in L chest.
-What is the treatment approach? |
Congenital diaphragmatic hernia:
-50% survival - depends on pulm hypoplasia, pulm HTN. -80% on L. both lungs usually dysfunctional (contralateral dysfx not well understood) -80% have cardiac, neural tube anomalies, malrotation -Tx: stabilization c/ high-freeq ventilation, ECMO, inhaled NO, prostacyclin (pulm vasodilator) -> repair after 24-72 hrs to improve tolerance of surgery -usually abdominal approach, repair c/ mesh. Run bowel to r/o visceral anomalies. -Bochdalek's #1, posterior > Morgagni's rare, anteriro -Eventration = failure of diaphragm to fuse |
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baby in resp distress, hypoxic. Bowel in L chest.
-What is the prognosis? What determines the prognosis? |
Congenital diaphragmatic hernia:
-50% survival - depends on pulm hypoplasia, pulm HTN. -80% on L. both lungs usually dysfunctional (contralateral dysfx not well understood) -80% have cardiac, neural tube anomalies, malrotation -Tx: stabilization c/ high-freeq ventilation, ECMO, inhaled NO, prostacyclin (pulm vasodilator) -> repair after 24-72 hrs to improve tolerance of surgery -usually abdominal approach, repair c/ mesh. Run bowel to r/o visceral anomalies. -Bochdalek's #1, posterior > Morgagni's rare, anteriro -Eventration = failure of diaphragm to fuse |
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what is the most common type of congenital diaphragmatic hernia?
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Congenital diaphragmatic hernia:
-50% survival - depends on pulm hypoplasia, pulm HTN. -80% on L. both lungs usually dysfunctional (contralateral dysfx not well understood) -80% have cardiac, neural tube anomalies, malrotation -Tx: stabilization c/ high-freeq ventilation, ECMO, inhaled NO, prostacyclin (pulm vasodilator) -> repair after 24-72 hrs to improve tolerance of surgery -usually abdominal approach, repair c/ mesh. Run bowel to r/o visceral anomalies. -Bochdalek's #1, posterior > Morgagni's rare, anteriro -Eventration = failure of diaphragm to fuse |
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cryptorchidism: _% have other anomalies
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cryptorchidism: 15% have other anomalies
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"
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Cystic hygroma is a cystic lymphatic malformation, thought to develop from sequestered lymphatic sacs that fail to communicate with peripheral draining channels. The cyst may be unilocular or multilocular and of variable size. The cyst wall is of variable thickness, and can be fibromuscular and contain mural thrombi. The fluid within the cyst is proteinaceous and may be haemorrhagic especially in combined lymphaticovenous malformations. 70–80% of lymphangiomas occur in the neck, usually in the posterior cervical triangle. Other sites include the axillary region (20%), superior mediastinum, mesentery, retroperitoneum, pelvis and lower limbs.
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What is the #1 complication of cystic hygroma?
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Cystic hygroma = lymphangioma
-Tx: resect -INFECTION = #1 complication (no malignant degeneration) -In lateral cervical, submandibular regions of neck |
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In normal neonates, renal function is characterized by
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obligatory diuresis
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A newborn male infant develops abdominal distention and vomiting of bile-stained fluids. A plain x-ray of the abdomen shows two gas shadows in the upper abdomen. Proper operative correction requires
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Duodenal atresia: #1 cause of duodenal obstruction in newborns (<1 wk). After 1 wk old, malrotation #1
-usually distal to ampulla. Bilious vomiting, intol to PO -Ass'd c/ polyhydraminos in mother. Also c/ cardiac, renal, and other GI anomalies. -Failure of vacuolization of duodenum from solid cords -20% have Downs => check chromosomal studies -Ab xray: double bubble -Tx: resuscitaton, DUODENOJEJUNOSTOMY or duodenoduodenostomy |
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A 7-year-old child sustained abdominal trauma. During an uneventful recovery, he develops signs and symptoms of duodenal obstruction. Endoscopy and an upper gastrointestinal series with barium show a duodenal hematoma. The most appropriate management is
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Missed duodenal hematoma can present c/ high SBO 12-72 hrs p/ injury
-UGI shows "stacked coins" or "coiled spring" appearance -TPN + NG tube cures 90% in 2-3 wks |
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gastroschisis is 2/2 __
-looks like... |
Gastroschisis:
-intrauterine rupture of umbilical cord => involution of right umbilical vein -> lateral (RIGHT) defect, no sac -only 7% have other anomalies, e.g. atresia |
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baby has no BM in 1st 24 hrs. X-ray shows colonic distension.
-On exam, anus looks normal. explosive stool after finger in anus Diagnosis made by ... |
Hirschprung's:
-#1 cause of pediatric colon obstruction -No BM in 1st 24 hrs => dx by rectal bx (absence of ganglion cells in myenteric plexus) |
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consulted for infant c/ meconium in urine. Suspect __
Tx is _ |
imperforate anus:
-in high, have meconium in urine (fistula to bladder, vagina, or urethra) => need colostomy |
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what is the treatment for hirchsprung's enterocolitis?
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Hirschprung's:
-#1 cause of pediatric colon obstruction -No BM in 1st 24 hrs => dx by rectal bx (absence of ganglion cells in myenteric plexus) -Enterocolitis => treat c/ NS enema + abx |
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what are the 3 indications for contralateral exploration during ped inguinal hernia repair?
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Inguinal hernia:
-2/2 patent processus vaginalis. 3% of infants. 10% of preemies. M>F -60% RIGHT, 30% L, 10% bilat -extends into internal ring (unlike hydrocele) -If able to reduce, do repair c/ high ligation 3 days later -Urgent repair if unable to reduce -Explore contralateral side if L-sided, female, or <1 y/o |
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what is treatment for inguinal hernia in a 3 year old?
-if able to reduce.. -If unable to reduce... |
Inguinal hernia:
-2/2 patent processus vaginalis. 3% of infants. 10% of preemies. M>F -60% RIGHT, 30% L, 10% bilat -extends into internal ring (unlike hydrocele) -If able to reduce, do repair c/ high ligation 3 days later -Urgent repair if unable to reduce -Explore contralateral side if L-sided, female, or <1 y/o |
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Intestinal atresia:
-2/2 _ -_% are multiple -duodenal atresia ass'd c/ genetic illness _; 1/3 have defects in what other organ? |
intestinal atresia:
2/2 intrauterine vascular events. mother may have polyhydramnios. 10% are multiple -Duodenal atresia = #1 neonatal duodenal obstruction. bilious vomiting, "double bubble" on xray. Ass'd c/ Trisomy 21. 1/3 have cardiac defects |
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A 34 week premature neonate is delivered emergently by cesarean section. In the first hours of life, the neonatal nurses notice that the patient’s abdomen has become distended and tympanic. A small bowel follow through is ordered by the neonatologist on call. A diagnosis of proximal ileal atresia is made. What is the embryologic cause of ileal atresia?
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All intestinal atresias except duodenal atresia are the result of intrauterine vascular accidents.
Duodenal atresia is the result of failure to recannualize the duodenum during development in utero. |
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Intussusception
-Drug _ may be helpful -who requires surgery? |
Intussusception:
-reduce c/ contrast/air enema (max column height of barium 1 meter; max air pressure 120 mmHg; up to 1 hour) -IV glucagon relaxes smooth muscle, may help -usually >3 y/o -to OR for children peritonitis or free air; all adults require surgery 2/2 liklihood of malignancy @ lead point |
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what is the max barium column height and air pressure used to reduce intussusception?
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Intussusception:
-reduce c/ contrast/air enema (max column height of barium 1 meter; max air pressure 120 mmHg; up to 1 hour) -IV glucagon relaxes smooth muscle, may help -usually >3 y/o -to OR for children peritonitis or free air; all adults require surgery 2/2 liklihood of malignancy @ lead point |
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baby c/ Trisomy 21 presents c/ bilious vomiting.
-must consider _ |
intestinal atresia:
2/2 intrauterine vascular events. mother may have polyhydramnios. 10% are multiple -Duodenal atresia = #1 neonatal duodenal obstruction. bilious vomiting, "double bubble" on xray. Ass'd c/ Trisomy 21. 1/3 have cardiac defects |
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For a 4 year old boy, the minimum volume of urine needed to get ride of solutes in urine is ...
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According to most reference adults require 0.5cc, infants 2cc and non-infant children require 1cc/kg/hr.
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what is an appropriate IV fluid bolus in a child (e.g. 5 y/o, 30 Kg)
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20 cc/Kg fluid bolus = 500 cc in this child
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what is a Ladd's procedure (4 main steps)?
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Malrotation:
-Upper GI series for bilious vomiting in any child <3 y/o => duodenum doesn't cross midline. -75% present in 1st month, 90% in 1st year -#1 duodenal obstruction in non-neonates -IMMEDIATE surgery to avoid necrosis of entire sm bowel 2/2 volvulus, strangulation of SMA -Ladd's procedure: appendectomy, take down bands, counterclockwise rotation 270 deg, fix cecum in LLQ |
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3 wk old infant presents c/ bilious vomiting.
-Next test = _ |
Malrotation:
-Upper GI series for bilious vomiting in any child <3 y/o => duodenum doesn't cross midline. -75% present in 1st month, 90% in 1st year -#1 duodenal obstruction in non-neonates -IMMEDIATE surgery to avoid necrosis of entire sm bowel 2/2 volvulus, strangulation of SMA -Ladd's procedure: appendectomy, take down bands, counterclockwise rotation 270 deg, fix cecum in LLQ |
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how is a meckel's diverticulum detected on imaging?
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Meckel's diverticulum: 2/2 patent omphalomesenteric duct
-on anti-mesenteric border, 2 ft from ileocecal valve -2% of population, 2% symptomatic, 2 tissue types (pancreatic, gastric) 2 common presentations: diverticulitis, GI bleed (#1 GI bleed in children) -meckel scan = technicium 99 => gastric mucosa lights up |
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what is the #1 source of GI bleed in children?
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Meckel's diverticulum: 2/2 patent omphalomesenteric duct
-on anti-mesenteric border, 2 ft from ileocecal valve -2% of population, 2% symptomatic, 2 tissue types (pancreatic, gastric) 2 common presentations: diverticulitis, GI bleed (#1 GI bleed in children) |
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Meconium ileus in CF:
Good dx and tx accomplished by ... |
Meconium ileus (10% of cystic fibrosis):
-Obstruction in terminal ileum +/- ground-glass or soar-suds appearance of thick bowel contents stuck to wall -Complications: perforation -> meconium pseudocyst or free perf -Barium or gastrograffin enema for dx and tx (effective in 80%) -N-acetylcysteine enemas work better than barium for relieving obstruction -Also needs sweat Cl- test or PCR for Ca- channel defect -If surgery needed, can do manual decompression + create a vent for N-ACETYLCYSTEINE ANTEGRADE ENEMA |
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newborn passes no stool in 1st 24 hrs.
Xray shows distended loops of sm bowel, no air-fluid levels. Colon decompressed. Transition point in RLQ. -Next step = _ |
Meconium ileus (10% of cystic fibrosis):
-Obstruction in terminal ileum +/- ground-glass or soar-suds appearance of thick bowel contents stuck to wall -Complications: perforation -> meconium pseudocyst or free perf -Barium or gastrograffin enema for dx and tx (effective in 80%) -N-acetylcysteine enemas work better than barium for relieving obstruction -Also needs sweat Cl- test or PCR for Ca- channel defect -If surgery needed, can do manual decompression + create a vent for N-ACETYLCYSTEINE ANTEGRADE ENEMA |
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NEC typically presents as ...
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NEC:
-bloody stool after initiating feeds in preemie -to OR if free air, peritonitis, acidosis/thrombocytopenia, clinical deterioration (resect, ostomies) -Must do contrast eval before reconnecting bowel weeks later 2/2 stenoses in 20% |
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in baby that has undergone ex lap for NEC, __ is necessary prior to returning to OR for reversal of ostomy
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NEC:
-bloody stool after initiating feeds in preemie -to OR if free air, peritonitis, acidosis/thrombocytopenia, clinical deterioration (resect, ostomies) -Must do contrast eval before reconnecting bowel weeks later 2/2 stenoses in 20% |
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_ = #1 solid pediatric malignancy
-Ass'd c/ biomarkers .... -Ass'd c/ oncogene ... |
Neuroblastoma:
-#1 solid pediatric malignancy, usually age 0-2 -From neural crest cell. most often on adrenals -90% have increased VMA -high HVA (homovanillic acid) => worse prognosis -30% cure -ass'd c/ N-myc -= |
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Toddler presents c/ initially asymptomatic fixed abdominal mass from flank to midline of abdomen, then secretory diarrhea, HTN, unsteady gait (opsomyoclonus syndrome)
-Suspect _ |
Neuroblastoma:
-#1 solid pediatric malignancy, usually age 0-2 -Neural crest cells. most often on adrenals. 90% have high VMA. high HVA (homovanillic acid) => worse prognosis -30% cure -ass'd c/ N-myc -Usually asymptomatic; rest are extensive locoregional or metastatic dz. Sx are secretory diarrhea, raccoon eyes (orbital mets), HTN, opsomyoclonus syndrome |
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the 5 anomalies of cardiac, pericardium, stenum, diaphragm, and __ are known as Cantell pentology
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omphalocele:
-midline defect, has peritoneal sac that may contain liver or other non-bowel contents. -frequently have other anomalies (cardiac, pericardium, stenum, diaphragm), trisomy 13/18/21, Beckwith-Weidmann |
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Pulmonary sequestration:
-Extralobar type supplied by _ type artery, _ type vein -Intralobar type supplied by _ type artery, _ type vein |
Pulmonary sequestration:
-Extralobar: systemic artery + vein -Intralobar: aorta in, pulm vein out -Neither communicates c/ tracheobronchial tree -Tx: resection (lobectomy) -INFECTION = #1 presentation (not as resp distress in newborn) |
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Pulmonary sequestration:
Typically presents as __ Tx is _ |
Pulmonary sequestration:
-Extralobar: systemic artery + vein -Intralobar: aorta in, pulm vein out -Neither communicates c/ tracheobronchial tree -Tx: resection (lobectomy) -INFECTION = #1 presentation (not as resp distress in newborn) |
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An 8 year old child with history of chronic respiratory infections is diagnosed with extralobar pulmonary sequestration. In regards to this condition, which correctly describes the vascular anatomy
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Pulmonary sequestrations are congenital lesions with no abnormal connection to tracheobronchial tree. Arterial supply is always systemic, directly from aorta. Drainage is either to systemic veinous system (extralobular sequestration, infants) or pulmonary veinous system (intralobular sequestration, older children). The treatment of choice consists of thoracotomy and resection
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3 week old male presents c/ forceful vomiting, mass in RUQ.
-Next test should be... |
Pyloric stneosis:
-3-12 wk old firstborn M c/ projectile non-bilious vomiting + olive-shaped mass in RUQ -Dx; US shows pylorus at least 4mm thick or 14 mm long -Tx: pyloromyotomy. D10, correct lytes before PR |
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ultrasound findings c/w pyloric stenosis
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Pyloric stneosis:
-3-12 wk old firstborn M c/ projectile non-bilious vomiting + olive-shaped mass in RUQ -Dx; US shows pylorus at least 4mm thick or 14 mm long -Tx: pyloromyotomy. D10, correct lytes before PR |
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Sacrococcygeal teratoma;
-Prognosis is predicted by _ -Tx is _ |
sacrococcygeal teratoma:
-80-85% female -90% benign at birth. great potential for malignancy. -almost all have exophytic component -AFP = good marker -<2 months => 90% benign ->2 months => 90% malignant -Tx: coccygectomy + long term follow-up -Poor response to chemo, XRT |
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15M presents c/ acute R scrotal pain, high-riding R testicle.
What is the operative approach? |
Testicular torsion:
-peaks in 15 y/o -Involved testis almost never viable -Usually have intravaginal torsion of the sermatic cord if viable. Torsion usually towards midine. -Tx; BIALTERAL ORCHIOPEXY or resection plus contralateral orchiopexy |
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young man presents c/ painful testicular massw x 3 mo
Diagnosis is most likely _ -Tx is _ |
Most likely testicular tumor
-Torsion wouldn't be present 3 months -Tx: orchiectomy by trans-inguinal approach (no scrotal incision to not disrupt lymphatic plane) |
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5% of tracheoesophageal fisulas are type A = _
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Tracheoesophegeal fistula:
-A (5%) = blind esophagus, no fistula = no air in GI tract -C (90% = Common) = blind esophagus, distal TEF. spit up feeds, NG won't pass. R extrapleural thoracotomy, primary repair, +/- sternohyoid flap, +/- divide azygos, G tube, -E; most likely to present as adult (?). H config. of esophagus + trachea. Not ass'd c/ atresia -If premature, <2500g, or sick => Repogle tube, treat resp sx, G tube, delay repair. -Complications of repair: GERD (may need fundoplication later) > leak, empyema, fistula -Survival related to birthweight, ass'd anomalies |
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what determines survival in baby with tracheoesophgeal fistula?
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Tracheoesophegeal fistula:
-A (5%) = blind esophagus, no fistula = no air in GI tract -C (90% = Common) = blind esophagus, distal TEF. spit up feeds, NG won't pass. -R extrapleural thoracotomy, primary repair, +/- sternohyoid flap, +/- divide azygos, G tube, -E; most likely to present as adult (?). H config. of esophagus + trachea. Not ass'd c/ atresia -If premature, <2500g, or sick => Repogle tube, treat resp sx, G tube, delay repair. -Complications of repair: GERD (may need fundoplication later) > leak, empyema, fistula -Survival related to birthweight, ass'd anomalies |
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patient with unilateral undescended testicle should undergo repair @ _ y/o
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Undescended testicles:
-higher risk of testicular ca -chromosomal studies indicated if both testicles not in scrotum -MRI if can;t feel testicle in inguinal canal by age 2 -division of spermatic vessels = acceptable methods of gaining length if testicles don't reach scrotum |
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90% of tracheoesophageal fisulas are type _, which is ...
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TE fistula:
-5% type A= blind esophagus, no fistula = no air in GI tract -90% type C ("Common") = blind esophagus, distal TEF. spit up feeds, NG won't pass -VATER = vertebral, anorectal (imperf anus in 10%), TEF, radius, renal anomalies |
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what is the treatment for tracheoesophageal fistula?
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Tracheoesophegeal fistula:
-A (5%) = blind esophagus, no fistula = no air in GI tract -C (90% = Common) = blind esophagus, distal TEF. spit up feeds, NG won't pass. -R extrapleural thoracotomy, primary repair, +/- sternohyoid flap, +/- divide azygos, G tube, -E; most likely to present as adult (?). H config. of esophagus + trachea. Not ass'd c/ atresia -If premature, <2500g, or sick => Repogle tube, treat resp sx, G tube, delay repair. -Complications of repair: GERD (may need fundoplication later) > leak, empyema, fistula -Survival related to birthweight, ass'd anomalies |
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what does VACTERL stand for?
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Vertebral
Anorectal (imperf anus) Cardiac TEF Radius/Renal Limb anomalies |
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Prognosis for Wilm's tumor depends on __
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Wilms tumor = nephroblastoma
-80% cure c/ nephrectomy. Prognosis depends on tumor grade (analplastic, sarcomatous = worst prognosis). -Mean age @ dx = 3. -frequent mets on bone, lung. can resect pulm mets if resectable. -May be ass'd c/ Beckwith -Weidman syn (hemihypertrophy, cryptorchidism, Drash syn, aniridia) -All pts except Stage 1 <500g get actinomycin + vincristine. Add doxorubicin for srage II or >500g. Add ab XRT if stage 3. |
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3 y/p presents c/ hematuria, HTN. Must consider _
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Wilms tumor = nephroblastoma
-80% cure c/ nephrectomy. Prognosis depends on tumor grade (analplastic, sarcomatous = worst prognosis). -Mean age @ dx = 3. -frequent mets on bone, lung. can resect pulm mets if resectable. -May be ass'd c/ Beckwith -Weidman syn (hemihypertrophy, cryptorchidism, Drash syn, aniridia) -All pts except Stage 1 <500g get actinomycin + vincristine. Add doxorubicin for srage II or >500g. Add ab XRT if stage 3. |
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Patient has hx of hemihypertrophy, cryptorchidism, Drash syndrome, aniridia. Now presents c/ hematuria. Consider _
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Wilms tumor = nephroblastoma
-80% cure c/ nephrectomy. Prognosis depends on tumor grade (analplastic, sarcomatous = worst prognosis). -Mean age @ dx = 3. -frequent mets on bone, lung. can resect pulm mets if resectable. -May be ass'd c/ Beckwith -Weidman syn (hemihypertrophy, cryptorchidism, Drash syn, aniridia) -All pts except Stage 1 <500g get actinomycin + vincristine. Add doxorubicin for srage II or >500g. Add ab XRT if stage 3. |
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Shortly after birth, an infant is noted to have a large 5 cm soft, fluctuant, multi-loculated mass on the neck. The most likely diagnosis is:
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Cystic hygroma is a cystic lymphatic malformation, thought to develop from sequestered lymphatic sacs that fail to communicate with peripheral draining channels. The cyst may be unilocular or multilocular and of variable size. The cyst wall is of variable thickness, and can be fibromuscular and contain mural thrombi. The fluid within the cyst is proteinaceous and may be haemorrhagic especially in combined lymphaticovenous malformations. 70–80% of lymphangiomas occur in the neck, usually in the posterior cervical triangle. Other sites include the axillary region (20%), superior mediastinum, mesentery, retroperitoneum, pelvis and lower limbs.
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