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Case 1
39 week gestational age Normal pregnancy and vaginal delivery Apgars 91,105
Started breastfeeding and started to have multiple episodes of bright yellow/green emesis
Prenatal work-up
Trisomy
Case 1
Clinical examination HR 160, RR 40, BP 80/50 O2 sats 100% room air HEENT: macroglossia, epicanthic fold of the eyelid, upslanting palpebral fissures Chest: Good AE=AE Cardiac: holosystolic III/VI murmur, normals S1, S2 Abdomen: non distended, soft, nontender, no erythema, no HSM Normal female genitalia MSK/Neuro: Simian crease, slight decreased muscle tone.
Case 1
Investigations
Bloodwork
Imaging Any
ECHO
VSD
GI
Duodenal atresias/webs small bowel atresias malrotation/midgut volvulus GERD Meconium ileus pyloric stenosis Inguinal hernia NEC
GI
Colonic atresia Meconium plug Hirschsprungs Small Left Colon Syndrome Microcolon-Intestinal Hypoperistalsis Syndrome Imperforate anus
causes
GERD
Radiological workup
KUB/Cross-table lateral Contrast enemas for distal obstructions UGI for malrotation/proximal atresias
Duodenal atresia
Management
NGT
Resuscitate
Surgical approach
duodenoduodenostomy
B. Abdominal X-ray is usually normal. C. Results from disruption of fetal blood supply. D. Operative repair involves duodenal resection. E. Concomitant abnormalities can include annular pancreas, esophageal atresia, or VACTERL lesions.
B. Abdominal X-ray is usually normal. C. Results from disruption of fetal blood supply. D. Operative repair involves duodenal resection. E. Concomitant abnormalities can include annular pancreas, esophageal atresia, or VACTERL lesions.
Duodenal atresia
Duodenal Atresia
Associated with Downs syndrome in 30% Atresia seen in 10% of Downs patients Vomiting can be bilious or non-bilious Abdominal X-ray shows double-bubble Best repaired by bypass -> duodenoduodenostomy or duodenojejunostomy
Case 2
2 day old infant in newborn nursery Sent to NICU for evaluation of bilious emesis
Physical Examination
Chest
Cardiac
Abdo
Nondistended, generalized tenderness Soft, no discoloration, no masses, no HSM No inguinal hernias
Work-up
Bloodwork
Imaging
Management of Malrotation/volvulus
1. Entry into abdominal cavity and evisceration (open) 2. Counterclockwise detorsion of the bowel (acute cases) 3. Division of Ladds cecal bands 4. Broadening of the small intestine mesentery 5. Incidental appendectomy 6. Placement of small bowel along the right lateral gutter and colon along the left lateral gutter
Ladd Procedure
Malrotation
Occurs in 1/200 1/500 live births Symptomatic in 1/6000 live births 30-62% have associated anomaly Up to 75% present w/in 1st month of life Classic presentation is infant with bilious emesis
May
Malrotation
Due to abnormal fixation of midgut to retroperitoneum leads to narrow base of mesentery which can easily twist Ladd Procedure
Reduce volvulus by rotating counterclockwise Division of Ladds bands between cecum and duodenum/right gutter Division of adhesions to widen mesentery Run bowel to r/o obstructions Appendectomy Place bowel in nonrotated position
Case 3
2 day old infant in NICU 3 Consulted for abdominal distention and bilious emesis Work-up and differential
How would you confirm diagnosis and what would you see Classification scheme
Case 4
3 day old infant Failure to pass stools, abdominal distention and bilious emesis Work-up and differential
B. Most often requires operative intervention. C. Presents as a neonatal bowel obstruction. D. X-rays may reveal a stippled pattern in the RLQ (soap bubble sign). E. May be relieved by water-soluble contrast enema.
B. Most often requires operative intervention. C. Presents as a neonatal bowel obstruction. D. X-rays may reveal a stippled pattern in the RLQ (soap bubble sign). E. May be relieved by water-soluble contrast enema.
Meconium Ileus
Newborn bowel obstruction secondary to inspissated meconuim in distal ileum Enema reveals microcolon -> may be therapeutic Non-operative management successful in 2/3 OR required for perforation or failed enema
may
Management
Fluid resuscitaion Gastric decompression Pulmonary support as needed Contrast enema with water soluble contrast
Surgery
2-4% NAC, 50% hyperosmolar agent via appendix Alternative surgical techniques involve resection, anastomosis, and temporary enterostomy through which postoperative irrigations may be delivered
adhesive meconium peritonitis giant cystic meconium peritonitis or pseudocyst meconium ascites infected meconium peritonitis
Case 5
An 8 hr old infant drools and spits up his first feed. A tube is passed into the esophagus and a film is obtained.
8%
1%
85%
2%
4%
Atresias detected by inability to pass NGT/OGT TEF w/o atresia presents with recurrent aspiration Low-risk infants should get primary repair
long gap (>3 vertebral bodies) repair is delayed high-risk babies get gastrostomy
Case 6
A listless 9-month-old boy presents with acute onset of severe intermittent abdominal pain. Rectal exam is guaiac positive. What is the most likely diagnosis? A. Meckels diverticulum. B. Acute appendicitis. C. Intussusception. D. Intestinal polyp. E. Gastritis.
A. B. C. D. E.
Intussusception
Telescoping of terminal ileum into large intestine Contrast enema for diagnosis will reduce 80%
air pressure to 120 mmHg, barium to 100 cm H2O 10% recurrence, often within hours
Intussusception
Plain AXR
Look
Which of the following statements is TRUE with respect to neonatal abdominal wall defects?
B. Gastroschisis is frequently associated with other anomalies. C. A Silastic silo is rarely employed in management of these defects. D. Mortality is higher in gastroschisis. E. Operative management of omphalocele usually requires bowel resection.
Which of the following statements is TRUE with respect to neonatal abdominal wall defects?
B. Gastroschisis is frequently associated with other anomalies. C. A Silastic silo is rarely employed in management of these defects. D. Mortality is higher in gastroschisis. E. Operative management of omphalocele usually requires bowel resection.
Omphalocele
over 50% have associated cardiac, GI, GU, musculoskeletal, or CNS anomalies
Omphalocele
Omphalocele
Non-operative management with escharotic agent OR for reduction and closure of abdominal wall
keep intra-abdominal pressure < 20 mmHg large defects require skin flap or prosthetic Silastic silo most common, reduce daily for 3-10 days
Post-op complications include sepsis, GE reflux, inguinal hernias, abdominal wall hernia
Gastroschisis
Initial management
aggressive fluid replacement (2-3X normal) protection of exposed bowel w/occlusive dressing
Small bowel
Colon
Bladder
Stomach
Gastroschisis
Post-op complications:
abdominal compartment syndrome sepsis necrotizing enterocolitis abdominal wall cellulitis prolonged ileus short gut syndrome w/ TPN dependence
Case 7
3. A 1.5 kg, 30-wk preemie develops abdominal distention and bloody stool after 1st feedings. Which of the following is TRUE regarding his condition?
A. Supportive treatment includes stopping all feeds, NGT drainage, IVF, serial abdominal exams and radiographs. B. IV antibiotics not indicated unless pathogen identified. C. Barium enema is the imaging modality of choice. D. Overall mortality reported as 50-60%. E. Intestinal stricture formation is rare.
Case 7
A. Supportive treatment includes stopping all feeds, NGT drainage, IVF, serial abdominal exams and radiographs. B. IV antibiotics not indicated unless pathogen identified. C. Barium enema is the imaging modality of choice. D. Overall mortality reported as 50-60%. E. Intestinal stricture formation is rare.
feeding intolerance vomiting abdominal distention progressive sepsis autonomic instability (Apneas and Bradys) abdominal wall erythema +/- mass
Labs:
X-rays: distended loops c/w ileus, pneumatosis intestinalis May appear normal or mild ileus at first Progression demonstrates portal venous air (pathognomonic)
Pathogenesis
No
Prevention
Breast
Medical Treatment
NPO, NGT, TPN AXR q 8 hr Usually necessitates surgery within 24 hr or not at all NPO for 10 to 14 days after radiographic evidence of disease has abated Broad spectrum Abx
Indications for OR are free air (absolute), fixed abdominal mass, abdominal wall erythema, failure to improve (controversial)
OR for resection of dead bowel, formation of stomas second-look laparotomy 24-48 hrs if needed Peritoneal drainage
Overall mortality 20-40% Long term complications of strictures, short bowel syndrome
Case 8
4. A full-term newborn has not passed meconuim by DOL 2. Which of the following is FALSE regarding his likely diagnosis?
A. B. C. D. E.
It is more common in males. Suction rectal biopsy is rarely adequate for diagnosis. Enterocolitis is a significant cause of mortality. Disease is most often confined to the distal colon. Barium enema may be normal.
Case 8
A. B. C. D. E.
It is more common in males. Suction rectal biopsy is rarely adequate for diagnosis. Enterocolitis is a significant cause of mortality. Disease is most often confined to the distal colon. Barium enema may be normal.
Hirschsprungs Disease
proximal extension of aganglionosis lack of peristalsis and failure of sphincter relaxation rectosigmoid only in 75%, entire colon in 8%
Hirschsprungs Disease
Presents as failure to pass meconium w/in 24 hrs or constipation in older child Diagnosis best made by rectal biopsy
suction
Hirschsprungs Disease
Hirschsprungs Disease
Hirschsprungs Disease
Ganglion cells
Hirschsprungs Disease
1. Absence of ganglion cells 2. Hypertrophic nerve trunks
Hirschsprungs Disease
Swenson
Soave
Duhamel
Hirschsprungs Disease
Enterocolitis
12
58% ? Fecal stasis Life threatening Treat with rectal irrigation and flagyl
Case 9
Newborn infant, 36 week gestational age, delivered for PROM No prenatal care Significant respiratory distress at birth requiring emergent intubation Apgars 2 and 5
Case 9
CDH
Prenatal:
Polyhydramnios Interventions
Gentle ventilation nitric oxide surfactant high frequency, oscillating ventilation muscle paralysis, induced alkalosis spontaneous respiration, permissive hypercapnea perfluorocarbon ventilation combinations of the above extracorporeal life support SURGERY once physiolgically stable
ECMO CANNULATION
ECMO CANNULATION
VENO-ARTERIAL CANNULATION
ECMO CANNULATION
VENO-VENOUS CANNULATION
ECMO Circuit
CDH - Survival
Prognosis: Pulmonary recovery: Overall reported survival varies among institutions. When all resources, including ECMO, are provided, survival rates range from 4069%. Long-term morbidity: Significant long-term morbidity, including chronic lung disease, growth failure, gastroesophageal reflux, and neurodevelopmental delay, may occur in survivors.
Case 10
A 5-wk-old boy presents with 3 days of non-bilious projectile vomiting and dehydration. Which of the following is TRUE about his condition?
B. C. D. E.
UGI series is the diagnostic procedure of choice. Delay in diagnosis leads to metabolic acidosis. Most commonly seen in females. Fluid replacement consists of NS + KCL
A 5-wk-old boy presents with 3 days of non-bilious projectile vomiting and dehydration. Which of the following is TRUE about his condition?
B. C. D. E.
UGI series is the diagnostic procedure of choice. Delay in diagnosis leads to metabolic acidosis. Most commonly seen in females. Fluid replacement consists of NS + KCL
Pyloric Stenosis
1 in 600 births, male: female ratio 4:1, 3-12 weeks Gastric outlet obstruction due to hypertrophy of pyloric muscle Progressive, projectile non-bilious vomiting Hypochloremic, hypokalemic metabolic alkalosis
renal compensation for hypovolvemia thickness > 5 mm, channel length > 15 mm
Pyloromyotomy
Case 11
A 6-wk-old infant presents with jaundice. A sonogram appears normal. HIDA scan fails to demonstrate emptying into the duodenum. What is the next best step in management?
B. Follow closely until 3 months of age, then do Kasai. C. Percutaneous liver biopsy. D. Initiate anti-inflammatory therapy. E. Laparotomy with operative cholangiogram and liver biopsy, then Kasai if warranted.
A 6-wk-old infant presents with jaundice. An abdominal USG appears normal. HIDA scan fails to demonstrate emptying into the duodenum. What is the next best step in management?
A. List for liver transplant. B. Follow closely until 3 months of age, then do Kasai. C. Percutaneous liver biopsy. D. Initiate anti-inflammatory therapy. E. Laparotomy with operative cholangiogram and liver biopsy, then Kasai if warranted.
Biliary Atresia
Jaundice, conjugated hyperbilirubinemia, firm hepatomegaly due to biliary cirrhosis Lab work up should include LFTs, Alpha-1 antitrypsin, TORCH infections, sweat test, hepatitis Sono shows no extrahepatic ducts, tiny gallbladder HIDA scan reveals no emptying into the duodenum Liver biopsy reveals cholestasis and bile duct proliferation
Kasai Portoenterostomy
Roux-en-Y limb of jejenum sutured to porta where atretic bile ducts exit hepatic parenchyma Results depend on age (10 weeks), anatomy and histology of atretic bile ducts, ? degree of cirrhosis
overall: 1/3 fail immediately Long term survival in 25% of those that have drainage Results of liver transplantation not affected by Kasai procedure
Biliary Atresia
Biliary Atresia
Kasai Portoenterostomy
Pulmonary Sequestration
Cystic mass of nonfuctioning primitive lung tissue not connected to tracheobronchial tree
Extrapulmonary
Intrapulmonary (90%)
Males 3-4:1 Systemic arterial supply 95% Systemic venous drainage >80%
Pulmonary Sequestration
tract
Air trapped in the lobe Leads to adjacent lobe atelectasis Shifts mediastinum to opposite side More common in the upper lobes CXR for diagnosis Nonop management low vent pressure/volume, positioning Resection provides definitive treatment
Case 1
18mos old female Presents to your office with a mass above her left eyebrow What next? Differential diagnosis
Evaluation of mass
H&P
Age Onset
Rapidity
Consistency
Color Mobility
Tenderness
Fluctuation
PE
Case 1
Differential diagnosis
Differential Diagnosis
Congenital
Branchial cleft cysts Thyroglossal duct cyst Dermoid cyst Vascular malformation
Inflammatory lesions
Reactive lymphadenopathy Granulomatous disease
Lymphatic Hemangioma
Suppurative lymphadenitis
Noninflammatory benign
Inclusion cyst Fibromatosis Keloid
Differential Diagnosis
Benign neoplasms
Neurofibroma
Lipoma Paraganglioma
Malignant Neoplasm
Lymphoma
Hodgkins NonHodgkins
Goiter Thyroid
Thyroid
nodule
Case 2
2 year old male Mass on side of neck Noticed recently and slowly has increased in size
One
episode where it was erythematous and tender Treated with antibiotics and resolved
Case 2
Branchial arches
Case 3
Case 3
An 8 y.o. boy has a recurrent painful swelling in a 2cm mass in the midline of his neck below the hyoid bone. Which is TRUE?
B. surgical excision includes the pyramidal lobe of the thyroid C. the structure originates at the foramen cecum D. Fistula tracts drain laterally at the inferior border of the sternoclaidomastoid E. Simple excision can be done with local anesthesia
An 8 y.o. boy has a recurrent painful swelling in a 2cm mass in the midline of his neck below the hyoid bone. Which is TRUE?
B. surgical excision includes the pyramidal lobe of the thyroid C. the structure originates at the foramen cecum D. Fistula tracts drain laterally at the inferior border of the sternoclaidomastoid E. Simple excision can be done with local anesthesia
Arise from duct formed when developing thyroid passes from lingual foramen cecum through/near hyoid bone to neck Most common midline neck mass in kids
May
entire duct to level of foramen cecum, including part of hyoid bone to prevent recurrence Periop antibiotics unnecessary, 4% infection rate
Sistrunk
Sistrunk