Congenital and Dev Disorders Flashcards

1
Q

Antenatal imaging procedure to detect gastroschisis?

A

Prenatal ultrasound

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2
Q

Management options for Gastroschisis?

A

Primary reduction (Double set-up)
Silastic silo

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3
Q

Gastroschisis is most strongly associated with which anomaly/anomalies?

A

Bowel abnormalities, atresia or stenoses

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4
Q

Interventions for Gastroschisis patients made prior to transfer?

6 steps

A
  1. Wrap bowels in clean kitchen wrap (bowel bag)
  2. Maintain normothermic
  3. NPO
  4. IV Fluid
  5. NGT
  6. Explain management to parents
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5
Q

Appropriate management of gastroschisis after primary reduction?

A

Observation

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6
Q

Long term complications after recovery from gastroschisis?

A
  1. Short Bowel Obstruction
  2. Short Gut Syndrom
  3. CV catheter-related sepsis
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7
Q

Sac is covered with peritoneum without overlying skin and insertion of distal umbilical cord.

A

Omphalocele

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8
Q

Management and Treatment for Omphalocele

A
  1. Wrap in moist, sterile dressings
  2. Prompt surgical repair
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9
Q

Omphalocele > 10cm

A

Giant omphalocele

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10
Q

How can you diagnose omphalocele prenatally?

A
  1. Ultrasonography
  2. Maternal a-fetoprotein (increased)
  3. Amniocentesis with chromosomal studies (for congenital anomalies)
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11
Q

Patient presents with macroglossia, what syndrome is most likely present?

A

Beckwith-Wiedemann chromosomal syndrome
(umbilical defect, gigantism, macroglossia, visceromegaly, pancreatic islet cell hyperplasia)

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12
Q

Other coexisting abnormalities commonly seen in patients with omphalocele?

A
  1. Cardiac anomalies - VSD, ASD, TOF, Ectopia cordis
  2. GI anomalies - Duodenal atresia (common in gastroschisis)
  3. Malignant tumor, neuroblastoma, adrenocortical tumors
  4. Chromosomal anomalies
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13
Q

Pre-operative interventions for patients with omphalocele

7 steps

A
  1. NGT with cont. drainage
  2. Sterile dressing w/ moist towels
  3. Plastic foilage
  4. Lateral position
  5. IV access
  6. Transport in heated incubator
  7. Rectal irrigation - NSS
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14
Q

Treatment options for omphalocele

A
  1. Reduce ophalocele, repair abdominal wall
  2. Immediate operation
  3. Excision of the sac
  4. Cylindrical tube (silo)
  5. Non-operative management
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15
Q

Absence of an anal opening

A

Imporforate anus

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16
Q

Initial laboratory tests and imaging for patients with imporforate anus?

A
  1. Cross-table lateral radiograph (invertogram)
  2. Xray of spine and chest
  3. Ultrasonography
  4. ECG
  5. MRI
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17
Q

What are the usual associated anomalities in patients with anorectal malformations?

A

VACTERL association
1. Vertebral/spinal cord abnormalities
2. Anorectal malformations
3. Congenital cardiac disease
4. TEF
5. Esophageal atresia
6. Renal anomalies
7. Limb defects

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18
Q

Diagnostic test/Imaging for Vertebral/spinal abnormalities?

A

Xray (Babygram), MRI for spinal injury

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19
Q

Diagnostic test/Imaging for Anorectal malformation?

A

Physical Exam

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20
Q

Diagnostic test/Imaging for Congenital cardiac disease?

A

2D Echo

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21
Q

Diagnostic test/Imaging for TEF/Esophageal atresia?

A

NGT insertion resistance
Drooling/excessive salivation

22
Q

Diagnostic test/Imaging for renal anomalies?

A

Renal ultrasound

23
Q

Diagnostic test/Imaging for limb defects?

A

Observation, PE

24
Q

Plans and definitive management for patients with imporforate anus?

A

1st - Colostomy
2nd - Posterior Sagittal Anorectoplasty (after 4-6 months)

25
Q

What are the pertinent information on history and PE of a patient with intestinal atresia?

A
  1. Vomiting (emesis)
  2. Polyhydramnios
  3. Association with congenital anomaly (Down Syndrome)
26
Q

Differential diagnosis of Intestinal atresia?

A
  1. Duodenal atresia
  2. Malrotation (Vovulus)
  3. Jejunal atresia
27
Q

Imaging signs seen in patients with intestinal atresia?

A

Double bubble sign (duodenal atresia, malrotation) More bubbles in jejunal atresia

28
Q

Diagnostic examinations for intestinal atresia?

A
  1. Babygram AP, cross-table lateral = Double bubble sign
  2. 2D Echo
  3. KUB ultrasound
  4. Chromosomal analysis
29
Q

Treatment modalities for intestinal atresia

A

SUPPORTIVE
1. OGT insertion
2. IV Fluids
3. CBC, Blood typing, electrolytes
SURGICAL
1. Exploratory laparotomy, repair of duodenal atresia

30
Q

Possible complications of intestinal atresia?

A
  1. VACTERL
  2. Anastomotic leak
31
Q

Presence of palpable mass on the inguinoscrotal area

A

Inguinal hernia

32
Q

Differential diagnosis when inguinoscrotal mass is present?

A
  1. Inguinal hernia
  2. Hydrocele
  3. Testicular torsion
  4. Inguinal lymphadenopathy
33
Q

Diagnostic tests for inguinal hernia?

A
  1. Inguinoscrotal ultrasound - defect on the floor of inguinal area, peristalsis/bulging omentum
34
Q

3 types of Inguinal hernia based on severity

A
  1. Reducible
    *2. Incarcerated - non reducible, obstruct site
    *3. Strangulated - compromised blood supply, non-viable, necrotic

* TRUE SURGICAL EMERGENCIES

35
Q

Treatment modalities for inguinal hernia

A

SUPPORTIVE
1. IV Fluids
2. Antibiotics
SURGICAL
1. Inguinal exploration, herniotomy

36
Q

BEST time to operate on inguinal hernia?

A

On diagnosis
ASAP for incarcerated and strangulated

37
Q

Complication of inguinal hernia and surgical intervention?

A

Strangulation

38
Q

Most common/Initial symptom of patients with Meckel’s diverticulum?

A

Hematochezia / Massive painless GI bleeding

39
Q

Differential diagnosis of Meckel’s diverticulum

A
  1. Bleeding Meckel’s diverticulum
  2. Bloody diarrhea
  3. Intussusception
  4. Others (Trauma, anal fissure, juvenile polyp)
40
Q

Diagnostic examinations for Meckel’s diverticulum?

A

CBC, blood typing
Meckel’s diverticulum scintigraphy / Meckel’s scan
Colonoscopy

41
Q

Meckel’s diverticulum scintigraphy results

A

(+) on left upper quadrant (stomach) - normal
(+) on left lower quadrant - Meckel’s diverticulum

42
Q

Treatment modalities for Meckel’s diverticulum?

A

Supportive
1. IV Fluids
2. Blood transfusion of pRBC - correction of anemia and blood loss
3. Antibiotics based on px. clinical picture
Surgical
1. Wedge-resection anastomosis of diverticulum via laparoscopic-assisted or open technique

42
Q

Treatment modalities for Meckel’s diverticulum?

A

Supportive
1. IV Fluids
2. Blood transfusion of pRBC - correction of anemia and blood loss
3. Antibiotics based on px. clinical picture
Surgical
1. Wedge-resection anastomosis of diverticulum via laparoscopic-assisted or open technique

43
Q

Possible complications of Meckel’s diverticulum?

A
  1. Hypovolemic shock (failure of supportive treatment)
  2. Anastomotic leak
44
Q

Rule of 2s: Meckel’s diverticulum

A

Incidence is roughly 2% of the population
If symptomatic clinically recognized at 2 y/o
2 inches in maximal dimension
Located 2 ft of the ileocecal valve

45
Q

Common location of Meckel’s diverticulum?

A

Ileocecal valve (2 ft)

46
Q

Vomiting, severe pain (intermittent colicky abdominal pain, blood-tinged mucoid stools, presence of mass (RLQ/LLQ)

A

Intussusception

47
Q

Differential diagnosis of intussusception

A
  1. Intussusception
  2. Acute gastroenteritis
  3. Strangulated inguinal hernia
48
Q

Initial management for intussusception

A
  1. IV Fluids
  2. NGT decompression
  3. IV antibiotics
  4. CBC, chem panel, blood typing
49
Q

Imaging modalities for intussusception

A
  1. Abdominal xray
  2. Ultrasound - donut/target sign on transverse, pseudokidney on longitudinal
    3. Barium/air contrast enema - GOLD STANDARD
50
Q

Definitive treatment for intussusception

A
  1. Surgical exploration
  2. Reduction of intussusception
  3. Appendectomy (optional)
  4. Local / segmental resection
  5. Primary anastomosis
51
Q

Golden period of intussusception

A

6-8 hours