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542 Section III / Subspecialty Surgery

MECONIUM PERITONITIS

What is it?

Sign of intrauterine bowel perforation;

 

sterile meconium leads to an intense

 

local inflammatory reaction with eventual

 

formation of calcifications

What are the signs?

Calcifications on plain films

MECONIUM PLUG SYNDROME

What is it?

What is it also known as?

What are the signs/ symptoms?

What is the nonoperative treatment?

What is the major differential diagnosis?

Is meconium plug highly associated with CF?

Colonic obstruction from unknown factors that dehydrate meconium, forming a “plug”

Neonatal small left colon syndrome

Abdominal distention and failure to pass meconium within first 24 hours of life; plain films demonstrate many loops of distended bowel and air-fluid levels

Contrast enema is both diagnostic and therapeutic; it demonstrates “microcolon” to the point of dilated colon (usually in transverse colon) and reveals copious intraluminal material

Hirschsprung’s disease

No; 5% of patients have CF, in contrast to meconium ileus, in which nearly all have CF (95%)

ANORECTAL MALFORMATIONS

What are they?

Malformations of the distal GI tract in

 

the general categories of anal atresia,

 

imperforate anus, and rectal atresia

IMPERFORATE ANUS

 

 

 

What is it?

Congenital absence of normal anus

 

(complete absence or fistula)

 

Chapter 67 / Pediatric Surgery 543

Define a “high” imperforate

Rectum patent to level above

anus.

puborectalis sling

Define “low” imperforate

Rectum patent to below puborectalis

anus.

sling

Which type is much more

Low

common in women?

 

What are the associated

Vertebral abnormalities, Anal abnormalities,

anomalies?

Cardiac, TE fistulas, Esophageal Atresia,

 

Radial/Renal abnormalities, Lumbar

 

abnormalities (VACTERL; most

 

commonly TE fistula)

What are the signs/

No anus, fistula to anal skin or bladder,

symptoms?

UTI, fistula to vagina or urethra, bowel

 

obstruction, distended abdomen,

 

hyperchloremic acidosis

How is the diagnosis made?

What is the treatment of the following conditions:

Low imperforate anus with anal fistula?

High imperforate anus?

HIRSCHSPRUNG’S DISEASE

Physical exam, the classic Cross table “invertogram” plain x-ray to see level of rectal gas (not very accurate), perineal ultrasound

Dilatation of anal fistula and subsequent anoplasty

Diverting colostomy and mucous fistula; neoanus is usually made at 1 year of age

What is it also known as?

Aganglionic megacolon

What is it?

Neurogenic form of intestinal obstruction

 

in which obstruction results from

 

inadequate relaxation and peristalsis;

 

absence of normal ganglion cells of the

 

rectum and colon

What are the associated

Family history; 5% chance of having a

risks?

second child with the affliction

544 Section III / Subspecialty Surgery

 

What is the male to female

4:1

ratio?

 

What is the anatomic

Aganglionosis begins at the anorectal line

location?

and involves rectosigmoid in 80% of cases

 

(10% have involvement to splenic flexure,

 

and 10% have involvement of entire colon)

What are the signs/

Abdominal distention and bilious vomiting;

symptoms?

95% present with failure to pass

 

meconium in the first 24 hours; may also

 

present later with constipation, diarrhea,

 

and decreased growth

What is the classic history?

Failure to pass meconium in the first

 

24 hours of life

What is the differential

Meconium plug syndrome, meconium

diagnosis?

ileus, sepsis with adynamic ileus, colonic

 

neuronal dysplasia, hypothyroidism,

 

maternal narcotic abuse, maternal

 

hypermagnesemia (tocolysis)

What imaging studies should

AXR: reveals dilated colon

be ordered?

Unprepared barium enema: reveals

 

constricted aganglionic segment

 

with dilated proximal segment, but

 

this picture may not develop for 3 to

 

6 weeks; BE will also demonstrate

 

retention of barium for 24 to 48 hours

 

(normal evacuation 10 to 18 hours)

What is needed for

Rectal biopsy: for definitive diagnosis,

definitive diagnosis?

submucosal suction biopsy is adequate in

 

90% of cases; otherwise, full-thickness

 

biopsy should be performed to evaluate

 

Auerbach’s plexus

What is the “colonic

Transition (taper) from aganglionic small

transition zone”?

colon into the large dilated normal colon

 

seen on BE

What is the initial

In neonates, a colostomy proximal to the

treatment?

transition zone prior to correction, to allow

 

for pelvic growth and dilated bowel to

 

return to normal size

Chapter 67 / Pediatric Surgery 545

What is a “leveling” Colostomy performed for Hirschsprung’s colostomy? disease at the level of normally innervated

ganglion cells as ascertained on frozen section intraoperatively

Describe the following procedures:

Swenson Primary anastomosis between the anal canal and healthy bowel (rectum removed)

Duhamel

Anterior, aganglionic region of the

 

rectum is preserved and anastomosed to

 

a posterior portion of healthy bowel; a

 

functional rectal pouch is thereby created

 

(Think: duha dual barrels side by side)

7

0 '

F

R

H

“Dual barrels”

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