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546 Section III / Subspecialty Surgery

Soave

What is the new trend in surgery for Hirschsprung’s disease?

What is the prognosis?

A.k.a. endorectal pull-through; this procedure involves bringing proximal normal colon through the aganglionic rectum, which has been stripped of its mucosa but otherwise present (Think: SOAVE SAVE the rectum, lose the mucosa)

No colostomy; remove aganglionic colon (as confirmed on frozen section) and perform pull-through anastomosis at the same time (Boley modification)

Overall survival rate 90%; 96% of patients continent; postoperative symptoms improve with age

MALROTATION AND MIDGUT VOLVULUS

What is it?

Failure of the normal bowel rotation,

 

with resultant abnormal intestinal

 

attachments and anatomic positions

Where is the cecum?

With malrotation, the cecum usually ends

 

up in the RUQ

Chapter 67 / Pediatric Surgery 547

What are Ladd’s bands? Fibrous bands that extend from the abnormally placed cecum in the RUQ, often crossing over the duodenum and causing obstruction

 

Ladd’s

 

 

 

 

 

 

 

bands

 

 

 

 

 

7

 

 

 

0

 

 

 

HRF

 

What is the usual age at

33% are present by 1 week of age, 75%

onset?

by 1 month, and 90% by 1 year

 

What is the usual

Sudden onset of bilious vomiting (bilious

presentation?

vomiting in an infant is malrotation

 

until proven otherwise!)

 

Why is the vomiting bilious?

“Twist” is distal to the ampulla of Vater

How is the diagnosis made?

Upper GI contrast study showing cutoff

 

in duodenum; BE showing abnormal

 

 

position of cecum in the upper abdomen

What are the possible

Volvulus with midgut infarction, leading to

complications?

death or necessitating massive enterectomy

 

(rapid diagnosis is essential!)

 

What is the treatment?

IV antibiotics and fluid resuscitation with

 

LR, followed by emergent laparotomy with

 

Ladd’s procedure; second-look laparotomy

 

if bowel is severely ischemic in 24 hours to

 

determine if remaining bowel is viable

 

548 Section III / Subspecialty Surgery

 

 

What is the Ladd’s

1.

Counterclockwise reduction of

procedure?

 

midgut volvulus

 

2.

Splitting of Ladd’s bands

 

3.

Division of peritoneal attachments to

 

 

the cecum, ascending colon

 

4.

Appendectomy

In what direction is the

Rotation of the bowel in a

volvulus reduced—clockwise

counterclockwise direction

or counterclockwise?

 

 

Where is the cecum after

LLQ

reduction?

 

 

What is the cause of bilious

Malrotation with midgut volvulus

vomiting in an infant until

 

 

proven otherwise?

 

 

OMPHALOCELE

 

 

 

 

What is it?

Defect of abdominal wall at umbilical

 

ring; sac covers extruded viscera

How is it diagnosed

May be seen on fetal U/S after 13 weeks’

prenatally?

gestation, with elevated maternal AFP

What comprises the “sac”?

Peritoneum and amnion

What organ is often

The liver

found protruding from

 

 

an omphalocele, but is

 

 

almost never found with a

 

 

gastroschisis?

 

 

What is the incidence?

1 in 5000 births

How is the diagnosis made?

Prenatal U/S

What are the possible

Malrotation of the gut, anomalies

complications?

 

 

What is the treatment?

1.

NG tube for decompression

 

2.

IV fluids

 

3.

Prophylactic antibiotics

 

4.

Surgical repair of the defect

What is the treatment of a small defect ( 2 cm)?

What is the treatment of a medium defect (2–10 cm)?

Chapter 67 / Pediatric Surgery 549

Closure of abdominal wall

Removal of outer membrane and placement of a silicone patch to form a “silo,” temporarily housing abdominal contents; the silo is then slowly decreased in size over 4 to 7 days, as the abdomen accommodates the viscera; then the defect is closed

What is the treatment of “giant” defects ( 10 cm)?

Omphalocele

Silastic silo

Omphalocele

reduced

4 '0

HR

Skin flaps or treatment with Betadine® spray, mercurochrome, or silver sulfadiazine (Silvadene®) over defect; this allows an eschar to form, which epithelializes over time, allowing opportunity for future repair months to years later

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