Hirschsprung
Maternal magnesium sulfate or other tocolytic medications can cause significant ileus in the newborn that mimics Hirschsprung's disease.
clinicalMarc Levitt0:55 ↗
Hypothyroidism and opiate exposure (maternal addiction or overdose transmitted to baby) can present with abdominal distension and delayed meconium passage mimicking Hirschsprung's disease.
clinicalMarc Levitt0:55 ↗
Milk protein allergy in a fed baby can mimic Hirschsprung's disease on x-ray, and irrigations help even when it is not Hirschsprung's.
clinicalMarc Levitt0:55 ↗
Ten percent of patients with meconium plug have Hirschsprung's disease, so suction rectal biopsy should be performed even after successful plug passage.
epidemiologicalMarc Levitt2:00 ↗
After meconium plug passage, the contrast study should be repeated because the initial study with plug present will show dilated rectosigmoid, but the repeat study will reveal the transition zone more clearly.
clinicalMarc Levitt3:00 ↗
In total colonic Hirschsprung's disease, there is often no obvious transition zone on contrast study.
clinicalMarc Levitt3:00 ↗
Suction rectal biopsy is the gold standard for diagnosing Hirschsprung's disease, and some centers proceed directly to biopsy without contrast study.
clinicalMarc Levitt4:00 ↗
Contrast study serves as a surgical GPS map of the colon anatomy and is preferred before operating, even though biopsy alone can rule out Hirschsprung's.
opinionMarc Levitt4:00 ↗
The physiologic obstruction in Hirschsprung's disease leads to stasis, bacterial overgrowth, and bacterial translocation because mucosal integrity is abnormal.
clinicalMarc Levitt4:42 ↗
A constipated baby without Hirschsprung's disease will not develop enterocolitis because their mucosal integrity and IgA levels are normal.
clinicalMarc Levitt4:42 ↗
Down syndrome patients with Hirschsprung's disease have worse enterocolitis because they have a weaker immune barrier.
clinicalMarc Levitt4:42 ↗
Irrigation protocol: use a 20 French soft silicone Foley catheter with warm saline in 10-20cc aliquots, washing the inside of the colon by injecting, allowing drip-back, advancing the catheter a few centimeters, and repeating for up to 30 minutes.
clinicalMarc Levitt5:44 ↗
Irrigations should be performed two to three times per day and require a written protocol so nurses know exactly what is expected.
clinicalMarc Levitt5:44 ↗
If irrigations are performed correctly but do not reach the transition zone, the baby will remain distended and irrigations will not be effective.
clinicalMarc Levitt5:44 ↗
When irrigations fail despite correct technique, the surgeon must decide between a leveling colostomy (bringing dilated bowel to surface, ideally with frozen section confirmation) or an ileostomy with colonic biopsies.
clinicalMarc Levitt7:00 ↗
Ileostomy does not require intraoperative pathology at 3 AM, the baby will thrive, and full colonic pathology data can be obtained later, but it requires three operations instead of two.
clinicalMarc Levitt7:30 ↗
In regions where ileostomy management is difficult and babies can dehydrate quickly, ileostomy may not be appropriate.
clinicalMarc Levitt7:30 ↗
A leveling colostomy is usually accurate when placed in dilated bowel to the surgeon's eye, but the risk is opening a colostomy still in the transition zone.
clinicalMarc Levitt7:30 ↗
A loop ileostomy where both limbs are equal is inappropriate because stool will jump across and fill the aganglionic segment, causing continued distension and potential enterocolitis despite diversion.
clinicalMarc Levitt9:26 ↗
A Turnbull ileostomy (cutting the bowel completely on one side of the loop, intussuscepting it over a finger to create an end-stoma appearance with flat distal opening) or a divided ileostomy with proximal end brought up and distal limb tacked to it are both appropriate diversion techniques.
clinicalMarc Levitt9:26 ↗
Post-pull-through patients present with two distinct problems: obstruction (chronic distension, enterocolitis episodes, failure to thrive) or soiling (no distension, no enterocolitis, uncontrolled stooling).
clinicalMarc Levitt11:51 ↗
Anatomic causes of post-pull-through obstruction include inadequate Soave cuff (not cut enough, rolled up, or refused), twisted pull-through (180 or 360 degrees), Duhamel spur (two lumens not successfully joined), large Duhamel pouch reaching into pelvis, stricture, and transition zone pull-through.
clinicalMarc Levitt12:40 ↗
A Soave cuff feels like a rubbery circumferential ring outside the pull-through on digital exam.
clinicalMarc Levitt12:40 ↗
A twisted pull-through prevents the examining finger from entering the pelvis; on abdominal palpation you feel like you are hitting a wall.
clinicalMarc Levitt12:40 ↗
Contrast study may show a cuff by revealing extra presacral space on lateral view; the pull-through should hug the sacrum, and a cuff pushes it forward.
clinicalMarc Levitt12:40 ↗
In Duhamel patients, biopsy must be taken from the posterior wall because the anterior wall is the original aganglionic rectum.
clinicalMarc Levitt15:00 ↗
If anatomic causes and pathology are normal in an obstructed post-pull-through patient, the problem is non-relaxing sphincters, and empiric Botox is appropriate.
clinicalMarc Levitt15:00 ↗
Every child born with Hirschsprung's disease has 100% potential for bowel control because the continence mechanism is normal and the sphincters are strong, not weak.
clinicalMarc Levitt16:13 ↗
This is very different from anorectal malformation, where associated spinal problems, poor sacrum, or poor muscles can prevent continence despite a perfect operation.
clinicalMarc Levitt16:13 ↗
Overstretching of sphincters or starting transanal dissection too low (removing the dentate line) will cause iatrogenic fecal incontinence.
clinicalMarc Levitt17:10 ↗