Bilateral Wilm's Tumor - Complex Gastroschisis - Complex Ileal Atresia:...

Current standard for bilateral Wilms tumor is to start chemotherapy, give 2 cycles, and stop when tumor shrinkage plateaus (defined as less than 50% volume reduction).

When chemotherapy fails to shrink bilateral Wilms tumors and they have not been biopsied, changing to a more aggressive chemotherapy regimen should be considered to rule out anaplastic elements that might respond.

Biopsy of Wilms tumor is problematic because these are heterogeneous tumors and focal anaplasia may be missed on needle biopsy.

When Wilms tumors stop shrinking with chemotherapy, it is usually due to mesenchymal differentiation rather than anaplastic transformation.

Bilateral nephron-sparing surgery for Wilms tumor can be performed by placing kidneys on ice, clamping vessels, and performing sharp tumor dissection—surprisingly feasible even for large tumors.

Anaplastic recurrence of Wilms tumor portends a very bad outcome; salvage is difficult even with chemotherapy.

It is debated whether anaplasia in Wilms tumor is present initially or develops secondary to chemotherapy-induced differentiation; most pathologists believe it is present primarily, making these heterogeneous tumors.

Multifocal Wilms tumors raise concern about underlying embryologic kidney abnormalities and increased risk of developing additional tumors.

97% of bilateral kidney tumors in children are Wilms tumor, so biopsy is not needed upfront—chemotherapy should be started empirically.

For gastroschisis, bedside reduction can be attempted under sedation (rectal acetaminophen and small-dose fentanyl) without intubation, with success in approximately 80% of cases even when all bowel is eviscerated.

Gastroschisis babies are being delivered earlier by high-risk obstetric teams, resulting in less thick matted bowel at birth and higher rates of successful bedside reduction.

For gastroschisis closure using Tegaderm technique, if the fascia is not cut to widen the defect, most umbilical hernias will close spontaneously and do not require repair.

Spring-loaded Bianchi silos may enlarge the fascial defect because the compressive forces are directed outward at the ring level.

Tegaderm can be applied directly over reduced gastroschisis without Betadine or other topical agents, left in place for approximately 3 days, then converted to dry dressing once tissues are adherent.

Even when a gastroschisis silo fails and the defect dilates significantly, Tegaderm-only closure can achieve successful cicatrization over 8 weeks.

For gastroschisis with intestinal atresia and pristine bowel, an ostomy can be created at the umbilical fascial ring, with the downstream limb tacked adjacent, allowing for delayed anastomosis and ostomy closure after 2-4 weeks.

For gastroschisis with atresia and thick inflamed bowel, the bowel should be reduced into the abdomen without repair, allowing inflammation to resolve, then re-explored at 4-6 weeks.

Primary anastomosis of intestinal atresia in gastroschisis is feasible if the bowel is pristine, but the size discrepancy (massively dilated proximal, decompressed distal) creates a tenuous anastomosis in bowel that has been outside the abdomen.

When gastroschisis is reduced without closing the fascia, ambient intra-abdominal pressure is lower than with fascial closure, which may benefit bowel function.

In vanishing gastroschisis with markedly inflamed bowel, it is impossible to determine bowel viability or the presence of atresia from external appearance—exploration is necessary but resection should be avoided.

Re-exploration at 2 weeks after initial gastroschisis reduction (rather than the traditional 4-6 weeks) can reveal that inflamed 'gastroschisis bowel' has transformed into viable intestine suitable for anastomosis.

For apple-peel ileal atresia with ischemic distal bowel, if the bowel does not pink up on the operating table and is not twisted, resection with primary anastomosis is appropriate.

If apple-peel bowel is ischemic (not necrotic) and not twisted, waiting until the next day to reassess viability is reasonable before committing to resection.

Plication of massively dilated proximal bowel in intestinal atresia is helpful and the plication typically unravels over time, preserving bowel length for later lengthening procedures if needed.

Bianchi procedure is preferred over STEP for neonatal bowel lengthening because it creates two equal chambers without blind-ending outpouchings, and a patient can undergo Bianchi followed by STEP later, whereas STEP limits future lengthening options.

In newborns with questionable short-gut syndrome, tapering should be avoided to preserve bowel for potential lengthening procedures later; plication is preferred.

If a child has extensive bowel length and only a segment is dilated, tapering is reasonable.