What is the role of surgery in the treatment of pediatric omphalocele?

Updated: Nov 05, 2019
  • Author: James G Glasser, MD, MA, FACS; Chief Editor: Dharmendra J Nimavat, MD, FAAP  more...
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Answer

Ambrose Pare, a 17th-century French surgeon, accurately described the adverse consequences of opening the omphalocele sac during closure of the abdominal wall defect. [44] He advocated squeezing the sac to reduce the herniated viscera and, if reduction was not possible, painting the sac with escharotic agents to promote epithelialization and wound contracture. However, healing is slow utilizing this technique, and during this time, the sac may rupture and the infant succumb to infection.

Healing is hastened by mobilizing skin flaps to cover the omphalocele sac (Gross technique); however, this results in the creation of a large ventral hernia.

In 1967, Schuster developed a more expeditious technique to close giant omphaloceles and large ventral hernias. [45] A circumferential incision is made along the perimeter of the omphalocele, leaving the sac intact to protect the herniated viscera. The incision is extended in the midline, exposing the rectus fascia from xiphoid to pubis. Silon (Teflon-reinforced Silastic) sheets are sutured to the rectus muscles and approximated in the midline over the omphalocele sac. Reduction is effected by gradually approximating the Silon sheets, which pulls the rectus muscles over the liver and intestines. When the ventral bulge is leveled, the Silon sheets are removed with the omphalocele sac; permanent closure is obtained by approximating the rectus fascia or sewing an elliptical patch to the fascia and then covering the patch with skin flaps. If this is not feasible, biologic mesh may be placed over the liver and intestines. Ultimately, the patch will be vascularized and support the ingrowth of epithelial cells. (See the image below.)

Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). Silon sheets are pulled over the omphalocele sac, elevating the rectus muscles, and, because of their attachment to the costal arch, expanding the thoracic cavity. The Silon sheets are removed and replaced by a permanent Gore-Tex patch that is covered by skin flaps.

The use of a rigid patch is preferable (Gore-Tex, Surgisis). [39] The ellipse is tailored to give the anterior abdominal wall a concave appearance, lessening pressure upon the diaphragm. The patch is attached to the defect circumferentially: superiorly to the costal arch, inferiorly to the pubis, and laterally to the rectus fascia. Inflation of the lungs elevates the costal arch, which expands both the chest and the abdomen; the increased intra-abdominal pressure stretches the rectus muscles and stimulates their growth.

Dr Russell Jennings of Boston's Children's Hospital has developed an innovative technique whereby upward traction is applied to the silo housing the extruded viscera. The silo is firmly attached (sewn) to the circumference of the abdominal wall defect, and excess skin is attached to the silo. Upward traction stimulates growth of the abdominal wall over the extruded viscera, as opposed to pushing the extruded viscera into an undersized abdominal cavity. [46] (See the following images.)

 

Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). Rather than pushing the extruded viscera into the diminutive abdominal cavity, traction is applied to the abdominal wall and skin flaps. This stimulates growth and facilitates reduction of the extruded visceral and ultimate closure of the abdominal wall defect. Image courtesy of Russell Jennings, MD, Boston Children's Hospital.
Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). This photograph depicts progress in reducing the extruded viscera utilizing Dr Jennings's innovative technique. Image courtesy of Russell Jennings, MD, Boston Children's Hospital.

Synthetic patches (Gore-Tex) require skin coverage or they will ultimately be rejected. Biologic mesh (Alloderm, acellular human dermis; or Surgisis, intestinal submucosa) provides a scaffold for ingrowth of autogenous cells; hence, the body does not perceive them as foreign, and they are not as prone to infection and rejection. Alloderm stretches, and the ultimate result is a large, but epithelialized protrusion that ultimately will require repair. [47, 48]

“Component separation” is a technique pioneered in adult patients to repair ventral hernias. Bilateral incisions are made along the Spigelian line (semilunar line) to allow medial displacement of the rectus fascia and closure of the abdominal wall defect. More recently, this technique has been utilized in children. [49]

See the images below.

 

Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). Closure of a giant omphalocele with an Alloderm patch is shown.

 

Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). This image was obtained 2 months after implantation, revealing epithelialization of the Alloderm patch.

 

Pediatric omphalocele and gastroschisis (abdominal Pediatric omphalocele and gastroschisis (abdominal wall defects). Eight months after implantation, epithelialization is nearly complete, but a huge ventral hernia has developed.

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