PURPOSE: Abdominal wall laxity is typically associated with prune-belly syndrome (PBS).
Incomplete forms of PBS have been rarely reported with only the abdominal wall laxity. Herein, we describe a rare congenital abdominal wall defect that has been confused with PBS and illustrate the laparoscopic-assisted surgical technique used for reconstruction.
MATERIALS AND METHODS: Two boys with symmetrical, bilateral absence or hypoplasia of the internal and external oblique muscles and no genitourinary abnormalities underwent a laparoscopic-assisted abdominal wall reconstruction utilizing the technique previously described by Firlit. Each patient had a Ct scan which confirmed the absence of the oblique muscles. In one patient EMG data confirmed no electrical activity of the obliques. Radiologic evaluation of the urinary tracts revealed no abnormalities. The abdominal wall was plicated utilizing bilateral subcostal incisions.
RESULTS: Both patients had excellent cosmetic and functional results with no weakness or bulging of the lateral abdominal wall and improvement of associated symptoms.
CONCLUSIONS: We believe these two cases and their congenital abdominal wall defects are a rare and often misdiagnosed muscular deficiency separate from PBS. The novel laparoscopic-assisted surgical technique illustrated is feasible and highly successful for these and possible other patients with similar rare congenital abdominal wall defects.
Written by:
Fishman AI, Franco I. Are you the author?
Department of Pediatric Urology, New York Medical College, Valhalla, NY 10595, USA.
Reference: J Pediatr Urol. 2012 Dec 24. pii: S1477-5131(12)00286-0.
doi: 10.1016/j.jpurol.2012.11.012
PubMed Abstract
PMID: 23270915
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