Baba Aejaz A, Hussain Syed A, Shera Altaf H, Patnaik Rekha
Department of Pediatric Surgery, SKIMS, Srinagar, Kashmir, India.
Afr J Paediatr Surg. 2010 Jan-Apr;7(1):25-7. doi: 10.4103/0189-6725.59356.
Prune belly syndrome (PBS) is a rare congenital constellation of defects in pediatric surgical practice. Although anorectal anomalies have been reported in association with PBS, only few case of pouch colon with PBS has been reported. [1] In addition, our patient had deficient abdominal wall with absent dermatome in left upper quadrant, which has never been reported in the English literature. This association with abdominal wall deficiency and absent dermatome not only strengthens the theory of mesodermal arrest in the etiology of PBS but also points towards a defect in the ectodermal development.
梅干腹综合征（PBS）是小儿外科实践中一种罕见的先天性缺陷组合。虽然已有报道肛门直肠畸形与PBS相关，但仅有少数袋状结肠合并PBS的病例报道。[1]此外，我们的患者腹壁缺损，左上象限皮肤节段缺失，英文文献中从未有过此类报道。这种腹壁缺损与皮肤节段缺失的关联不仅强化了PBS病因中中胚层发育停滞的理论，也指向了外胚层发育的缺陷。
