Minimally conjoined omphalopagi: emphasis on embryogenesis and possibility of emergency separation.

Minimally conjoined omphalopagus twins (MCOTs) has been recognized in the last decade as a special subgroup in which omphalopagus twins have union of peritoneal cavities through anterior lower abdominal wall defect with union of distal small intestine and patent urachal structures and associating anorectal malformation. A careful review of the current literature revealed that MCOTs have usually been separated in emergency situations within the first hours of life due to ruptured omphalocele, gastroschisis, stillbirth of one of the twins, intestinal obstruction, or requirement of enterostomy for cloacal anomaly. Pediatric surgeons should be familiar with MCOTs and ready for emergency separation with thorough knowledge of the anatomical relationships of the connecting structures and the embryologic basis for this anomaly. We present a new set of MCOTs separated in emergency conditions with a review of the relevant English literature. We give special emphasis to the common surgical characteristics and a brief discussion on the embryogenesis of this rare condition.

The association of pulmonary lymphangioleiomyomatosis with renal and hepatic angiomyolipomas in a prepubertal girl: a previously unreported entity.

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare, diffuse progressive interstitial lung disease that affects females of childbearing age and is characterized by diffuse proliferation of abnormal smooth muscle fibers predominantly developing in the lung and leading to cystic destruction. A prepubertal 13-year-old girl with PLAM associated with renal and hepatic angiomyolipomas who was treated by nephroureterectomy and thoracoscopic pleurodesis is presented. To the best of our knowledge, this is the first reported case of PLAM associated with renal and hepatic angiomyolipomas at the prepubertal age. After evaluating the clinicopathologic features of this rare entity, the authors conclude that PLAM should be considered in the differential diagnosis of cystic pulmonary pathologies in children, particularly teenagers. The most important clinical aid is to bear this rare entity in mind when a child presents with renal and/or hepatic angiomyolipomas. Thoracoscopic pleurodesis is the most effective treatment modality for recurrent pneumothoraces.