Segmental absence of intestinal musculature with metachronous bowel perforations in an infant

Segmental absence of intestinal musculature is a rare condition. A female patient was born at 39 weeks gestational age with birth weight of 2,900 g. The patient was prenatally diagnosed as having segmental bowel distension in the fetal stage. She manifested bilious emesis with abdominal distension at day 1. Although excretion of viscous meconium was observed by gastrografin enema, gastrointestinal perforation developed. Emergency laparotomy and peritoneal drainage was required at that time and further laparotomy was performed on day 15. Multiple perforations were recognized discontinuously from the jejunum to the transverse colon, and jejunostomy was constructed. Additional bowel perforations occurred and re-exploration was required at day 43. We found newly formed small perforations in the proximal jejunum, ileum and the transverse colon and a tube jejunostomy and a colostomy were established. The patient required prolonged TPN management, which induced correlated cholestasis and liver failure, and died at day 143. Pathologic findings showed partial hypoplasia of the intrinsic muscle layer in the small intestine and diagnosed as segmental absence of intestinal musculature. Her disorder was unusual in its presentation, which included prenatal bowel dilatation, metachronous superimposed bowel perforation, and extensive discrete lesions from the jejunum to the transverse colon

Excision of recurrent appendix testis torsion

A 6-year-old boy developed left side scrotal pain. We diagnosed the patient as having left appendix testis torsion. We prescribed acetaminophen and the pain gradually improved. Four months after the initial episode of pain, the patient had scrotum pain again. Ultrasonography revealed a swollen appendage at the upper pole of the testis with calcification. We performed resection of the twisted appendix testis. Pathological examination revealed lymphocytic infiltration, fibrosis, edema, granulation tissue, and frequent foci of calcification. There was no necrosis. Some cases of appendix testis torsion whose initial symptoms are conservatively managed, require subsequent removal of the chronically inflamed appendage accompanied by acute inflammation for persistent or recurrent pain several months after appearance and resolution of the initial symptoms. Keywords: Acute scrotum, Torsion of appendix testis, Recurrence symptom