Imperforate anus, multiple intestinal atresia and unilateral facial hypoplasia: A case series

Abstract

Introduction

Imperforate anus and intestinal atresia are rare but well understood congenital disorders which affect the alimentary tract. Like intestinal atresia, facial hypoplasia is another congenital anomaly likely secondary to a vascular injury in utero.

Case presentation

Case 1 discusses a female born at 32 weeks with a prenatal diagnosis of duodenal atresia and discovery of imperforate anus and unilateral maxillary hypoplasia at birth. She was emergently taken to the operating room for bowel obstruction. During that operation, she underwent repair of multiple segments of atresia, a duodenoduodenostomy, and creation of an end ileostomy and mucus fistula. At two months of age, she returned to the operating room to restore continuity of the distal atretic bowel. At six months old she presented with a partial bowel obstruction secondary to a jejunal duplication cyst. Case 2 discusses a full-term male presenting with unilateral facial hypoplasia and imperforate anus at birth. Given failure to pass meconium and worsening abdominal distension he was taken to surgery, where multiple segments of intestinal atresia were identified. During this operation he was left in discontinuity following creation of a diverting loop ileostomy and colostomy. Two months later, he underwent repeat exploratory laparotomy during which multiple segments of atretic bowel were repaired. At four months of age, he underwent reversal of the diverting loop ileostomy followed by completion of posterior sagittal anorectoplasty at nine months old.

Conclusion

Intestinal atresias should be highly suspected in patients who present with anorectal malformations in conjunction with facial hypoplasia.