Hypoganglionosis in pregnancy: a case report

Abstract

IntroductionWe report a very rare case of isolated hypoganglionosis first diagnosed during early pregnancy, which should be discussed from an obstetric and a gastroenterological point of view.Case presentationA pregnant 18-year-old Caucasian woman presented at twelve weeks of gestation with lower abdominal pain, mild constipation and a large abdominal mass. Abdominal and pelvic magnetic resonance imaging demonstrated a megarectum and megasigmoid, and our patient was managed with medical therapy during her pregnancy, which occurred without major incidents. At the onset of labor, a fecaloma obstructing the pelvic outlet was detected, which required manual disimpaction. However, during the procedure a sudden continuous fetal bradycardia was detected. An emergency Cesarean section was performed but the fetus suffered hypoxic ischemic encephalopathy. One year after the delivery, our patient underwent a sigmoid resection. A histopathological analysis revealed a reduction of nerve cells in the myenteric and submucous plexus, suggesting hypoganglionosis.ConclusionAlthough there are some reports of pregnancies complicated by megacolon, they are too few and too old to delineate guidelines for clinical orientation. In our article, we discuss several issues regarding the management of these rare intestinal innervation disorders during pregnancy that we believe will enhance their obstetric and gastroenterological management during pregnancy.

Megarectum in pregnancy causing coital penetration failure

“…Laxatives of choice would be osmotic laxative rather than stimulant. 11 Examples are lactulose, magnesium salts, polyethylene glycol or sorbitol. 11 Towards later gestation, the issue regarding mode of delivery should be discussed and planned well ahead of time especially if the faecaloma obstructed the pelvic outlet.…”

“…11 Examples are lactulose, magnesium salts, polyethylene glycol or sorbitol. 11 Towards later gestation, the issue regarding mode of delivery should be discussed and planned well ahead of time especially if the faecaloma obstructed the pelvic outlet. If vaginal delivery is contemplated, one has to be ready to perform manual faecal disimpaction.…”

“…This procedure is done during labour to facilitate vaginal delivery, however it could also be associated with foetal compromise. 11 In many cases, assisted instrumental deliveries via forceps or ventouse are required. 11 If abdominal delivery is planned, care should be taken upon entering the peritoneal cavity in order to avoid injury to the redundant colon.…”

“…11 In many cases, assisted instrumental deliveries via forceps or ventouse are required. 11 If abdominal delivery is planned, care should be taken upon entering the peritoneal cavity in order to avoid injury to the redundant colon. 12 Management in our case was much easier as the pregnancy did not progress to term.…”

“…9 Transanal rectal biopsy plays important role to identify aganglionosis associated with Hirschprung's disease and this procedure can be safely performed during pregnancy under spinal anaesthesia. 11 Almost all cases of megarectum required rectal and to some extent colonic resection to rectify the condition. 14 Performing such operation during pregnancy however is associated with miscarriage and should be delayed until postpartum period.…”