Association of intestinal malrotation and Bochdalek hernia in an adult: a case report

Abstract

BackgroundLate presentations of congenital diaphragmatic hernia are rare and differ from the classic neonatal presentation. The association with other congenital malformations in children, mainly intestinal malrotation, is well documented. The diagnosis of this association in adults is very rare, and depends on a high degree of suspicion.Case presentationWe report a case of a 50-year-old female Caucasian patient with a previous history of intestinal malrotation diagnosed in adolescence and treated conservatively. She was referred to the hospital with signs and symptoms of intestinal obstruction. The patient undertook computed tomography that confirmed small bowel obstruction with no obvious cause, and a right subphrenic abscess with right empyema was also present. An exploratory laparotomy was performed that revealed an intestinal malrotation associated with a right gangrenous and perforated Bochdalek hernia. Resection of the affected small bowel, closure of the Bochdalek foramen and the Ladd procedure were carried out.ConclusionThis case shows a rare association of two rare conditions in adults, and highlights the challenge in reaching the diagnosis and management options.

Right‐sided Bochdalek hernia in an elderly patient: a case review of adult Bochdalek hernias from 1982 to 2015 in Japan

“…However, if the foramen is small, herniation may not easily occur, and BH may remain asymptomatic until herniation occurs later in life. This mechanism may account for the presentation of adult BH …”

“…The optimal strategy for BH surgical treatment is controversial . Authors who are in favor of the transabdominal approach emphasize its facility to not only reduce the hernia contents to the abdominal cavity but also to deal with complications such as obstruction, strangulation, perforation, or coexisting congenital anomaly . In contrast, the transthoracic approach is advocated by other authors because of the convenience of dissecting adhesions between the pleura and hernia contents .…”

“…Congenital diaphragmatic hernia is a congenital anomaly caused by embryonic developmental failure of the diaphragm, and Bochdalek hernia (BH) accounts for approximately 95% of cases . Most cases of BH present with acute cardiorespiratory failure during the neonatal period; thus, BH in adults is unusual . Regarding the side of occurrence, 80–90% of BH arise on the left side .…”

Small bowel obstruction in an adult patient with situs ambiguous and mid gut malrotation

“…Presentation with chronic abdominal pain, vomiting, diarrhea, abdominal distension and early satiety is common. Acute presentations with intestinal obstruction due to small bowel volvulus are less common [4].…”

Malrotation and abdominal pain: A diagnosis eluding the unprepared mind

“…Another minority expresses as a chance finding during imaging or surgery for some other cause. Cases do occasionally present due to associated anomalies like congenital diaphragmatic hernia [2], esophageal atresia [3], etc.…”