Right Upper Quadrant Pain in a 21-Year-Old Man

Abstract

Question: A 21-year-old man with attention deficit hyperactivity disorder developed acute-onset, severe, right upper quadrant pain shortly after being taken into police custody. He volunteered no further past medical history or gastrointestinal symptoms other than pain. On examination, vital signs were: Heart rate, 68; blood pressure, 110/74 mmHg; temperature 37.5°C; respiratory rate, 14; and SpO2, 97% on room air. There was generalized guarding with marked rebound tenderness maximally in the right upper quadrant; electrocardiograph showed normal sinus rhythm and erect chest x-ray did not demonstrate a pneumoperitoneum. Bloods included: Hemoglobin, 16.3 g/dL; white cell count, 6.8 × 109/L; urea, 2.8 mmol/L; creatinine, 71 μmol/L; bilirubin, 17 μg/L; alkaline phosphatase, 65 U/L; alanine transferase, 10 U/L; and C-reactive protein, 48 mg/L; all other blood results were unremarkable. A provisional diagnosis of an acute appendicitis was made and he was taken to the operating room without further imaging, where the following was found (Figure A, B). What is the diagnosis? See the Gastroenterology web site () for more information on submitting your favorite image to Clinical Challenges and Images in GI. On laparoscopy, a classical mid-gut malrotation was found with a centrally positioned caecum. In addition, a large Meckel's diverticulum was seen arising from the ileum behind Ladd's bands in the right upper quadrant (Figure A). On open conversion, Ladd's bands were divided and the gut mobilized to the anatomical position. Given the broad-based diverticulum, small bowel resection with end-to-end anastomosis was performed, together with cecopexy and appendectomy. Histologic examination revealed Meckel's diverticulitis with areas of ectopic duodenal mucosa (Figure B) and minor regenerative changes throughout adjacent small bowel. Later, on gaining a collateral history, it transpired that he had a lifelong record of hospital admissions for abdominal complaints for which no cause had been identified. Intestinal malrotation is a congenital anomaly affecting 1 in 500 births1Torres A.M. Ziegler M.M. Malrotation of the intestine.World J Surg. 1993; 17: 326-331Crossref PubMed Scopus (191) Google Scholar and arises as a deviation from the normal 270° counterclockwise rotation of the gut during embryogenesis. Overall, 80% present in the neonatal period,2Von Flue M. Herzog U. Ackermann C. et al.Acute and chronic presentation of intestinal nonrotation in adults.Dis Colon Rectum. 1994; 37: 192-198Crossref PubMed Scopus (108) Google Scholar although some remain undetected until adulthood when they may develop obstruction owing to volvulus or internal herniation. Others are found incidentally or after imaging for intermittent abdominal pain, which may represent episodes of spontaneously resolving subacute obstruction. In this case, because the bowel appeared grossly normal, symptoms were attributed to the Meckel's diverticulum. These 2 mid-gut abnormalities are not classically associated and indeed it is difficult to determine whether the malrotation was an incidental finding in this case. If so, the inflamed diverticulum may have been a serendipitous occurrence given that surgical correction of malrotation is the only prophylaxis against future catastrophic complications. This case demonstrates that serious underlying pathology cannot be reliably ruled out by routine admission investigations, highlighting the importance of clinical intuition in the absence of objective evidence. Furthermore, of the reported cases of intestinal malrotation presenting acutely in adult life, many are associated with a long history of nonspecific pain, emphasizing the need for thorough investigation of chronic pain beginning in childhood, regardless of distracting behavioral or psychiatric issues.