Megacolon: an Unusual Presentation of Malrotation

Pediatr Surg Int (1994) 9:592-594 © Springer-Verlag 1994

Megacolon: an unusual presentation of malrotation

M. Ayas, D. H. Teitelbaum, N. Uitvlugt, A. G. Coran, and A. D. Olson

Departments of Pediatrics and Surgery, Section of Pediatric Surgery, University of Michigan Medical School and C. S. Mott Children's Hospital, Ann Arbor, Michigan, USA

Accepted 4 January 1994

Abstract. This report describes a 9-year-old boy with a highly unusual variant of malrotation: massive colonic distention secondary to peritoneal bands and a localized volvulus. The presentation and treatment are presented.

Key words: Malrotation - Megacolon - Colonic volvulus

Introduction

Malrotation, or non-rotation of the gastrointestinal (GI) tract, can be manifested by bilious emesis, abdominal pain, and volvulus. Errors in the complex embryologic rotation of the large and small bowel can produce a Fig. 1. Abdominal radiograph showing dilated colonic loops multitude of rotational anomalies [1]. Fig. 2. Barium enema illustrates partial ob- This report describes a patient who struction of transverse colon with markedly presented with massive colonic dis- vomiting for 8 days. Emesis was accompanied dilated proximal colon tapering into normal- tention due to a variant of rotation not by liquid diarrhea for the last 3 days. Five days sized descending colon previously described in the surgical prior to admission he began to complain of generalized abdominal pain, which progres- literature. The purpose is to alert pe- sively increased. Two days before he pre- initial laboratory findings were as follows: Na diatricians and surgeons of a unique sented, he started to vomit bilious material and 137 mEg/l; K 4.6 mEgl/l; C1 103 mEg/l; CO2 radiologic and clinical presentation of became increasingly listless, with poor oral 22 mEg/l; urea nitrogen 8 mg/dl; creatinine malrotation. intake. There was no previous history of fever, 0.6 mg/dl; Ca 9.1 mg/dl; phosphorus 4.4 ms/ blood in the stool, or vomiting. His medical dl; aspartate aminotransferase 14 U/l; alanine history consisted of constipation and irritable aminotransferase 20 U/l; lactate dehydrogen- bowel syndrome diagnosed 2 years prior to ase 16 U/l; alkaline phosphatase 112 U/l; Case report admission, which was treated with metamucil White blood cell count 10.8 ram3; hemo- and a high-fiber diet. His parents reported that globin 11.2 g/dl; hematocrit 33.5%; amylase A 9-year-old male presented to the C. S. Mott he had had intermittent distention of his ab- 34 U/l; lipase 19 U/l; and sedimentation rate Children's Hospital with the complaint of domen throughout the last 3 years. 5 mm/h. On physical examination the abdomen was Abdominal radiographs showed massively markedly distended, tympanitic to percussion, dilated bowel loops in the right and left upper Correspondence to: D. H. Teitelbaum, Section and tender throughout. No hepatosplenome- quadrants consistent with the right and trans- of Pediatric Surgery, Mort Children's Hospital, gaiy was found, and the bowel sounds were verse colon (Fig. 1). A diagnosis of colonic F3970 Box 0245, Ann Arbor, Michigan 48109, diminished. Rectal examination showed nor- volvulus was considered and colonoscopy was USA mal stool, which was hematest negative. The performed, which showed that the colon was 593

been reported as the presenting symptoms in patients with malrotation [3]. These patients have also been mis- diagnosed as having milk intolerance,

> ___ food allergies, and psychogenic vom- / ,'--V ---~ ( Lt ~ iting [4, 5]. The chronic, nonspecific ~\ i,.,i "1 ~ )1~_~~--~ nature of the symptoms in patients with late presentation often leads to a delay ~ ~ ~ -~------~-~2---f~_ __ /- ~./l~~ in diagnosis. Spigland, et al. reported ~---~, ~ /l I-~" ~. ~ ~- ~,~.~~' - /21~:-~ " ~ the mean delay in diagnosis to be 1.7 ~--~l~ t, @-~'~ \ years in patients presenting at an older age, compared to only 5 days in infants who present early [4]. Our patient is typical of this older group. His symptoms of chronic, poorly characterized Fig. 3. Upper gastrointestinal series shows abdominal pain and abdominal disten- abnormal rotation of duodenum, with duode- tion made the diagnosis quite difficult. nojejunal junction inferior and to right of Our patient's presentation was also normal location quite unique in that megacolon as an Fig. 4. Diagram of operative findings. Note isolated finding of malrotation has not non-rotation of small bowel (duodenum (D) to been previously described. Malrotation right of midline) and horizontal peritoneal has been reported in association with partially obstructed at the splenic flexure and bands fixing splenic flexure, causing colon to in the mid-transverse region; marked dilata- become kinked and obstructed megacolon only secondary to Hirsch- tion was noted proximal to the sites of ob- spmng's disease [6]. Lassman reported struction. A decompressing tube was placed the association of megacolon and by colonoscopy in the mid-transverse colon; bands, causing a localized volvulus of this malrotation, with delayed migration repeat abdominal radiographs showed resolu- portion of colon (Fig. 4). The transverse and degeneration of intramural gang- tion of the dilated segment. The tube was colon was markedly dilated and was decom- lionic cells; this ganglionic abnorma- removed 2 days later and the colonic disten- pressed by untwisting the large bowel. The tion recurred. Colonic biopsies showed mild, lity was not found in our patient [7]. It peritoneal bands between the right colon and was obvious during surgery that the nonspecific inflammation with ganglion cells the right retroperitoneal region were divided, present throughout the rectum and colon. A correcting the duodenal compression and nar- dilatation of the colon was due to a barium enema showed a transition at the mid- rowing of the base of the transverse colonic partial volvulus caused by peritoneal descending colon from a dilated transverse mesentery. The duodenum and proximal small bands. Unlike a typical midgut volvu- colon to a normal caliber distally. The colon bowel were positioned to the right of the spine lus associated with malrotation, this at the hepatic flexure was enlarged to 16 cm in and the entire colon positioned to the left. diameter (Fig. 2). The cecum appeared normal one was isolated to the colon. Colonic Finally, and inversion appendectomy was volvulus is an unusual condition in and was located in the fight lower quadrant. performed. Follow-up of the child 7 months An upper gastrointestinal series (UGI) showed after the operation showed him to be comple- children that usually involves the sig- a normal esophagus and stomach with no tely asymptomatic and thriving normally. Ab- moid or cecum [8] but has not been evidence of gastric outlet obstruction. The dominal radiographs showed normal colon previously reported with malrotation. duodenal bulb was normal; the first and caliber. Colonic distention can also be seen in second portions of the duodenum were in a Twelve months after the child's initial normal location, but the third and fourth patients with the rare anomaly of re- operation he presented with abdominal disten- verse rotation of the intestine, whereby portions were anterior and did not cross the tion. Abdominal x-rays suggested a recurrent midline. The junction of duodenum and jeju- volvulus. He was taken to surgery where a the transverse colon is located behind num was in the midline at the level of the 1st similar volvulus of the transverse colon was the superior mesenteric artery [9]. This lumbar vertebra and was medial, inferior, and discovered. He underwent a resection of the entity is, however, clearly distinct from anterior to the expected location of the liga- transverse and right colon with primary ana- ment of Treitz. There was no evidence of the one described here. stomosis. He is currently asymptomatic 5 Figure 4 demonstrates the unique obstruction, abnormal dilatation, or mucosal months following this last surgery. irregularity of the small intestine (Fig. 3). anatomy of this case. Ladd's bands Based on the radiologic studies, it was felt were present in the classic locations, that a malrotation might be the cause of the but also extended from the ascending colonic obstruction. The patient was taken to Discussion and transverse colon to the left lateral the operating room and the abdomen was entered through a transverse fight upper quad- abdominal wall. It was these lateral rant incision. Following evisceration of the Although malrotation, or non-rotation, bands that apparently held the splenic bowel, tracing the course of the intestine it most commonly presents with prox- flexure in place and caused it to be- was observed that the distal duodenum and imal obstructive symptoms within the come obstructed. In an extensive, re- proximal jejunum were compressed by perito- first 2 years of life, older children and cent review of the various anatomic neal bands (Ladd's bands) between the right adults can present with confusing and patterns of malrotation, no description colon and the fight upper quadrant. There was often less specific symptoms [2]. no ligament of Treitz and the duodenum was corresponding to our patient's findings anterior to the superior mesenteric vessels. Chronic abdominal pain, intermittent was reported [1]. This case history il- The mesentery of the transverse colon was vomiting, chronic diarrhea, malab- lustrates how difficult the diagnosis of restricted to a narrow base by these peritoneal sorption, and failure to thrive have all malrotation can be, and indicates the 594 importance of both a UGI and barium References cells and nerves. Acta Neuropathol 74: enema in making the diagnosis. The 281-286 barium enema in our patient showed 1. Bill AH Jr and Grauman D (1966) Ra- 6. Powell DM, Othersen HB, and Smith CD (1989) Malrotation of the intestines in the cecum in the proper location with tionale and technic for stabilization of mesentery in cases of nonrotation of the children: the effect of age on presentation no evidence of malrotation. Approxi- midgut. J Pediatr Surg 1: 127-136 and therapy. J Pediatr Surg 24:777-780 mately 5% of patients with malrotation 2. Brandt ML, Pokorny WJ, McGill CW, and 7. Schey WL, Donalson JS, and Sty JR will have a normally rotated colon [4]. Harberg FJ (1985) Late presentation of (1993) Malrotation of bowel: variable The UGI clearly showed the duodenal midgut malrotation in children. Am J Surg patterns with different surgical con- siderations. J Pediatr Surg 28: 96-101 malposition diagnostic of intestinal 150:767-771 8. Spigland N, Brandt ML, and Yazbeck S malrotation. 3. Filston HC, and Kirks DR (1982) The association of malrotation and Hirsch- (1990) Malrotation presenting beyond the In summary, we describe a case of sprung's disease. Pediatr Radiol 12: 6-10 neonatal period. J Pediatr Surg 25: 1139- non-rotation of the small bowel pre- 4. Hiltunen KM, Syrja H, and Matitainen M 1142 senting with colonic obstruction. This (1992) Colonic volvulus. Eur J Surg 158: 9. Wang CA and Welch CE (1963) Anoma- report emphasizes that a high index of 607-611 lies of intestinal rotation in adolescents and children. Surgery 54:839-855 suspicion of malrotation must be 5. Lassmann G (1987) Megacolon in third- maintained in any child with sig- phase malrotation with delay of migration and degeneration of intramural ganglionic nificant GI symptoms.