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INTESTINAL MALROTATION: An Atypical and Delayed Presentation

ARSHAD S KHAN*, GIRISH D BAKHSHI**, KUNDAN K PATIL+,HEMANT G BORSE+, ANSHUL A GOVILA+

*Associate Professor and Unit Head; **Lecturer; +Resident, Department of Surgery, Grant Medical College and JJ Group of Hospitals, Mumbai 400008, India.

Malrotation syndromes are typically presented during the first few months of life but may sometimes appear later in life, causing difficulties and mistakes in diagnosis. We present a case of 15 years old female who presented with recurrent episodes of vomiting. Diagnosis was delayed because respective symptoms were not adequately considered in older children and adults.

INTRODUCTION

Many individuals with intestinal malrotation never develop the syndrome in the older age. Patients who were previously treated for congenital diaphragmatic hernia or congenital abdominal wall defects have intestinal malrotation as an associated anomaly.[1] In most of the patients followed up after Ladd procedure due to intestinal malrotation in infancy there was no recurrence of the symptoms.[2] Ladd procedure stabilizes but does not remove the anomaly. Also, there are people who are not aware that they have the anomaly. Minor or severe abdominal disorders may be present for a long time or the anomaly may be completely asymptomatic.

Malrotation syndromes are clinically evident in the first month of life in 64% of the patients with the syndrome and in 82% in the first year of life.[3]The onset of clinical presentation is rare later in life [4] and the symptoms are obscure and usually of prolonged duration. The diagnosis is often delayed and various others, including psychogenic disorders, are made before a final, correct one.[5] Bill divided intestinal malrotation according to the time of occurrence in three embryonic stages.[6] Malrotation syndromes in children are most commonly presented as Ia or IIIa type. These are also the most serious types. Clinically, they are manifested as duodenal obstruction caused by volvulus of the midgut.[7] They become apparent when the gut is filled with air in the first days of life. If a complete volvulus does not develop, the patients present with the symptoms of subtotal obstruction of the duodenum and the ileus develops later. Other types are very rare and are often found accidentally at operations performed for other reasons. After the second year of life, the manifestations of malrotation are very rare. These patients develop stable subtotal obstruction of the duodenum and/or intermittent volvulus.[8] The prevalence of nonrotation without volvulus and symptoms of obstruction is not known.[6]

CASE REPORT

A 15 year-old female presented with recurrent vomiting episodes since 3 months. She was intensely vomiting for three days prior to the admission and developed symptoms of dehydration. On admission, the abdominal wall was soft with minimal distension in epigastrium. Peristalsis was weak. Abdominal X-ray was unremarkable.Upper GI endoscopy revealed dilated stomach and duodenum with patulous pylorus. Contrast examination revealed giant, dilated stomach with obstruction of the third part of the duodenum (Fig.1). Ultrasonography showed superior mesenteric vein to the left of superior mesenteric artery, which is pathognomonic of malrotation. Contrast C.T.scan revealed (Fig.2) a classical "whirl-pattern" seen in malrotation with midgut volvulus. [9] On surgery, nonrotation of type IIIa with volvulus of the midgut was found. There were no ischaemic changes of the gut and Ladd procedure was per formed.[10] Postoperatively, the clinical status improved and normalized with weight gain of 2 kg in one month without any complications.

Fig. 1 Fig.2
Fig. 1 Barium study showing dilated stomach and duodenum
with obstruction of the third part of duodenum.
Fig. 2 CT scan showing "Whirl sign" of midgut volvulus.

DISCUSSION

Midgut volvulus is a complication of bowel malrotation. During embryologic development, intestinal rotation primarily occurs in the midgut, the segment of bowel supplied by the superior mesenteric artery. During the late stage of gut rotation, anchoring of the mesentery and bowel to the posterior abdominal wall occurs. The small-bowel mesenteric root forms a diagonal line from the Treitz’s ligament to the caecum. In malrotation, the mesenteric root is shortened, which allows volvulus to occur. Malrotation is also associated with peritoneal or Ladd’s bands, which can cause duodenal obstruction.

The reason for a delayed presentation of malrotation syndromes is not known. There is a balance between the narrowing of the duodenum and the torsion of the midgut. The degree of the duodenal obstruction may influence clinical symptoms. It may be obstructed enough to cause some disorders, but not enough to cause ileus. The syndrome appears at the point of critical narrowing.[6] This implies that many people live with asymptomatic intestinal malrotation or have only small disorders. Our patient remained asymptomatic till 15 years and then started developing symptoms. Intermittent vomiting in the older child is sometimes mis-diagnosed. Many children carry the diagnosis of psychogenic vomiting for months or years before the correct diagnosis of malrotation is made. [11] Delayed diagnosis of intestinal malrotation may be responsible for severe dehydration.[12] as in our case. Modern ultrasound examination is very helpful in the diagnosis. The position of the superior mesenteric vein left to the artery suggests malrotation in almost all cases. The position of the vein anterior to the artery is found in 30% cases of intestinal malrotation. [13] This method is applicable as a screening test when considering a differential diagnosis. Ultrasonography was diagnostic in our case also and CT scan was done for academic interest.

The most common type of malrotation is Ia or IIIa. [8] In our case there was IIIa type of malrotation for which Ladd procedure was done. Postoperatively patient became asymptomatic with weight gain of 2 Kg in 1 month.

In summary, malrotation leading to midgut volvulus is rare in adults, however it should be considered as a differential possibility in the diagnosis of abdominal disorders in older children and adults. Ultrasonography can be used for screening in diagnostic dilemma. The CT findings in midgut volvulus can be pathognomonic if the classic whirl pattern around a central superior mesenteric artery is seen. CT allows the rapid diagnosis of this unusual condition in the adult patient presenting with abdominal pain or bowel obstruction of uncertain cause. Surgery in the form of Ladd procedure is the treatment of choice.

ACKNOWLEDGEMENT

We would like to thank Dean and Dr. MG Rathod, Head of the Department of Surgery, Grant Medical College and JJ Group of Hospitals for granting us permission to publish this case report.

REFERENCES

1. Filston HC, Kirks DR. Malrotation - The ubiquitous anomaly. J Pediatr Surg 1981; 16 : 614-20.

2. Ford EG, Senac MO Jr, Srikanth MS, Weitzman JJ. Malrotation of the intestine in children. Ann Surg 1992; 215 : 172-8.

3.Stewart DR, Colodny AL, Dagget WC. Malrotation of the bowel in infants and children: A 15 year review. Surgery 1976; 79 : 716-20.

4. Ruiz-Tartas A, Arizaga-Rovalino P, Fernandez-Lobato R, Marin-Lucas FJ, Jimenez-Miramon FJ, Moreno- Azcoita M. Intestinal malrotation in an adult. Rev Esp Enferm Dig 1994; 86 : 701-2.

5.Van Roye S, Vandelanotte M, Proot L, Lanckneus M. Chronic small bowel obstruction due to intestinal malrotation in the older child: an often missed diagnosis. Acta Chir Belg 1993; 93 : 262-4.

6.Bill AH. Malrotation of the intestine. In: Ravitch MM, Welch KJ, Benson CD, editors. Pediatric surgery. Chicago: Year Book Medical. 1979; 912-23.

7.Nair R, Hadley GP. Intestinal malrotation - experience with 56 patients. S Afr J Surg 1996; 34 : 73-5.

8.Lewis JE. Partial duodenal obstruction with incomplete duodenal rotation. J Pediatr Surg 1966; 1 : 47-53.

9.Shaff M, Himme L, Farb E, Sacks GA. " The whirl-sign" A CT finding in volvulus of the small bowel. J Comput. Asst Tomogram 1985; 9 : 40.

10.Torres AM, Ziegler MM. Malrotation of the intestine. World J Surg 1993; 17 : 326-31.

11.Stewart DR, Colodny AL, Dagett WC. Malrotation of the bowel in infants and children : A 15 year review. Surgery 1976; 79 : 716-20.

12.Koshida R, Sakazume S, Maruyama H, Okuda N, Ohama K, Asano S. A case of pseudo-Bartter’s syndrome due to intestinal malrotation. Acta Paediatr Jpn 1994; 36 : 107-11.

13. Weinberger E, Winters WD, Liddel RM, Rosenbaum DM, Krauter D. Sonographic diagnosis of intestinal malrotation in infants: importance of the relative positions of the superior mesenteric vein and artery. AJR 1992; 159 : 825-8.


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