An adult with midgut volvulus causing diagnostic challenge: Case report

Abstract

Introduction and importance

Midgut volvulus is an extremely rare cause of abdominal pain in adults, often due to intestinal malrotation. Its nonspecific and insidious presentation can lead to significant diagnostic delays, increasing the risk of intestinal ischemia, necrosis, or gangrene. Early recognition and prompt surgical intervention are crucial to improving outcomes.

Case presentation

We report a case of a 23-year-old Ethiopian male with a history of recurrent abdominal discomfort and intermittent vomiting since childhood. Despite multiple clinical evaluations and normal laboratory investigations, the diagnosis remained elusive until an abdominal computed tomography (CT) scan revealed midgut volvulus. The patient underwent an emergency Ladd's procedure and appendectomy. His postoperative recovery was uneventful, and symptoms resolved completely on follow-up.

Clinical discussion

Midgut volvulus is primarily a pediatric diagnosis, with adult cases being extremely rare. When it does occur in adults, it typically presents with vague gastrointestinal symptoms, contributing to diagnostic delays. CT imaging remains the gold standard for diagnosis. The mainstay of treatment is surgical correction via Ladd's procedure. Awareness of this rare but serious condition is essential to reduce morbidity and mortality.

Conclusion

Clinicians should consider midgut volvulus in adults presenting with chronic or unexplained gastrointestinal symptoms, particularly in the absence of prior abdominal surgery. Timely diagnosis and surgical management are key to preventing life-threatening complications.

Highlights

• •Adult midgut volvulus is rare and often leads to diagnostic delays due to its nonspecific presentation.
• •It may present with chronic symptoms or as an acute emergency.
• •Early recognition and treatment are essential to prevent ischemia, necrosis, and high morbidity or mortality.
• •A CT scan is the gold standard for diagnosis.
• •Ladd’s procedure is the definitive surgical treatment.

1. Introduction

Midgut volvulus can occur at any age, though its occurrence in adults is rare [1]. It may present insidiously or acutely. The acute presentation is a true surgical emergency that can lead to necrosis of the twisted bowel segment [2]. This condition arises from an arrest in rotation or fixation of the gastrointestinal tract, resulting in a shortened mesenteric “anchor” that allows the small bowel to twist around the superior mesenteric artery (SMA) as its axis [3].

Patients may experience long-standing, vague, non-specific gastrointestinal symptoms, often leading to extensive investigations before a diagnosis is made [4]. A delayed diagnosis can have catastrophic outcomes, including bowel ischemia, necrosis, or gangrene. Imaging studies such as ultrasound and CT scans help identify the “whirlpool” appearance, which is characteristic of midgut volvulus. CT scan is the gold standard for diagnosis [5,6]. As midgut volvulus is a significant cause of small bowel obstruction, prompt clinical suspicion and early operative intervention are crucial to prevent ischemia and gangrene [7]. Herein, we present and discuss a case of midgut volvulus in a 23-year-old male patient with diagnostic challenge. This work has been reported in line with the SCARE criteria [8].

2. Case report

A 23-year-old male presented to our outpatient department with a history of recurrent abdominal discomfort, bloating and intermittent vomiting since childhood. He denied any history of fever, hematemesis, melena, jaundice, or altered bowel habits. He had several visits to medical center for these complaints and was repeatedly treated symptomatically without definitive diagnosis. His past medical and surgical history was unremarkable. On physical examination, the patient was hemodynamically stable, and no abnormal findings were noted.

The patient underwent the following investigations, all of which returned normal results: complete blood count, renal function test, liver function test, erythrocyte sedimentation rate, serum amylase, random blood sugar, hepatitis B and C viral markers, stool microscopy, and urine analysis. However, a contrast-enhanced abdominal CT scan performed at our facility revealed a classic “whirlpool sign” indicative of midgut volvulus (Fig. 1).

Fig. 1.

Arterial Phase Abdominal CT scan showing small bowel rotating around the superior mesenteric artery (blue arrow)/the mesentery; constituting the whirl pool sign (red circle). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)

He was subsequently hospitalized and taken for exploratory laparotomy. Intraoperatively, congenital malrotation was confirmed. The small bowel mesentery was narrow and elongated, resulting in the clockwise rotation of the small bowel, which was entirely located on the left side of the abdomen. An adhesion band extending from the duodenum and jejunum to the posterior abdominal wall was identified. There were no ischemic changes in the intestine. A Ladd's procedure and incidental appendectomy were performed successfully. The patient had an uneventful postoperative course and was discharged on the second postoperative day. At his 2-week and 3-month follow-up visits, he remained asymptomatic, with complete resolution of his prior complaints.

3. Discussion

Midgut volvulus occurs when the small bowel rotates around its mesenteric vascular pedicle, commonly seen in pediatric populations. Around 85 % of cases present within the first two weeks of life, and more than 90 % before the first birthday [9,10]. It is rare in adults, with reported incidences ranging from 0.00001 % to 0.19 %. While pediatric cases are often due to congenital malformations, adult midgut volvulus is usually secondary to postoperative adhesions [7]. It has also been reported during pregnancy, accounting for 25 % of intestinal volvulus cases [11].

Embryologically, intestinal rotation occurs in three stages. During weeks 10–12 of gestation, the midgut undergoes a 270° rotation within the abdominal cavity. This process brings the duodenal “C” loop behind the superior mesenteric artery, positioning the ascending colon on the right, the transverse colon above, and the descending colon on the left. Any anomalies in this stage can result in nonrotation, malrotation, or reversed rotation [12].

Patients with midgut volvulus typically present with an insidious onset of nonspecific gastrointestinal symptoms over a prolonged period. These symptoms are often vague and remitting, persisting for months or even years [1]. Common complaints include intermittent crampy abdominal pain, bloating, weight loss, and nausea or vomiting. Intermittent bowel obstruction due to Ladd band compression or intermittent midgut volvulus is considered the primary pathophysiologic mechanism [13]. However, in about 10–15 % of adults, there may be an abrupt onset of severe abdominal pain, nausea, vomiting, hematemesis, or hematochezia, sometimes accompanied by hemodynamic instability [2].

Recurrent volvulus occurs in 1.8–8 % of cases, necessitating a heightened awareness among clinicians. The probable mechanism is a midgut volvulus with partial duodenal obstruction being relieved spontaneously [3]. As evident in our case, Physical examination might show nothing of note in between the attacks [2].

Midgut volvulus can be promptly diagnosed through imaging. Conventional radiographs may often appear normal but can occasionally reveal a distended stomach and proximal duodenum with reduced distal bowel gas [14]. An upper gastrointestinal series may show the “bird's beak” appearance at the point of volvulus [15]. Ultrasound can identify dilated and fluid-filled small bowel loops and reversed orientation of the superior mesenteric vein (SMV) relative to the superior mesenteric artery (SMA). However, this finding is neither sensitive nor specific. In contrast, spiral appearance of mesenteric vessels termed “whirlpool” is virtually diagnostic of midgut volvulus [16]. It can be seen on color-flow Doppler ultrasound, as the transducer is moved craniocaudally [14,17].

The gold standard for diagnosing midgut volvulus is a CT tomography (CT) scan, which offers superior diagnostic accuracy [7]. It can reveal small bowel dilation, wall thickening, intramural gas, portal venous gas, ischemia, and necrosis, with an accuracy exceeding 83 % [[18], [19], [20]]. Additionally, catheter angiography can detect specific findings such as tapering or abrupt termination of the mesenteric vessels and the twisting of mesenteric vessels, also known as the “barber pole sign “ [21]. Multidetector CT angiography is superior, as it provides detailed information about the degree of volvulus, the location of obstruction, intestinal ischemia, and associated anomalies in adjacent organs [22].

Midgut volvulus has an associated mortality rate of approximately 5 % [13]. Delayed diagnosis worsens outcomes due to the increased likelihood of intestinal ischemia and necrosis. The hallmark symptom of “pain out of proportion” to clinical findings often indicates impending intestinal vascular compromise, necessitating urgent surgical intervention [18].

The classic treatment for incomplete intestinal malrotation is Ladd's procedure, as performed in this case. The procedure consists of four key steps: counterclockwise detorsion of the volvulus, division of Ladd's bands, broadening of the small bowel mesentery, and division of adhesions around the SMA. Additionally, the small bowel is repositioned on the right side of the abdomen, the large bowel on the left, and an appendectomy is performed [23].

The Ladd procedure can be performed using either open laparotomy or a laparoscopic approach. Laparoscopic surgery has been shown to be a feasible and safe alternative to open surgery for treating intestinal malrotation with midgut volvulus in neonates. In adults, several studies have documented successful laparoscopic Ladd procedures for malrotation without volvulus. However, in cases of chronic midgut volvulus, laparoscopy poses significant challenges due to extensive intra-abdominal adhesions [24]. Hence, an open laparotomy was deemed more appropriate in our case.

The severity of the initial presentation is a major determinant of the surgical outcome. Mortality rates for midgut volvulus vary between 0 % and 25 %, with acute cases having the highest mortality rates. Among pregnant women, maternal and fetal mortality rates can reach 13 % and 35 %, respectively. Significant postoperative morbidity (up to 60 %) can also occur [1].

4. Conclusion

Midgut volvulus should be considered in the differential diagnosis of patients with uncertain or longstanding abdominal pain, particularly those without a history of abdominal surgery. Early diagnosis is crucial, as delayed recognition carries a grave prognosis.

Abbreviation

CT

computed tomography

SMA

superior mesenteric artery

SMV

superior mesenteric vein

CRediT authorship contribution statement

Megersa Regassa – Conceptualization, Data collection, literature review, manuscript drafting.

Abate Bane Shewaye– Conceptualization, Data collection, literature review, manuscript drafting.

Zebeaman Tibebu– Conceptualization, Data collection, literature review, manuscript drafting.

Kaleb Assefa Berhane – Conceptualization, Data collection, literature review, manuscript drafting.

Informed consent statement

Written informed consent was obtained from the patient for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

Ethical clearance was obtained from the institutional review board of the affiliated institution with Ref.No AMC/C/R- 035/2024.

Guarantor

Abate Bane Shewaye.

Funding support

No funding was obtained.

Declaration of competing interest

The authors report no conflicts of interest in this work.