European Journal of Cardiovascular Medicine

Midgut volvulus is a surgical emergency involving abnormal twisting of the small intestine around the superior mesenteric artery (SMA). It most often occurs as a complication of intestinal malrotation, a congenital condition caused by incomplete rotation and fixation of the gastrointestinal tract during embryogenesis. Normally, between the fourth and twelfth weeks of gestation, the midgut completes a 270-degree counter clockwise rotation around the superior mesenteric vessels before settling into the abdominal cavity with proper fixation. The incidence of midgut volvulus is predominantly confined to the pediatric population, with 64–80% of cases occurring within the first month of life and approximately 90% within the first year.[1] Adult presentation is rare, with an incidence of only 0.2–0.5%, and merely 15% of these cases manifest with symptomatic midgut volvulus.[2] The rarity of this condition in adults often leads to diagnostic delays, as clinicians may not readily consider this diagnosis when evaluating adult patients with gastrointestinal symptoms.

 

The adult clinical presentation varies significantly from that in paediatric cases. Children usually show sudden symptoms like bilious vomiting and intestinal obstruction signs, whereas adults often have long-lasting, episodic symptoms that resemble other gastrointestinal issues, complicating diagnosis. A delayed diagnosis can lead to severe complications such as bowel ischemia, necrosis, perforation, and even death.

 

We present a case of spontaneous midgut volvulus in a 23-year-old woman who experienced recurrent vomiting episodes and was initially treated for acute gastroenteritis. This case highlights the need to consider rare diagnoses in adults with ongoing gastrointestinal symptoms that do not improve with conservative treatment. The report follows the SCARE 2023 guidelines for surgical case reports.[3]

 

CASE PRESENTATION

A 23-year-old woman came to our facility with three days of ongoing vomiting, occasional diarrhoea, low urine output, intermittent abdominal bloating, epigastric pain, headache, dizziness, and severe fatigue. Her symptoms began after eating at a local restaurant. This was also her third similar episode within three weeks.

 

Medical History

The patient had two earlier episodes with the same symptoms, about two weeks apart. During those times, she was hospitalized elsewhere and diagnosed with acute gastroenteritis. She received conservative treatment, including IV fluids, empirical antibiotics, and antiemetics. Significant electrolyte imbalances were found and corrected during these stays. She was discharged after her symptoms improved each time.

 

The patient reported no history of chronic medical conditions, cyclical vomiting syndrome, migraine, prior surgeries, substance abuse, or psychiatric issues like anxiety or panic disorder. Her menstrual cycles were normal. During past hospital stays, a psychiatrist assessed her and prescribed clonazepam and propranolol for suspected anxiety symptoms. She was referred to our facility one week after her second discharge due to a recurrence of symptoms.

 

Physical Examination

On presentation, the patient was mildly dehydrated, with a heart rate of 110 bpm and no fever. Her oxygen saturation on room air was 95%. Cardiac and lung examinations showed no abnormalities. Abdominal examination showed a soft, non-tender, non-distended abdomen with decreased bowel sounds. There were no signs of peritoneal irritation. Neurological assessment and meningeal signs were negative.

 

Laboratory Investigations

Initial laboratory examination showed notable electrolyte imbalances, including hyponatremia (sodium 130 mEq/L), severe hypokalemia (potassium 2.3 mEq/L), metabolic acidosis (bicarbonate 18 mEq/L), and hypochloremia. Blood urea nitrogen and creatinine levels were mildly elevated, indicating prerenal azotemia likely due to dehydration. Tests, including complete blood count, liver function panel, stool analysis and culture, urinalysis, urine toxicology, and serum lead measurements, were all normal. Hormonal assessments, including thyroid function tests, serum cortisol, and prolactin levels, also appeared normal. Aggressive intravenous fluid resuscitation was initiated, and electrolyte abnormalities were corrected. Empirical antibiotic therapy and antiemetic medications were administered. Despite these interventions, the patient's vomiting persisted without improvement.

 

Imaging Investigations

Initial plain radiography and ultrasonography of the abdomen performed at our facility and during previous admissions were reported as normal. Given the persistence of symptoms despite appropriate conservative management and the recurrent nature of her presentation, a contrast-enhanced computed tomography (CECT) scan of the abdomen was obtained. The CECT scan revealed marked distention of the stomach and duodenum with evidence of twisting and coiling of the jejunal and proximal ileal loops around the superior mesenteric artery. A characteristic tapering of oral contrast material within these loops demonstrated the pathognomonic "whirlpool" or "corkscrew" sign (figure 1), indicative of midgut volvulus. The distal ileal and colonic segments appeared collapsed. These radiological features were diagnostic of midgut volvulus, and urgent surgical intervention was planned.

Figure 1: CT-Abdomen with contrast Axial abdominal CT shows a distended stomach and proximal duodenum. The small intestine coil into a classic whirlpool sign around the superior mesenteric vessels, suggesting midgut volvulus with proximal small bowel obstruction.

 

Surgical Intervention

The patient underwent an exploratory laparotomy conducted by experienced surgical specialists familiar with this rare condition. During surgery, findings confirmed a diagnosis of midgut volvulus with a 360-degree clockwise twist of the small bowel around the superior mesenteric artery. The duodenum was abnormally located on the right side of the abdomen. Additionally, Ladd's bands, a fibrous peritoneal adhesion stretching from the cecum to the right upper quadrant, were observed, compressing the duodenum and causing obstruction.

 

A Ladd procedure was carried out with several steps:

• Initially, the bowel was gently detorsed in a counter clockwise direction, with viability confirmed by the return of normal colour and peristalsis.
• Next, Ladd's bands were divided to alleviate duodenal compression.
• Then, the small bowel was moved to the right side of the abdomen, and the colon to the left, establishing a non-rotated anatomical position to reduce the risk of recurrent volvulus.
• Following this, adhesinolysis was performed to widen the mesentery's root, creating a broad-based structure.
• Finally, an appendectomy was done to avoid future diagnostic issues, given the abnormal position of the cecum.

The bowel showed no signs of ischemia or necrosis despite the 360-degree volvulus, which is a positive finding. Haemostasis was achieved, and the abdomen was closed in layers.

 

Figure 2: Intraoperative presentation of malrotation

 

Postoperative Care

The patient's postoperative recovery was unremarkable. She tolerated oral intake smoothly, and her bowel function returned quickly. Electrolyte levels stabilized, and she was hemodynamically stable during her hospital stay. She was discharged on the 5th postoperative day with dietary advice and follow-up instructions. After Twelve months of follow up, she remained asymptomatic and her preoperative symptoms completely resolved.

Midgut volvulus in adults is a rare condition that presents diagnostic difficulties because of its infrequent occurrence and diverse symptoms. Unlike the sudden and severe symptoms often seen in infants, adults may experience chronic, intermittent or recurrent gastrointestinal symptoms (as seen in our patient) that are frequently mistaken for more common gastrointestinal problems like gastroenteritis, functional dyspepsia, or irritable bowel syndrome. Normal development of the intestines involves a series of complex rotation and fixation processes. During weeks 4 to 12 of embryonic development, the midgut temporarily herniates into the umbilical cord, completes a 270-degree counter-clockwise rotation around the superior mesenteric vessels, and then returns to the abdominal cavity. The duodenojejunal junction is fixed to the left of the midline at the ligament of Treitz, and the cecum descends to the right lower quadrant. Any disruption at any stage of this embryological development process can lead to intestinal malrotation. Figure 3: This schematic diagram shows the A) normal anatomy of the broad peritoneal attachment to the small bowel on the left, and the narrow stalk-like peritoneal attachment of the small bowel in the B) malrotation malformation, making it prone to twisting Stringer and colleagues classified intestinal malrotation into three types: • Type I (non-rotation) • Type II (duodenal malrotation with subtypes IIa, IIb, and IIc) • Type III (combined cecal and duodenal malrotation with subtypes IIIa, IIIb, IIIc, and IIId).[4,5] Our case falls under Type IIIa, marked by the duodenum positioned to the right and an abnormally high cecum, both factors increasing the risk of volvulus. In intestinal malrotation, the mesenteric attachment to the posterior abdominal wall is abnormally narrow, forming a fragile pedicle that can allow the midgut to twist. Additionally, fibrous peritoneal bands known as Ladd's bands, which extend from the abnormally positioned cecum to the lateral abdominal wall or liver, may cross and compress the duodenum, leading to obstruction. This anatomical configuration predisposes to midgut volvulus, where the small intestine rotates around the narrow mesenteric base, potentially cutting off blood flow through the superior mesenteric vessels. Recent advancements in molecular genetics have pinpointed several genetic factors linked to intestinal malrotation. Heterozygous inactivation of the FOXF1 gene, which produces a forkhead transcription factor vital for the formation of the dorsal mesentery, has been connected to malrotation [6]. Disrupted mesenteric development hampers proper intestinal fixation, increasing the risk of volvulus. Likewise, mutations in the BCL6 corepressor (BCOR) gene, important for establishing left-right asymmetry and regulating signalling pathways that control normal intestinal rotation, have also been linked to malrotation.[7] Midgut volvulus presents quite differently in adults compared to children. Adults often have chronic, intermittent symptoms such as recurring abdominal pain, nausea, vomiting, early satiety, and occasional bowel obstruction.[1,2] These vague symptoms frequently lead to multiple healthcare visits and different diagnoses before arriving at the correct one. In this case, the patient was initially treated for acute gastroenteritis twice before midgut volvulus was correctly identified. Several factors can trigger acute volvulus in adults with chronic malrotation. Eating large meals suddenly, especially after fasting for a long time, is a known precipitating factor. The weight and size of the food can promote the rotation of the mobile midgut around its narrow mesenteric pedicle. This has been observed during Ramadan, when breaking a daylong fast with large meals has been linked to cases of acute volvulus episodes.[8] Diagnosis of midgut volvulus in adults requires a high level of suspicion, especially when conventional imaging tests show normal results [9,10,11]. Plain abdominal X-rays and standard ultrasounds may appear normal, particularly in cases of intermittent or partial obstruction. Doppler ultrasound can suggest malrotation when the superior mesenteric vein is located to the left of the superior mesenteric artery on transverse images, reversing the usual anatomical relationship [9]. Contrast-enhanced CT has become the preferred diagnostic tool for detecting midgut volvulus [4]. The characteristic "whirlpool sign", which shows the twisted bowel and mesenteric vessels spiraling around the SMA, is highly specific for this condition. This radiological sign, along with clinical symptoms and laboratory findings, allows for a definitive preoperative diagnosis and quick surgical treatment. It’s important to remember that the whirlpool sign can also be seen in ovarian torsion, which should be considered when diagnosing female patients with acute abdominal pain from the adnexa region.[12] The Ladd procedure, first described by William Ladd in 1936, is the standard surgical approach for midgut volvulus. It involves several essential steps: • Detorsion of the volvulus (usually counterclockwise), • Division of Ladd's bands to relieve duodenal obstruction, • Repositioning the small bowel on the right and colon on the left to prevent rotation, • Widening the mesenteric base through adhesiolysis, and • Performing an appendectomy to avoid future diagnostic confusion due to the abnormal caecal position. Timing of surgery is crucial to avoid bowel ischemia or necrosis; resection of damaged tissue is necessary depending on the severity of the ischemia. Fortunately, our patient had a complete 360-degree volvulus but maintained bowel viability, likely because the torsion was intermittent and surgery was performed promptly. This case is notable for several reasons. Firstly, it highlights the diagnostic difficulties in identifying midgut volvulus in adults, as shown by two previous admissions with different diagnoses. Secondly, even with a complete 360-degree twist, there was no sign of bowel ischemia, indicating that spontaneous detorsion may have occurred intermittently before surgery. Thirdly, the repeated symptoms over three weeks suggest that intermittent midgut volvulus should be on the list of possibilities for adults experiencing recurrent unexplained vomiting and electrolyte imbalances, especially when standard imaging results are normal. This report is limited by its single-case nature. Additionally, genetic testing for mutations in FOXF1 or BCOR genes was not performed, which might have provided additional insights into the aetiology of malrotation in this patient.