A case report of hepatic and renal dysfunction complicating midgut malrotation in an adult

Abstract

A 39-year-old man had an unusual presentation of jaundice and acute renal dysfunction complicating midgut malrotation. Diagnosis by computed tomography scan enabled prompt surgery and functional correction of the malrotation, with a full return to normal life.

BACKGROUND

To the best of our knowledge there have been no previous case reports documenting malrotation of the midgut presenting in middle life and causing hepatic and renal dysfunction due to associated mechanical disturbances.

CASE PRESENTATION

A 39-year-old man was referred to the accident and emergency department by his general practitioner with a 5 day history of vomiting and upper abdominal pain. He had been unable to keep down any food or fluid and had not opened his bowels for 5 days.

He described the pain as more of a discomfort, which radiated to his back. He had also noticed that his skin had become yellow and that his urine was dark. There were no risk factors for viral hepatitis and there was no significant drug, past medical or family history. He was a non-smoker and consumed around 20 units of alcohol per week, with no history of alcohol excess. He worked on a building site and had noticed some dead rats on the site.

On admission he was apyrexial, haemodynamically stable, alert, orientated and clinically dry and jaundiced. Abdominal examination showed minimal epigastric tenderness and no other signs. The patient was admitted initially under the care of the gastroenterology team.

INVESTIGATIONS

• Na=130 mmol/l, HCO₃=34 mmol/l, urea=21.8 mmol/l, creatinine=284 μmol/l, Hb=19.4 g/dl, white blood cell count=11.8 (neutrophils 9.3) × 10⁹/l, bilirubin=144 μmol/l, alanine aminotransferase (ALT)=241 IU/l and gamma glutamyl transferase (GGT)=78 IU/l.

• Mean corpuscular volume, coagulation tests, alkaline phosphatase and amylase were normal.

• Urine and blood cultures were negative.

• ECG and chest x ray were normal.

• Abdominal ultrasound showed no hepatobiliary abnormality; however, the midline was obscured by bowel gas.

• Leptospira serology, hepatitis serology (A, B, C), toxicology, alpha-fetoprotein and autoimmune profile were all negative.

• Abdominal x ray, gastroscopy and abdominal computed tomography (CT) (see Treatment)

DIFFERENTIAL DIAGNOSIS

• Leptospirosis was a possibility in view of his occupation.

• Gastrointestinal tract obstruction with sepsis and systemic complications.

• Viral hepatitis.

• The possibility of drug overdose was considered, though there was no clear indication in the history to suggest that.

TREATMENT

Despite continuous supportive treatment with intravenous fluids and intravenous clarithromycin (for leptospirosis), the patient’s renal and hepatic function continued to deteriorate. He remained unable to eat or drink, with persistent vomiting. Abdominal examination on the second day of his admission revealed a succussion splash, and an abdominal x ray showed a slightly dilated stomach with no other specific findings. Therefore, a gastroscopy was arranged to rule out gastric outlet obstruction. This showed a stomach completely full of bile actively refluxing through a gaping pylorus. The possibility of small bowel obstruction was raised, and a surgical opinion was obtained. Abdominal CT scan was arranged to be done immediately; due to his renal dysfunction, intravenous contrast was not used. The CT scan showed distension of the stomach and proximal part of the duodenum. The third part of the duodenum was abnormally placed and did not cross the midline. There was transposition of the superior mesenteric artery and vein. The small bowel appeared to spiral around the mesenteric vessels (whirlpool sign). The findings were those of midgut malrotation and volvulus (fig 1). He was therefore prepared for theatre on the second day his admission and underwent a laparotomy.

Figure 1.

Computed tomography scan of the abdomen showing the “whirlpool sign” of the mesenteric vessels.

The principle findings were: the caecum was in the right upper quadrant, and attached to the kidney; below this was a much dilated duodenum; the stomach was grossly dilated; the small bowel was collapsed and somewhat twisted.

Procedure

The adhesions of the caecum to the right upper quadrant (Ladd’s bands) were divided. The caecum was then fully mobilised and placed in the left iliac fossa. The grossly distended duodenum was freed down to the right iliac fossa. After this manoeuvre, the duodenum then took a straight course down the right paracolic gutter. The small bowel was laid in a good order so that the duodenum would empty freely into the small bowel. An incidental appendectomy was performed to both prevent future diagnostic uncertainty and to aid in adhesion formation to anchor the caecum. The caecum was then sutured in place in the left iliac fossa. The bowel was replaced in its new position with no obvious kinks.

OUTCOME AND FOLLOW-UP

The patient had an uncomplicated postoperative recovery. He began to eat on day 3 and opened his bowels on day 4. Furthermore his renal and liver function tests quickly returned to normal postoperatively and his serology was negative. He was discharged home 4 days after his operation. Follow-up 6 weeks and 6 months after discharge confirmed an excellent postoperative outcome, showing full recovery of his renal, liver and gastrointestinal tract function.

DISCUSSION

Midgut malrotation results from non-rotation or incomplete rotation of the primitive intestinal loop around the axis of the superior mesenteric artery (SMA) during fetal life.¹ In 1932, Ladd published an article on congenital duodenal obstruction and presented a surgical correction of malrotation.² The basics of this method are still used in surgical treatment today. The overall incidence of malrotation is unknown because some patients may present years later or remain asymptomatic for life. Sixty per cent of such patients are diagnosed during the first month of life, 20% between 1 month and 1 year of age, whereas 20% of cases present after the first year of life, but typically during childhood.³ As the symptoms are not specific, with a low index of suspicion in adults, the clinical diagnosis is usually made through imaging or at laparotomy.⁴ Unlike the paediatric population, most adult patients with gut malrotation lack other congenital anomalies and are free of symptoms related to their malrotated bowel. Matzke et al mentioned that the incidence of midgut malrotation in adults is 0.2%, which is usually asymptomatic and discovered incidentally.⁵ Fewer than 50 cases describing symptomatic malrotation in adults have been reported in the literature over the past 80 years.³ Symptoms are often mild and longstanding, before suddenly worsening. The causes of acute deterioration in the symptoms are unknown.⁶ For a patient with midgut volvulus, a characteristic “whirlpool sign” appearance is seen on CT scan, indicating mesenteric rotation around a narrow based pedicle.⁵

Abnormal liver function test results in our patient could be explained on the basis of acute mesenteric ischaemia caused by torsion of the malrotated midgut around the SMA (due to short mesenteric attachment of the small intestine in such anomalies). This would cause mechanical obstruction of the SMA and/or its venous outflow, resulting in reactive vasospasm in the SMA region with tissue hypoxia and bowel wall spasm, followed by mucosal barrier disruption. As the ischaemia persists, bacteria, toxins, and vasoactive substances would be released into the systemic circulation. This might cause death from septic shock, cardiac failure, or multisystem organ failure or dysfunction before bowel necrosis actually occurs. On the other hand, acute renal failure could be explained by severe dehydration due to repeated vomiting, in addition to the probability of direct toxic effects of circulating toxins on the kidneys’ function.

To the best of our knowledge, there are no similar case reports published in literature describing adult midgut malrotation complicated by hepatic and renal dysfunction. However, other modes of presentation have been reported (table 1).

Table 1.

Modes of presentation of adult midgut malrotation

Author and year	Age (years), gender	Mode of presentation and duration
Hsu7 2008	84, female	Vague upper abdominal pain, nausea and vomiting (N&V) and weight loss (1 year)
Bray3 2007	25, male	Persistent diffuse abdominal pain
Fu8 2007 (12 cases)	35, female	Abdominal pain for 2 months
	29, male	Abdominal pain, N&V, upper gastrointestinal (GI) bleeding for 10 days
	33, male	Upper abdominal pain and weight loss for years
	24, male	Lower abdominal pain for 3 days
	26, female	Upper abdominal pain and weight loss for 1 year
	22, female	Abdominal pain and vomiting for 1 week
	58, male	Abdominal pain for 1 day
	23, male	Abdominal pain and distension for 3 years
	23, male	Abdominal pain with N&V for 6 months
	22, male	Abdominal pain, vomiting and diarrhoea for 5 days
	22, male	Chronic abdominal pain for 12 years
	25, female	Abdominal pain with N&V for 10 h
Pelta9 2006	50, female	Right upper abdominal Pain (few hours)
Jayathillake10 2005	20, male	Acute abdominal pain and vomiting for 3 h
Kapfer11 2004 (3 cases)	22, male	Acute appendicitis
	80, male	Pain/mass in the left upper quadrant of the abdomen
	24, female	Upper abdominal pain and vomiting (6 weeks)
Gamblin12 2003	36, male	Colicky abdominal pain of less the 24 h
Puvaneswary13 2003	18, female	Abdominal pain, nausea, constipation (3 weeks)
Jean14 2003	72, male	Abdominal pain for 2 weeks
Zissin1 1999 (18 cases)	15–79,12 females	Incidental finding on CT scan indicated for non-GI tract symptoms (except in one case)
Bodard15 1994	24, male	Crampy abdominal pains and vomiting over with recurrent alcoholic pancreatitis (1 year)
Fukuya16 1993 (7 cases)	21, male	Intermittent crampy abdominal pain with N&V since the age of 1.5 years
	28, male	Intermittent abdominal pain with N&V since childhood with peptic ulcer disease
	34, male	Intermittent crampy abdominal pain with N&V for 2–3 months
	57, male	Alternating bowel habits and lower abdominal pain for years
	33, male	Acute abdominal pain with N&V for 3 days
	34, male	Acute abdominal pain with N&V for 2 days
	22, male	Acute abdominal pain with N&V for 1 day
Konings17 1990	69, male	Abdominal pain and vomiting for 5 days
Rowsome18 1987 (2 cases)	46, male	Abdominal pain and bloating after meals (2 months)
	47, male	Abdominal pain, N&V, diarrhoea (1 week)

LEARNING POINTS

• Midgut malrotation in adults is an uncommon but potentially serious cause of small bowel obstruction (SBO). The diagnosis should be considered in any patient with SBO without a surgical history of abdominal operations (that is, high index of suspicion with focused history and clinical examination, in addition to the appropriate investigations).

• Midgut malrotation in adults usually presents with non-specific symptoms making diagnosis difficult, which could result in bowel ischaemia and systemic complications due to delay in the diagnosis.

• In this case, diagnosis by CT scan enabled prompt surgery and functional correction of the malrotation with a full return to normal life.