STANFORD MULTIDISCIPLINARY SEMINARS

Adult Intestinal Malrotation: When Things Turn the Wrong Way

Owen P. Palmer • Horace H. Rhee •

Walter G. Park • Brendan C. Visser

Received: 27 June 2011 / Accepted: 29 June 2011 / Published online: 30 July 2011

� Springer Science+Business Media, LLC 2011

Case Presentation and Evolution

A 26-year-old man presented to a local emergency

department with hematemesis and melena associated with

mild mid-epigastric abdominal discomfort and light-head-

edness. He had no prior history of gastrointestinal (GI)

bleeding, but he recalled having as a young child, up to

10 years of age, intermittent self-limited attacks of crampy

abdominal pain that were never severe enough to prompt

evaluation. He had no other significant medical history. His

past surgical history was notable only for skull surgery as

an infant. He took no medications on a regular basis, and

used non-steroidal anti-inflammatory drugs infrequently.

From 18 to 21 years of age, he indulged in heavy alcohol

use but currently had only 3–4 drinks per week. On pre-

sentation, his hemoglobin was 13.7 g/dl and his blood urea

nitrogen 29 mg/dl; otherwise, his laboratory values were

unremarkable. An esophagogastroduodenoscopy (EGD) to

the second portion of the duodenum reportedly showed

mild gastritis and no esophageal varices. He was sent home

on a proton-pump inhibitor after he was observed 24 h

without further GI bleeding.

Within 12 h of discharge from the emergency depart-

ment, he experienced recurrent melena and light-headed-

ness. On return to the hospital, he had tachycardia, his

hemoglobin had dropped to 7.3 g/dl, and his blood urea

nitrogen was now 36 mg/dl. He was resuscitated with four

units of packed red blood cells. A computed tomography

(CT) scan of the abdomen revealed a vascular abnormality

of the superior mesenteric vein (SMV) with possible

intestinal malrotation. The patient was transferred to our

center for further evaluation and possible intervention.

On admission, his post-transfusion hemoglobin was

10.1 g/dl, and he was experiencing no further GI bleeding.

A triphasic CT scan was obtained to further characterize

the lesion noted on the previous CT scan (Fig. 1). In the

third portion of the duodenum, an enhancing lobulated

structure was seen draining into a large vein. Radio-

graphically, this structure appeared to be a varix, but a

hypervascular mass remained in the differential diagnosis.

‘‘Swirling’’ of the SMV around the superior mesenteric

artery was noted to be consistent with intestinal malrota-

tion. A short segment of the superior mesenteric artery

(SMA) was thrombosed with reconstitution distally. There

was no evidence of ischemic bowel. No other varices were

noted.

As the patient was clinically stable, an EGD with

endoscopic ultrasound (EUS) was performed and it

revealed a raised structure in the third portion of the duo-

denum with normal overlying mucosa (Fig. 2a). EUS

revealed the underlying structure to be hypervascular, most

consistent with a vascular malformation (Fig. 2b). At the

apex of the lesion there was a small ulcerative defect

(arrow), which was the likely source of bleeding.

O. P. Palmer (&) � B. C. Visser

Department of Surgery, Stanford University Medical Center,

Stanford, CA, USA

e-mail: oppalmer@stanford.edu

B. C. Visser

e-mail: bvisser@stanford.edu

H. H. Rhee � W. G. Park

Department of Medicine, Division of Gastroenterology

and Hepatology, Stanford University Medical Center,

Stanford, CA, USA

e-mail: hrhee@stanford.edu

W. G. Park

e-mail: wgpark@stanford.edu

123

Dig Dis Sci (2012) 57:284–287

DOI 10.1007/s10620-011-1818-4

At exploratory laparotomy the third and fourth portions

of the duodenum tracked anteriorly, as expected with his

diagnosis of malrotation. The venous malformation was

immediately apparent (Fig. 3a). A cholecystectomy was

performed to enable access to the biliary tree. A 5 French

pediatric feeding tube was passed via the cystic duct into

the duodenum to ensure the resection was distal to the

ampulla. The duodenum was divided with a gastrointestinal

anastomosis (GIA) stapler at the ligament of Treitz.

Mobilization of the duodenum revealed the varix inserting

into the posterior duodenal wall (Fig 3b). The lumen of the

duodenum was opened and inspected in situ to ensure the

entire vascular lesion had been removed (Fig. 3c). The

duodenal mesentery of the third and fourth portions of the

duodenum was ligated. The duodenum was divided prox-

imal to the vascular malformation. He then underwent a

Ladd’s procedure to correct the malrotation. A primary

hand-sewn side-to-side duodeno-jejunostomy was per-

formed. On pathologic review, there was evidence of a

venous malformation but no tumor was found. Both the

gallbladder and appendix were normal. The resected lesion

is shown in Fig. 3d.

The anastomosis showed no evidence of an anastomotic

leak in a small bowel follow-through study on post-oper-

ative day 2 (Fig. 4). He tolerated diet well and he was

discharged home on post-operative day 5. On six-week

outpatient follow-up, he was recovering well, tolerating a

regular diet, and was free from abdominal pain or further

episodes of GI bleeding.

Discussion

Intestinal malrotation is a diagnosis primarily made very

early in life. Approximately 80% of all cases will present in

the first month of life and 90% of all cases within the first

year. The incidence of malrotation in adults is rare, esti-

mated to be approximately 0.2% of the population [1].

Acute symptoms of malrotation most often present as

severe abdominal pain from volvulus. In contrast, chronic

symptoms of malrotation usually present as a more vague

intermittent abdominal pain, nausea, vomiting, dyspepsia,

and gradual weight loss. Chronic symptoms of malrotation

often remain mis-diagnosed [2, 3]. Our patient may have

had some chronic symptoms (intermittent abdominal pain)

as a young child, but these had completely resolved during

his adult years.

In this patient, although the venous malformation and

intestinal malrotation might be incidentally related, it is

possible that the torsion on the mesenteric vessels caused

Fig. 1 Images from the porto-venous phase of a triphasic axial CT of

the abdomen. In A, the varicosities can be been seen in the third

portion of the duodenum (solid arrow). The anterior course of the

duodenum is evident (solid line). In B, ‘‘swirling’’ of the SMV around

a thrombotic segment of the SMA is seen (dashed arrow). The SMA

quickly reconstitutes with contrast (not shown)

Fig. 2 The lesion, as seen

endoscopically (a), was noted to

be soft and compliant with air

insufflation. Endoscopic

ultrasound revealed a

hypervascular lesion (b)

Dig Dis Sci (2012) 57:284–287 285

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by the malrotation led to long-term partial vascular

obstruction. The presence of a segmental length of SMA

thrombosis with distal reconstitution shows that the arterial

side was affected. We postulate that the varicosities, which

led to his presentation of GI bleeding, developed secondary

to long-standing partial venous occlusion of the SMV.

This case shows that intestinal malrotation can present,

albeit rarely, as an upper GI hemorrhage and illustrates that

CT of the abdomen provides sufficient accuracy to make

the diagnosis [4]. The treatment of choice involves surgical

resection of the involved segment of bowel and Ladd’s

procedure [5]. First described by Dott in [6] and later by

Ladd in [7], surgical intervention has remained the primary

treatment for malrotation. Four steps of the Ladd’s opera-

tion are classically described: counterclockwise detorsion

of any intestinal volvulus, ligation of the adhesive perito-

neal attachments known as Ladd’s bands to allow mobili-

zation of the bowel, broadening of the mesenteric base with

restoration of the bowel to its embryological non-rotated

state, and an incidental appendectomy [6, 7]. Broadening

the mesenteric base reduces the risk of recurrent volvulus

around a narrow vascular pedicle. Because the appendix is

located in the left lower quadrant after completion of a

Ladd’s procedure, an appendectomy prevents the possi-

bility of future diagnostic confusion because of left-sided

appendicitis.

Fig. 3 Intra-operative photographs show the lesion before dissection

(a). The third portion of the duodenum (Duo) is notably anterior. The

gallbladder (Gb) and liver (L) are labeled for reference. The posterioraspect of the duodenum is seen after dissection with the varicosity

(arrow) inserting directly into the back wall of the duodenum (b). The

specimen was opened in situ to confirm complete excision of the

lesion (c); a small amount of bleeding from the lesion can be seen

(arrow). The final sample included the third and fourth portion of the

duodenum (d)

Fig. 4 Post-operative abdominal X-ray with contrast showed no

evidence of a leak. The colon is seen entirely in the left hemi-

abdomen as expected after the Ladd’s procedure

286 Dig Dis Sci (2012) 57:284–287

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This case further emphasizes the rationale for correcting

even incidentally discovered cases of intestinal malrotation

in both adults and children [1, 5, 8–10]. Intestinal malro-

tation should be considered in adults with otherwise

unexplained abdominal symptoms.

Key Messages

• Intestinal malrotation can lead to mesenteric varicosi-

ties and present as a GI bleeding.

• Malrotation should be included in the differential

diagnosis for adults with otherwise unexplained abdom-

inal symptoms. CT scan of the abdomen provides

sufficient accuracy to establish the diagnosis.

• Ladd’s procedure is the treatment of choice for patients

found to have malrotation, even if found incidentally.

References

1. Kapfer SA, Rappold JF. Intestinal malrotation—not just the

pediatric surgeon’s problem. J Am Coll Surg. 2004;199:628–635.

2. von Flue M, Herzog U, Ackermann C, Tondelli P, Harder F.

Acute and chronic presentation of intestinal nonrotation in adults.

Dis Colon Rectum. 1994;37:192–198.

3. van den Hoven I, Roumen RM. Chronic recurrent crampy

abdominal pain owing to partial intestinal malrotation. Surgery.

2010;147:893–894.

4. Pickhardt PJ, Bhalla S. Intestinal malrotation in adolescents and

adults: spectrum of clinical and imaging features. AJR Am JRoentgenol. 2002;179:1429–1435.

5. Durkin ET, Lund DP, Shaaban AF, Schurr MJ, Weber SM. Age-

related differences in diagnosis and morbidity of intestinal mal-

rotation. J Am Coll Surg. 2008;206:658–663. Epub. 2008/02/01.

6. Dott NM. Anomalies of intestinal rotation: their embryology and

surgical aspects with reports of five cases. Br J Surg. 1923;

251–286.

7. Ladd W. Congenital obstruction of the duodenum in children. NEngl J Med. 1932;206:277–283.

8. Mehall JR, Chandler JC, Mehall RL, Jackson RJ, Wagner CW,

Smith SD. Management of typical and atypical intestinal mal-

rotation. J Pediatr Surg. 2002;37:1169–1172.

9. Penco JM, Murillo JC, Hernandez A, De La Calle Pato U,

Masjoan DF, Aceituno FR. Anomalies of intestinal rotation and

fixation: consequences of late diagnosis beyond two years of age.

Pediatr Surg Int. 2007;23:723–730.

10. Malek MM, Burd RS. Surgical treatment of malrotation after

infancy: a population-based study. J Pediatr Surg. 2005;40:285–

289.

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