Management of Typical and Atypical Intestinal Malrotation
By John R. Mehall, John C. Chandler, Rachel L. Mehall, Richard J. Jackson,
Charles W. Wagner, and Samuel D. Smith
Little Rock, Arkansas
Background: “Atypical Malrotation”has been increasingly
diagnosed at the authors’ institution.
Methods: The authors reviewed retrospectively 201 consec-
utive operations for malrotation over 5 years to anatomically
classify, and describe results of operation for, atypical mal-
rotation. The ligament of Treitz (LOT) was classiﬁed as high
if left of midline and above the 12th thoracic vertebra, low if
left of midline and below the 12th thoracic vertebra, and
typical if absent or right of midline.
Results: A total of 201 patients underwent operation for
malrotation, in 176 there were adequate radiologic studies to
allow classiﬁcation of the LOT. Typical malrotation was
present in 75 patients, low LOT in 56, and a high LOT in 45.
Volvulus was more common in the Typical group compared
with the other 2 groups (12 of 75 v 1 of 56 low; 1 of 45 high;
P Ͻ .05) as were internal hernias (18 of 75 v 6of56low,1of
45 high; P Ͻ .05). Complications occurred in 13% of typical
versus 22% low and 21% of high patients (P ϭ .10). Low and
high LOT patients had 13% and 11% incidence of persistent
symptoms postoperatively versus 0% of typical patients.
Conclusion: Atypical malrotation patients are at signiﬁcantly
lower risk of volvulus and internal hernia compared with
typical malrotation patients, and operation appears to come
with increased morbidity.
J Pediatr Surg 37:1169-1172. Copyright 2002, Elsevier Sci-
ence (USA). All rights reserved.
INDEX WORDS: Intestinal malrotation, atypical malrotation.
HE CLINICAL ENTITIES collectively referred to
as malrotation encompass a wide variety of anat-
omy, ages, and clinical presentations. The classic pre-
sentation of malrotation occurs in the newborn period
with bilious vomiting and abdominal distension. Contrast
studies typically show the duodenum and ligament of
Treitz to be to the right of the midline and at operation
“Ladd’s Bands” extending across the duodenum from the
right upper quadrant to the cecum are divided.
malrotation also has been found in adults
and in a
variety on anatomic conﬁgurations between classic mal-
rotation and perfectly normal.
The presentation of mal-
rotation in older children is less dramatic and often is
misdiagnosed. Several investigators have tried to group
the various clinical presentations by age,
malrotation in older children is a distinct clinical entity.
At our institution there has been a dramatic increase in
the number of operations performed for malrotation in
recent years. This increase has been driven by a liberal-
ization of the radiologic criteria for malrotation. Cur-
rently, children whose anatomy is somewhere between
completely normal and classically malrotated are being
given the diagnosis of “atypical malrotation” or “malro-
tation variant” and are being referred for surgery. These
patients are a clinical and medicolegal challenge to the
surgeon who faces the dilemma of operating on patients
who do not have typical malrotation, and who may or
may not be symptomatic, but in whom the risks of not
operating are unknown. We reviewed retrospectively our
experience with malrotation over the last 5 years to better
understand this dilemma and to further deﬁne “atypical
malrotation.” In addition, we attempted to classify the
spectrum of malrotation based on anatomy to aid in
preoperative decision making and future prospective
studies of these patients.
MATERIALS AND METHODS
We reviewed retrospectively the records of consecutive patients
undergoing operation for malrotation, or in whom malrotation was
diagnosed at the time of operation, during a 5-year period (January
1995 to March 2000) at Arkansas Children’s Hospital. Arkansas
Children’s Hospital is a free-standing tertiary referral children’s hos-
pital serving the children of Arkansas and portions of adjoining states.
All operations were performed by 3 surgeons. Patients who had
abdominal wall defects (omphalocoele, gastroschisis) and diaphrag-
matic hernias were excluded from this analysis because these patients
represent unique subsets of intestinal rotational disorders. Charts were
reviewed for age at operation, sex, gestational age at birth, other
medical problems or anomalies, duration and type of preoperative
symptoms, type and results of imaging studies, ﬁndings at operation,
operation performed, duration of postoperative gastric decompression,
time required for resumption of oral intake, time from operation to
From the Department of Pediatric Surgery, Arkansas Children’s
Hospital, Little Rock, AR.
Presented at the 32nd Annual Meeting of American Pediatric Sur-
gical Association, Naples, Florida, May 20, 2001.
Address reprint requests to Samuel D. Smith, MD, Chief of Surgery,
Arkansas Children’s Hospital, 800 Marshall St, Slot 837, Little Rock,
Copyright 2002, Elsevier Science (USA). All rights reserved.
1169Journal of Pediatric Surgery, Vol 37, No 8 (August), 2002: pp 1169-1172