Chapter 40

Intestinal Malrotation
Klaas (N) M.A. Bax and David C. van der Zee

Introduction water-soluble contrast medium can be used. Complete

or partial obstruction gives a spiral (whirlpool or cork-
The transformation of the primitive gut from a simple screw) appearance of the proximal jejunum (Berdon
straight tube into the definitive folded and fixed con- 1995). In the absence of a volvulus, the duodenum has
figuration is a complex embryonic process in which a redundant Z-like appearance (Ablow et al. 1983). Ul-
both the gut and the mesenterium play an important trasonography can also demonstrate a dilated duode-
role. This process can be disturbed and can result in num with tapering configuration, fixed midline bowel,
different forms of malrotation and or malfixation and whirlpool sign (Chao et al. 2000).
(Touloukian and Smith 1998). This chapter will only Inversion of the relative position of the mesenteric
deal with incomplete rotation in which the duodenum vein and artery may also be an indication of intestinal
together with the terminal ileum and the superior malrotation. While the superior mesenteric vein nor-
mesenteric vessels are the contents of a narrow mesen- mally lies to the right of the artery, in malrotation the
teric stalk that is not fixed to the posterior peritoneum. vein lies to the left and more anteriorly. However, a
This configuration predisposes for volvulus of the high incidence of false-positives and false-negatives
whole small bowel and should be treated surgically as has been noted (Weinberger et al. 1992; Zerin and DiP-
soon as the diagnosis is made. iedro 1992). With ultrasound it is possible to diagnose
The clinical presentation of incomplete rotation intestinal volvulus in utero. Moreover combined with
may be that of an acute high gastrointestinal obstruc- Doppler it is possible to look at the vascularization of
tion or that of intermittent colicky abdominal pain. the twisted bowel segment (Yoo et al. 1999).
These symptoms seem to be caused by volvulus. The Malrotation can be diagnosed using computed to-
incidence of intestinal malrotation leading to clinical mography (CT) and magnetic resonance imaging
symptoms is estimated to be 1 in 6,000 live births, yet (MRI), using contrast agents (Ai et al. 1999; Marcos et
the incidence at autopsy is 0.5% and the incidence dur- al. 1999; Zissin et al. 1999). These examinations are
ing a barium meal 0.2% (Warner 1996). This seems to much more expensive and CT gives a much higher ex-
indicate that there are many asymptomatic cases. posure to X-rays. However these examinations may
Whether these cases have been truly asymptomatic re- help in finding other diagnostic parameters, for ex-
mains difficult to prove. ample aplasia of the uncinate process of the pancreas
Laparoscopy can be used either to treat symptom- (Zissin et al. 1999).
atic intestinal malrotation or to separate patients with In older children the diagnosis is often much more
doubtful intestinal malrotation on imaging into a difficult because of chronic and vague symptoms.
group that is prone to volvulus and that therefore re- Moreover various radiographic patterns of intestinal
quires treatment and into a group that is not. malrotation have been described (Long et al. 1996)
while the relationship with symptoms and possible
volvulus of these various forms of radiographically de-
Preoperative Preparation tected intestinal malrotation has not been clearly es-
tablished. To exclude malrotation, the duodenojejunal
Before the Induction flexure must be located to the left of the spine at the
of General Anesthesia level of the duodenal bulb halfway between the lesser
and greater curvature of the stomach. The question
The diagnosis of malrotation in symptomatic infants is however is not whether there is intestinal malrotation
usually not difficult. Abdominal cramps and bilious or not, but whether the intestinal malrotation predis-
vomiting in the absence of abdominal distension should poses for volvulus or not. If the ligament of Treitz is in
raise strong suspicion. An upper gastrointestinal series an equivocal position and not to the left of the spine at
with careful delineation of the duodenojejunal course L1 or L2 it is important to determine the position and
is the most important diagnostic tool and preferred to fixation of the cecum to assess the breadth of the mes-
a contrast visualization of the colon. As a contrast me- enteric pedicle. This can accurately be done by using
dium either air (Harrisson et al. 1999) or a low-osmolar laparoscopy (Waldhausen and Sawin 1996).
300 Abdomen – Gastrointestinal Tract

Fig. 40.1. Position of the

patient, crew and equipment.

In non-acute situations it is wise to washout the the table, which prevents the child from slipping down
large bowel in order to increase the working space. during the operation. In older children the legs can be
placed on abducted leg rests. Bandaging of the legs
fixes the child onto the table. Alternatively infants can
After Induction of General Anesthesia be placed transversely at the lower end of the operating
table.
In Utrecht often a locoregional technique is added to
general anesthesia. Especially in neonates it is impor- Crew, Monitors, and Equipment
tant to monitor respiratory gas exchange carefully. Hy- The surgeon stands at the lower end of the table or in
perventilation resulting in hypocarbia and respiratory between the legs in older children (7 Fig. 40.1). When
alkalosis should be avoided because of its negative in- the infant has been placed transversely, the surgeon
fluence on the brain. stands at the feet of the child. The camera operator
A urinary catheter is inserted in symptomatic chil- stands to the surgeon’s left side and the scrub nurse to
dren and in children receiving postoperative epidural the surgeon’s right side. The principal monitor stands
analgesia. to the right of the patient’s head or opposite the infant’s
head when the child has been placed transversely on
the table. Ideally all cables should all come from the
Positioning same direction.

Patient
The patient is positioned in a supine head up position Special Equipment
on the operating table (7 Fig. 40.1). In infants a short-
ened operating table should be used. The legs can then No special energy applying systems or special instru-
be enveloped with the lower end of the sheet covering ments are needed.
 Chapter 40 )NTESTINALÈ-ALROTATIONÈ 301

Technique

Cannulae

Cannula Method Diameter Device Position

of insertion (mm)
1 Open 6 Telescope 30° Infraumbilical fold
2 Closed 3.5 Surgeon’s left-hand instrument Pararectally at umbilical level
on the right
3 Closed 3.5 Surgeon’s right-hand instrument Pararectally at umbilical level
on the left
4 Closed 3.5 Diamond Flex liver retractor Subxiphoidal
or Kelly-type forceps

Fig. 40.2. Port position Procedure

Symptomatic Malrotation
It is not difficult to confirm the preoperative diagnosis.
The cecum and appendix are usually in a high and rath-
er medial position below the liver, to which they are at-
tached by a peritoneal band. The second part of the
duodenum looks long and tortuous. In symptomatic
neonates, there is always a volvulus of the mesenteric
stalk in a clockwise direction, which is easily recognized
(7 Fig. 40.3a,b). When the bowel is not acutely isch-
emic, it is advantageous to leave the volvulus initially, as
it retracts the bowel to the left. All peritoneal bands be-
tween the liver, retroperitoneum, and bowel are tran-
sected, and the duodenum is kocherized (7 Fig. 40.4).
Next the volvulus is undone in a counterclockwise di-
rection. The short band (anterior leaf of the mesenteric
stalk) fixing the ileocecal region to the duodenum is
transected (7 Fig. 40.5a,b) and the ileocecal region is
displaced to the left thereby widening the mesentery an-
Cannula Insertion teriorly. Care should be taken not to open the mesentery
of the ascending colon. To make sure that the malrota-
The first cannula is inserted in an open way through the tion is treated properly, the whole duodenum as well as
inferior umbilical fold and will contain the telescope the jejunum should lie on the right side. The easiest way
(5 mm, 30°). Two secondary cannulae are inserted, to obtain this is to free the duodenum in the distal direc-
each one pararectally at the umbilical level (7 Fig. 40.2). tion transecting all bands. During this dissection a ring-
These can be 3.5 mm in diameter for 3 mm instruments like peritoneal band encircling the duodenum becomes
for children below 1 year of age, or 6 mm in diameter apparent and should be transected anteriorly. This band
for 5 mm instruments for older children. In infants represents the end of the retroperitoneal part of the
20 cm-long instruments are used and in older children duodenum. The jejunum comes now into view. By pull-
30 cm-long instruments are used. It may be advanta- ing further and further on the duodenum and later on
geous to insert a fourth 3.5 mm cannula just under the the jejunum, the whole small bowel obtains a right-sid-
xiphoid process for a Diamond Flex liver retractor or ed position. A last check is made whether the anterior
for a Kelly-type forceps for the assistant. mesentery has been widened enough. If not, the ante-
rior leaf of the mesentery is further incised distally and
the adjacent bowel further displaced to either side.
Carbon Dioxide Pneumoperitoneum Whether the appendix should be removed at the
same time is debatable. If it is to be removed, the tip is
A pressure of 8 mmHg suffices when optimal muscle grabbed with the forceps in the left cannula and the
relaxation is guaranteed. Flow is arbitrarily set at 2 L/ appendix as well as cannula are withdrawn together
min in children below 1 year of age and at 5 L/min in (7 Fig. 40.6). The appendix is then removed outside
children beyond that age. the body and its stump repositioned.
302 Abdomen – Gastrointestinal Tract

a a

b b

Fig. 40.3. a View of a volvulus of the entire small bowel. The Fig. 40.5 a,b. After anticlockwise detorsion, the mesenteric
bluish color signifies ischemia. b Note the edematous me- stalk is widened anteriorly by cutting the anterior mesenteric
senteric stalk leaf. Veins dilated as a result of the volvulus are easily visible

Fig. 40.4. The operation starts with the mobilization of the Fig. 40.6. Removal of the appendix is optional. When appen-
right colon. Adhesive bands between the right colon and the dectomy is planned, the appendicular vessels should be cau-
right lateral abdominal wall are divided. Next the duodenum terized internally first, after which the appendix can be exteri-
is kocherized orized through the left paraumbilical port
 Chapter 40 )NTESTINALÈ-ALROTATIONÈ 303

broadness of the mesentery as well as its posterior fixa-

tion can easily be verified (7 Fig. 40.7). When there is
no malrotation, one should look at the ileocecal region
as well. A normally fixed ileocecal region argues against
malrotation (7 Fig. 40.8).

Postoperative Care

In symptomatic malrotation a nasogastric tube is left

behind until gastric retention has ceased. Normal feed-
ing can then be started. The same applies for cases of
doubtful malrotation in which at operation classic
malrotation was proven and needed to be treated.
When the diagnosis of malrotation could not be sub-
stantiated, the patient can be discharged the same day.
Fig. 40.7. In case of doubtful malrotation, visualization of the
ligament of Treitz is of paramount importance. In this patient
it can be seen that the first jejunal loop is normally suspended
to the left of the vertebral column Results

In the period 1994–2003, 15 neonates with intestinal

malrotation were approached laparoscopically. All
presented with biliary vomiting since birth. Median
birth weight was 3,540 g (2,210–4,390 g). Median age
at operation was 7.5 days (2–34 days). At laparoscopy
all proved to have volvulus but without necrosis.
Conversion was carried out in six patients, five
times through a formal laparotomy, and once through
a minilaparotomy. The reasons for conversion were:
technical pneumoperitoneum problems, unclear anat-
omy, not enough progress, chylous ascites, fibrotic
mesentery, and complex congenital anomalies, each in
one patient.
In one patient the diagnosis of malrotation was
missed at laparoscopy. At repeat but now open surgery
a volvulus without necrosis was still present. Two non-
converted patients presented with repeat volvulus. One
Fig. 40.8. The ileocecal region looks normal too. The appen- patient in the converted group developed adhesive
dix is attached with a band to the right lateral abdominal small bowel obstruction. There was no mortality.
wall In the same period 15 children were laparoscoped
because of doubtful malrotation on upper gastrointes-
tinal tract series made because of vomiting. Median
Doubtful Malrotation age was 208 days (30–2,934). Two children were known
If malrotation can not be excluded on imaging, lapa- to have situs inversus. In all children a broad mesen-
roscopy is an ideal tool to look at the width and fixa- teric stalk well fixed to the posterior peritoneum was
tion of the mesentery. If the mesentery is broad, the found, making a volvulus unlikely.
likelihood for volvulus is small and no further surgery During the same period under study, one 5.5-year-
is required. In contrast if the mesentery is narrow, the old girl with obstruction was laparoscoped and had a
stalk should be widened. paracolic hernia, which is considered a form of malro-
The position of the cannulae is the same as in symp- tation. Partial removal of the sac was curative.
tomatic malrotation. In patients without typical symp- Finally we have also laparoscoped three children,
toms but with a low and rather medially placed duode- aged 13, 15.5, and 16 years, with so-called superior
nojejunal flexure, usually a normal colon is seen with mesenteric artery syndrome. This has also be consid-
normally inserted mesentery of the transverse meso- ered to be a form of intestinal malrotation. In all the
colon onto the pancreas. By lifting up the transverse duodenojejunal flexure was detached but the results
colon, the duodenojejunal flexure is visualized and the have been disappointing.
304 Abdomen – Gastrointestinal Tract

Discussion References

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