Intestinal Malrotation

and Volvulus 27
Luisa Ferrero, François Becmeur,
and Olivier Reinberg

27.1 Introduction of the duodenum and wrote a reference article on

the treatment of malrotation, describing his surgi-
Intestinal malrotation is a common name for a large cal approach, the “Ladd’s procedure,” which still
variety of abnormalities of intestinal rotations and remains today the basic rules of good practice
attachments that occur during fetal development. [1]. By the 1950s Snyder and Chaffin [6] in the
United States and Grob [7, 8] in Switzerland have
done much to clarify our understanding of these
27.2 History malformations. Most of the available drawings
are reproductions of the initial drawings by Grob.
The first cases of malrotation were reported in the
literature in the mid-1700s [1, 2]. No progress
was made until normal intestinal embryology 27.3 Epidemiology
was first described by Meckel in 1817 [3] and
then later by Mall in 1897 who wrote the first The true incidence of malrotation remains
description of the embryology of the midgut [4]. unknown. According to literature it ranges from
In 1923, Dott applied the embryology to the 1/6000 [9, 10] to 1/200 [11] of all live births.
understanding of intestinal malrotations. Based Autopsy studies estimate that it may be as high as
only on five clinical observations, he correlated 1% of the total population [9, 12]. Males are
them with the sequences of embryological devel- more frequently affected than females with a
opment describing theoretically many of the ratio of 2:1 [6, 13, 14].
potential errors and their consequences without Case reports have suggested a strong concor-
having seen them [5]. In 1931, William E. Ladd dance of intestinal malrotation between identical
presented in a seminar five cases of obstruction twins and even between non-twin siblings.
Genetic factors may thus play some role in its
L. Ferrero pathogenesis [15–18].
Department of Pediatric Surgery, Regina Margherita Most cases of malrotation are discovered in
Children’s Hospital, Turin, Italy the first few months of life, and of those, most
F. Becmeur will present within the first week of life.
Department of Pediatric Surgery, University Approximately 90% are discovered before 1 year
Hospitals, Strasbourg, France
e-mail: Francois.Becmeur@chru-strasbourg.fr of age. However a significant percentage of
patients reach adulthood with an undiagnosed
O. Reinberg (*)
Department of Pediatric Surgery, Lausanne, malrotation [19]. Unfortunately, for those patients
Switzerland delays in diagnosis are common.

© Springer Nature Switzerland AG 2019 369

M. Lima, O. Reinberg (eds.), Neonatal Surgery, https://doi.org/10.1007/978-3-319-93534-8_27
370 L. Ferrero et al.

27.4 Embryology cord with the SMA forming the axe of a U-loop
in the sagittal plane (Fig. 27.1a).
The final anatomic arrangement of the midgut As it protrudes, the midgut makes a first rota-
follows a complex series of events [6]. By the 4th tion of 90° counterclockwise, so its distal part
week of fetal life, the embryo is about 5 mm, and comes to the left, and its proximal part is to the
the primitive intestine is an almost straight tube, right. The loop is now in a horizontal plane. The
the same length as the ectodermal and mesoder- distal part of the loop develops a pouch that will
mal germ layers, and lays on the midline. It con- become the cecum. The proximal part of the loop
sists, cephalad-caudally, in the foregut, midgut, becomes tortuous. These loops still lie outside
and hindgut. the abdominal cavity (Fig. 27.1b).
Rotation of the midgut happens during the 2nd By 10 weeks, the body of the embryo is now
month of the fetal life, to become the medial part large enough for the bowel to develop inside the
of the gastrointestinal tract (GIT). At this stage abdomen, so the midgut reintegrates the abdo-
the midgut is in continuity with the vitelline duct men. The proximal part of the loop returns first. It
inside the umbilical duct and still connected to passes under the distal one and comes to the left
the yolk sac. The aorta gives blood supply to the making a second 90° counterclockwise rotation.
GIT through three arteries, respectively, the coe- Then the distal part follows it passing in front of
liac artery for the foregut, the superior mesenteric the proximal part and rotates to the right. This is
artery (SMA) for the midgut, and the inferior making the third 90° counterclockwise rotation.
mesenteric artery for the hindgut. With this development, the proximal part of the
The GIT develops faster than the coelomic midgut becomes placed posteriorly to the distal
cavity resulting in a lack of space. Thus by one that will become the transverse colon with
6 weeks, it forced to herniate inside the umbilical the SMA between them (Fig. 27.1c).

a b c

Fig. 27.1  Normal embryological development of the of 90° counterclockwise, so its distal part comes to the
midgut [23]. (a) The midgut has not rotated yet and left, and its proximal part is to the right. The loop is now
remains in a sagittal plane. The aorta gives blood supply to in a horizontal plane. The distal part of the loop develops
the GIT through three arteries, respectively, the coeliac a pouch that will become the cecum. (c) The second 90°
artery for the foregut (up), the superior mesenteric artery counterclockwise rotation. The proximal part of the loop
(SMA) for the midgut (middle), and the inferior mesen- returns first, passes under the distal one, and comes to the
teric artery for the hindgut (lower). It forms an U-loop left. The distal part follows it passing in front of the proxi-
around the SMA. (b) The midgut has made a first rotation mal part and rotates to the right
27  Intestinal Malrotation and Volvulus 371

At this time, both parts of the midgut have which errors occur during these organogenetic
rotated 270° in a counterclockwise manner. Then steps either by omission or by opposite rotation(s).
the period of fixation lasts until after birth. The Three main types are described, i.e., nonrotation,
descending and ascending colon mesenteries fuse partial malrotation, and reversed malrotation [6,
with the retroperitoneum, and the small bowel is 9–11, 13, 21–23].
fixed by a broad mesentery from the duodenoje-
junal junction in the left upper quadrant to the
ileocecal valve in the right lower abdomen. The 27.5.1 Complete Nonrotation
broad base of the small bowel mesentery stabi-
lizes its position and prevents volvulus. In nonrotation, only the initial 90° counterclock-
According to Kluth, this description of the wise rotation occurs so that the duodenojejunal
processes of rotation is schematic. It has been junction lies on the right side and the colon lies of
done in order to better explain the background the left side of the SMA. It is characterized by the
of the pathology of malrotation than to study the small and large bowel coursing vertically and a
embryology of the midgut. Using his technique common longitudinal mesentery [23]. This mal-
of scanning electron microscopy pictures of the rotation is often called “left-sided colon”
developing midgut in a series of rat embryos, he (Fig. 27.2).
demonstrated that the primum movens of the
process was not the rotations but the lengthen-
ing of the bowel, mainly the small bowel, in a 27.5.2 Partial (Incomplete)
small cavity. Thus the bowel components enter Malrotation
into a position where space allows [20].
Partial (incomplete) malrotation implies a failure
of the midgut loop to complete the final 90°
27.5 Classification counterclockwise rotation; thus, terminal ileum
enters the abdominal cavity first. This term is
The term malrotation comprises a range of ana- used in all cases of anomalies in the arrangement
tomical anomalies in the arrangement of GIT in of the midgut ranging from a nonrotation to a
the abdominal cavity, each reflecting the time at normal rotation. In the most common forms, the

a b c

Fig. 27.2  Nonrotation. (a) Pure form; (b) with colonic the ileocecal valve faces to the right (Drawings from Max
adhesion to the stomach; (c) with a right position of the Grob) [8]
proximal colon compressing the second duodenum. Note:
372 L. Ferrero et al.

a b

Fig. 27.3  Partial (incomplete) rotation: two forms (a and extrinsic obstruction. (b) This is one of the commonest
b). In form (a) the cecum lies below the pylorus and is malrotations. Counterclockwise rotations have stopped at
fixed to the posterior abdominal wall by peritoneal bands 180°. Note: the ileocecal valve faces to the left (Drawings
(“Ladd’s bands”) that cross over the duodenum and cause from Max Grob) [8]

cecum lies below the pylorus and is fixed to the 180°, and the duodenojejunal loop has failed to
posterior abdominal wall by peritoneal bands cross the midline and lies to the right of the
(“Ladd’s bands”) that cross over the duodenum SMA.  The caecocolic loop has rotated from
and cause extrinsic obstruction. The duodenum almost 180° but no further and lies anterior to the
and small bowel are located on the right side of duodenum and to the SMA. Congenital adhesive
the SMA and the cecum and colon on the left bands (“Ladd’s bands”) course from the cecum to
(Fig. 27.3). the parietal peritoneum usually obstructing the
second part of the duodenum [23] (Fig. 27.4).

27.5.3 Malrotation
27.5.4 Reversed Malrotation
This term refers to anomalies occurring during
the second rotation. Several types have been When rotation is clockwise, the result is said
described according to the degree of rotation reversed malrotation in which the duodenum lies
accomplished. In the commonest type, the rota- anteriorly to the colon. Small intestine lies on the
tion has stopped at some point just before the left and large intestine on the right. The cecum is
27  Intestinal Malrotation and Volvulus 373

a b

Fig. 27.4  Malrotations: (a) these are results from reverse right in front of the duodenum. A compressive Ladd’s
second rotation following the initial counterclockwise band compresses the second duodenum (Drawings from
rotation. (b) In this type of malrotation, the cecum came Max Grob) [8]
from behind the mesentery and has passed toward the

found in the midline. Duodenum lies in front of 27.6 Associated Anomalies

the SMA and transverse colon behind it, which
may cause an extrinsic colonic obstruction. This Malrotation may occur in association with
is a rare malrotation (Fig. 27.5). other congenital abnormalities or syndromes
In addition to these terms, defining malrota- with wide differences according to authors
tions and abnormal fixation of the mesentery ranging from 17 to 60% of patients [14, 22,
must be mentioned as it may cause intestinal 25]. The most common associated ones are
internal herniation (“hernia mesocolica”) [14, other gastrointestinal abnormalities, espe-
24] (Fig. 27.6). cially jejunal and duodenal stenosis or atre-
In individuals with malrotation, the mesenteric sia, annular pancreas, and Hirschsprung’s
attachment of the midgut, particularly the portion disease. Malrotation of the bowel is always
from the duodenojejunal junction to the cecum, is present in children with congenital abdomi-
abnormally short. The gut is therefore prone to nal wall defects (omphalocele and gastros-
twist counterclockwise around the SMA.  This chisis) or congenital diaphragmatic hernia in
condition is known as midgut volvulus (Fig. 27.7). whom the normal embryologic positioning of
374 L. Ferrero et al.

a b

Fig. 27.5  Two types of reverse completed 180° clock- right side of the abdomen. (b) The colon can be com-
wise rotation. (a) The transverse colon comes to lie behind pressed by the SMA (Drawings from Max Grob) [8]
the SMA, but the cecum and proximal colon are in the

Fig. 27.7  Boy 3 weeks old. Urgent laparotomy for acute

Fig. 27.6  “Hernia mesocolica”: abnormal fixation of the midgut volvulus in a partial rotation. Discoloration with-
mesentery may cause intestinal internal herniation out necrosis. No resection
27  Intestinal Malrotation and Volvulus 375

the developing gut was disrupted. But many volvulus becomes complicated by intestinal
other malformations have been occasionally gangrene, perforation, and peritonitis. A high
described in association with malrotations index of suspicion for midgut volvulus is based
such as absence of the kidney or ureter, on the history, physical examination findings,
esophageal atresia, biliary atresia, imperfo- and presence of metabolic acidosis. A delay in
rate anus, and intestinal pseudo-­o bstruction. diagnosis and treatment may result in small
Several syndromes are associated with mal- bowel necrosis, short gut syndrome, and depen-
rotations. It may be present in patients with dence on TPN. Mortality in affected newborns
heterotaxy syndrome (asplenia or right isom- was approximately 30% by the 1950s and 1960s
erism and polysplenia or left isomerism). but today has markedly decreased down to
Patients presenting with this syndrome should 3–5% [32].
be investigated for the possibility of malrota-
tion [26]. It has also been described in asso-
ciation with Cornelia de Lange, cat eye, 27.8 Diagnostic Imaging
Coffin-Siris, Marfan, Prune-Belly syndromes, Investigations
and trisomy 21 [27].
Clinical diagnosis of malrotation must be con-
firmed by investigations.
27.7 Clinical Presentation Plain abdominal radiographs (Rx) are neither
sensitive nor specific for intestinal malrotation
The prenatal diagnosis of malrotation can be sug- [25, 33, 34]. They are usually performed to evi-
gested by identification of its complications, such dence an occlusion (Figs.  27.8a and 27.9a). A
as bowel dilatation, ascites, or meconium perito- duodenal obstruction gives a typical image of the
nitis, that can be evidenced on ultrasounds (US). double bubble sign whatever the cause, i.e., duo-
With US it is possible to diagnose intestinal vol- denal atresia or high located volvulus [21, 25].
vulus in utero. Combined with Doppler it gives Plain abdominal Rx may yield hints of abnor-
information on the viability of the involved intes- mally located bowel, e.g., small bowel markings
tinal segment [28–30]. predominantly on the right and large bowel on
Various clinical presentations may result from the left. Such findings should prompt further
failure of normal intestinal rotation and fixation, investigations. However a patient with midgut
ranging from chronic abdominal pain to acute volvulus may have a normal radiograph.
midgut volvulus. The most common features in The upper GI series (UGI) remains the imag-
newborns are bilious vomiting with or without ing reference standard for the diagnosis of malro-
abdominal distention associated with either duo- tation with or without volvulus [21, 22, 25, 35].
denal obstructive bands or midgut volvulus [13, Normally the duodenum descends to the right
25, 31]. Clinical diagnosis of malrotation with of the midline, courses transversely to the left,
volvulus is based on a high index of suspicion. and then ascends to the left of the midline at the
The major complications of malrotation is a level of the pylorus; thus the duodenojejunal
midgut volvulus and infarction of the bowel junction is located to the left of the vertebral body
that can be life-threatening if total or without at the level of the duodenal bulb on a standard AP
fatal issue can lead to a significant loss of bowel view [25], and the loops of the proximal jejunum
with a subsequent short bowel syndrome and are seen on the left of the midline. On a lateral
dependence on total parenteral nutrition (TPN). view, the duodenojejunal junction is located pos-
The infant presents in a shocked and collapsed teriorly [25]. However, variations of the normal
state with bilious vomiting (which often con- location may appear, particularly on frontal views
tains altered blood), abdominal tenderness with in the upper GI series, that mimic malrotation
or (more commonly) without distension, and [25, 36]. A grossly distended stomach may dis-
the passage of dark blood rectally. Edema and place the bulb. Then the stomach must be emp-
erythema of the abdominal wall develop as the tied and the position of the flexure reassessed.
376 L. Ferrero et al.

a b

Fig. 27.8 Boy D5. Partial rotation with obstructive that fails to cross midline looking down below the level of
Ladd’s bands. (a) Plain abdominal Rx performed to evi- the duodenal bulb. The proximal jejunal loops are in the
dence an occlusion showing gastric distension. Note that right abdomen. (c) UGI lateral view; the duodenojejunal
some gas has passed below the duodenum. (b) UGI AP junction has an anterior location
view; abnormal position of the duodenojejunal junction
27  Intestinal Malrotation and Volvulus 377

a b

c d

Fig. 27.9  Boy D15. Nonrotation type and volvulus. (a) that fails to cross midline looking down below the level of
Plain abdominal radiographs performed to evidence an the duodenal bulb. (d) The proximal jejunal loops are in
occlusion showing bowel distension. (b and c) UGI AP the right abdomen
view; abnormal position of the duodenojejunal junction

In infants, an inferior displacement of a normal In malrotation, the distal duodenum has an

duodenojejunal junction is a common variation abnormal course. On a strict AP view, this appears
seen on AP views. This is even more common in as an abnormal position of the duodenojejunal
prematures. It could be due to a relative mobile junction that fails to cross midline and is located
ligament of Treitz [36]. to the right of the vertebral body and, in some
378 L. Ferrero et al.

cases, below the level of the duodenal bulb torsion, described as “bird’s beak,” “corkscrew,”
(Figs.  27.8b and 27.9b, c). The duodenojejunal “twisted ribbon,” or “coiled” in appearance
junction may have an anterior location that can according to authors [21, 25, 37].
be depicted on a lateral view (Fig.  27.8c). In The sensitivity of the UGI series for the diag-
addition, in some malrotations, the duodenojeju- nosis of malrotation has been reported as
nal flexure may disappear making its localization 93–100%, but a sensitivity of only 54% was
difficult. Without occlusion, the contrast media reported for the diagnosis of midgut volvulus
demonstrates the presence of the proximal jeju- [25, 37].
nal loops in the right abdomen (Fig. 27.8d). The By 1987, ultrasounds (US) has been intro-
cecum is abnormally positioned in 80% of duced as an alternative for the diagnosis of mal-
patients with malrotation [21, 25, 35]. A midgut rotation, with emphasis on the relationship of
volvulus produces an obstruction of the descend- the superior mesenteric vessels and in the detec-
ing distal duodenum or the proximal jejunum tion of the so-called “whirlpool sign” in cases of
with the appearance of extrinsic compression and volvulus [38–42] (Fig. 27.10a, b). This is due to

a b

Fig. 27.10  Boy D12. Nonrotation type and volvulus. (a, b) US, whirlpool sign. (c) Peroperative view of the volvulus
27  Intestinal Malrotation and Volvulus 379

the rotation of the superior mesenteric vessels display the relationship between SMV and
associated with the twist of the bowel. Normally SMA as well as signs of volvulus such as the
the superior mesenteric vein (SMV) lies to the “whirlpool” sign. CT and MRI can also depict
right of the superior mesenteric artery (SMA). the location of both small and large bowel. An
In malrotation the SMV is coiling around the additional advantage of these imaging tech-
artery coming left to the SMA and more anteri- niques is that other abnormalities, in associa-
orly. The highest sensitivity is achieved when tion with syndromes or anomalies, can be
the “whirlpool sign” is shown, several studies illustrated. However CT is not considered to be
suggesting it to be diagnostic in 100% of the the first imaging modality of choice due to the
cases [12, 21, 37, 43–45]. related irradiation and should be restricted to
US has the potential benefits of portability and some unusual cases.
lack of radiation. Although US is an excellent
imaging modality, the results are strongly opera-
tor dependent. Additionally, due to the superim- 27.9 Treatments
posed intestinal air, both the SMV and the SMA
are not always clearly detectable. Orzech et  al. The surgical treatment of a malrotation includes:
reported sensitivity of 86.5%, specificity of 75%,
positive predictive value of 42%, and negative • Careful inspection of the bowel and of the
predictive value of 96% for US [31]. Several mesenteric root in order to recognize the type
studies have suggested that inversion of the supe- of malrotation. A precise description is better
rior mesenteric vessels, (i.e., the SMV to the left than the use of a classification type.
of the SMA), is diagnostic of malrotation in • Detorsion of the volvulus counterclockwise if
100% of the cases [12, 37, 43, 44]. Consequently, present.
an abnormal US study requires further radiologic • Lysis of all abnormal bands and adhesions of
and clinical investigation. peritoneum, the so-called Ladd’s bands,
However, it has been shown by other authors between the cecum and the duodenum. This is
that inversion of the SMV/SMA relationship can known as the “Ladd’s procedure” [1]
also be seen in patients with normal midgut rota- (Fig. 27.11).
tion [12, 21, 43] and in patients with abdominal • Straightening and freeing of the duodenum
masses and distal ileocolic intussusception [46]. such that it descends directly into the right
Furthermore, not all cases of malrotation have lower quadrant.
abnormal SMV/SMA orientation on US [47]. • Broadening of the base of the small bowel
Because of the lower sensitivity and specificity of mesentery by severing its serosal leaves as to
US compared with UGI, and because of the fact create the longest distance between the duode-
that US cannot estimate the length of the mesen- nojejunal junction and the ileocecal one.
teric base (which determines the risk of midgut • Placement of the bowel in a nonrotation posi-
volvulus), UGI has remained the gold standard tion in the abdomen with the duodenum and
diagnostic modality [31, 48]. upper jejunum on the right of the abdomen
Contrast enema (CE) has been used to demon- and the cecocolic loop in the left upper
strate the position of the cecum. However, CE is quadrant.
less reliable in identifying malrotation because
the position of the cecum and colon is highly The important steps are the broadening of the
variable and may even be normal [33]. Reversely mesentery which prevents recurrent volvulus and
20–30% of malrotations have a normally sited the freeing of the duodenum to relieve the gastro-
colon [42]. Today it is considered at suppress intestinal symptoms these patients have (emesis,
used in low diagnosis value used and is rarely reflux, failure to thrive). Turbid fluid at surgery is
used. almost always due to chylous ascites related to
Both computed tomography (CT) and mag- lymphatic congestion from partial volvulus and
netic resonance imaging (MRI) can be used to does not evidence a bowel perforation.
380 L. Ferrero et al.

resection has to be performed. It is wiser to wait

before resecting as reperfusion is frequently
observed after detorsion, warming, and observa-
tion. If viability is in question, it is better to plan
a second look procedure within 1 or 2 days than
to resect in haste.
In the absence of acute midgut volvulus,
patients with symptomatic malrotation can be
scheduled for an elective Ladd’s procedure.
The Ladd’s procedure can be performed open
or laparoscopically, but the steps remain the same
regardless of the approach.
If preferred, laparotomy is performed via an
upper abdominal, transverse, muscle-cutting
incision, extending mainly to the right side.
Exteriorization of the entire bowel is necessary,
avoiding traction to the mesentery, in order to
understand the anatomy. The volvulus is usually
untwisted by a counterclockwise rotation until
the transverse colon and cecum are brought ante-
riorly to the superior mesenteric pedicle. The
bowel is wrapped with warm moist pads until its
perfusion is returned to normal.
The use of minimally invasive surgical tech-
niques in the diagnosis and treatment of malro-
tation has been described in the literature since
Fig. 27.11  Division of obstructive Ladd’s bands the beginning of the 1990s [50–52]. Bax
reported nine cases of neonates being operated
laparoscopically between 1994 and 1997 for
Performing an appendectomy is debatable. A volvulus [50].
few decades ago, it was the rule. The argument For the laparoscopic procedure, the child is
put forward was that the appendix could be in an placed in a supine, anti-Trendelenburg, “frog”
unusual position, thus making the diagnosis of a position. The surgeon stands at the bottom end of
future appendicitis more difficult or even unrec- the table with the camera assistant to the left and
ognized. Today pediatric surgeons are more the scrub nurse to the right. The principal monitor
respectful of the appendix and avoid removing it is placed over the child’s head to face the surgeon
unnecessarily specially since the era of laparos- if possible or to the right of the patient’s head.
copy that makes its search easier. Much has been Three trocars are used: the first trocar is inserted
written about a pexy of the cecum to prevent in an open fashion through the inferior umbilical
future volvulus. There is no evidence that ceco- fold (Hasson’s technique) and will contain a ø
pexy improves outcomes or prevents from recur- 5 mm × 30° telescope, and two working trocars
rences [49]. Reversely it creates sites around are positioned, one pararectally on the right at
which a volvulus could occur. umbilical level and one in the left hypochon-
Patients with an acute abdomen require appro- drium. An extra cannula can be inserted subcos-
priate resuscitation and prompt operative explo- tally on the left to be used for retraction. Ladd’s
ration. If a segment of volvulized bowel is bands are divided, and the mesentery is widened
identified as ischemic, resection is not manda- laparoscopically using the same principles as
tory. Only in case of certainly necrotic segment, with the open technique [50, 53–56].
27  Intestinal Malrotation and Volvulus 381

They are major challenges in the laparoscopic lives. So the question raised is, should we perform
procedure for malrotation. In case of occlusion, preventive surgery? In 1993, Schey et  al. retro-
the bowel distension in an already limited field spectively reviewed 53 cases of pediatric and
reduces the surgeon’s vision as also does a adult malrotations and categorized them into 5
chylous ascites or inflammatory mesentery
­ distinct patterns based on relative positions of the
resulting from bowel suffering. At the end of the duodenojejunal junction and the cecum. They
procedure, the small operating field in a neonate suggested that configurations involving an abnor-
makes the assessment of the proper position of mal position of the duodenojejunal junction were
the bowel difficult. The use of laparoscopy is safe at highest risk for acute midgut volvulus and
and effective, and the number of reports in litera- should be surgically corrected, even if asymptom-
ture increases significantly. However a high rate atic. Configurations involving malrotation of the
of conversion is noted ranging from 12 to 33% cecum bear also a risk for volvulus but with less
[14, 54, 56, 57]. catastrophic consequences due to the smaller vas-
The debate between open and laparoscopic cular distribution involved. According to Schey,
approaches on Ladd’s procedure is still open. The these patients should not be operated unless
comparative studies between open and laparo- symptomatic [58]. In 2002, Mehall et  al. retro-
scopic approaches are limited by the small num- spectively reviewed 201 cases of pediatric malro-
ber of cases and subsequently by the lack of tation. They classified them into three groups
prospective randomized design [14, 53, 55]. In a based on the location of the duodenojejunal junc-
series comparing 2 similar groups of 20 neonates, tion. The junction was described as “typical” if it
each suffering malrotations and being operated was located right to the midline, “low” if it was
either open or by laparoscopy demonstrated that located left to the midline and below the vertebra
the laparoscopic group recovered full diet shortly T12, and “high” if it was located left to the mid-
and left the hospital earlier. Rehospitalization due line and above T12. Operative findings of volvu-
to recurrence of occlusive symptoms occurred in lus were more common in the “typical” cases as
30% of patients in the laparoscopic group versus compared with the other groups, namely, “low”
40% in the open group [14]. Additionally, what is and “high” cases. Operative complications and
believed to be an advantage of laparoscopy (less persistent symptoms after surgery occurred more
postoperative adhesions) could not be either one frequently in the “low” and “high” cases than in
if the bowel does not stay in the nonrotation posi- the “typical” cases. Given the lower risk of volvu-
tion at the end of the Ladd’s procedure. If malro- lus, higher operative morbidity, and lower success
tation cannot be excluded from imaging, rate, they concluded that consideration should be
laparoscopy is an ideal tool to look at the position given to nonoperative management of asymptom-
of the bowel and the appearance and the width of atic patients with duodenojejunal junctions classi-
the mesentery [56]. fied as “low” or “high” [59].
Malrotation that is discovered at the time of
operation raises an interesting dilemma with
respect to consent. Should the malrotation not be 27.10 Postoperative Course
involved in the disease process, its treatment
would be considered an additional procedure The postoperative course depends upon the indi-
except if consent can be given by the parents dur- cations for surgery and the intraoperative find-
ing the course of operation. ings. Dilatation of the duodenum and vascular
Another dilemma is the asymptomatic malro- compromise of the bowel might cause prolonged
tation discovered fortuitously. Asymptomatic ileus. It should be managed with expectant pol-
midgut volvulus bears a risk of sudden dramatic icy, maintaining gastric decompression through a
event with vascular compromise. No mean can nasogastric aspiration and IV fluids. Patient hav-
predict it. On the other hand, there are adult ing an extensive bowel injury with or without
patients who remain asymptomatic for their entire resection should benefit of a TPN.
382 L. Ferrero et al.

During the postoperative course of malrota- 16. Beaudoin S, Mathiot-Gavarin A, Gouizi G, et  al.

Familial malrotation: report of three affected siblings.
tions, intussusception may occur. The incidence is Pediatr Surg Int. 2005;21:856–7.
higher (3.1%) than for other laparotomies (0.05%) 17. Smith VL, Long F, Nwomeh BC. Monozygotic twins
[60]. The incidence of postoperative adhesive ileus with discordant intestinal rotation. Pediatr Radiol.
is 4%. The incidence of recurrent volvulus ranges 2006;26:1–3.
18. Touloukian RJ, Smith EI.  Disorders of rotation and
from 0.5 to 1.3% according to literature [23]. fixation. In: O’Neill JA, Rowe MI, Grosfeld JL, et al.,
editors. Pediatric surgery. 5th ed. St Louis: Mosby;
1998. p. 1199–214.
References 19. Kapfer SA, Joseph F, Rappold JF. Intestinal malrota-
tion – not just the pediatric surgeon’s problem. J Am
Coll Surg. 2004;199(4):628–35.
1. Ladd WE. Congenital obstruction of the duodenum in
20. Kluth D, Jaeschke-Melli S, Fiegel H. The embryology
children. N Engl J Med. 1932;206:277–83.
of gut rotation. Semin Pediatr Surg. 2003;12(4):275–9.
2. Kiesewetter WB, Smith JW.  Malrotation of
21. Strouse PJ. Disorders of intestinal rotation and fixation
midgut in infancy and childhood. Arch Surg.
(“malrotation”). Pediatr Radiol. 2004;34(11):837–51.
1953;77:483–91.
22. Jamieson D, Stringer D.  Small bowel. In: Babyn

3. Meckel JF.  Bildungsgeschichte des Darmkanals der
PS, editor. Pediatric gastrointestinal imaging and
Säugethiere und namentlich des Menschen. Dtsch
intervention. 2nd ed. Hamilton, ON: Decker; 2000.
Arch Physiol. 1817;3:1–84.
p. 311–32.
4. Mall FP.  Development of the human intestine and
23. Mentessidou A, Saxena A. Disorders of intestinal rota-
its position in the adult. Bull Johns Hopkins Hosp.
tion and fixation. In: Lima M, editor. Pediatric diges-
1898;9:197–208.
tive surgery. Switzerland: Springer; 2017. p. 245–54.
5. Dott NM.  Anomalies of intestinal rotation: their
24. Willwerth BM, Zollinger RM, Izant RJ.  Congenital
embryology and surgical aspects with report of five
mesocolic (paraduodenal) hernia. Embryologic basis
cases. Br J Surg. 1923;11:251–86.
of repair. Am J Surg. 1974;128(3):358–61.
6. Snyder WH, Chaffin L. Embryology and pathology of
25. Applegate KE, Anderson JM, Klatte EC.  Intestinal
the intestinal tract. Ann Surg. 1954;140:368.
malrotation in children: a problem-solving approach
7. Grob M. Uber Lageanomalien des Magendarmtracktes
to the upper gastrointestinal series. Radiographics.
infolge Störungen der fetalen Darmdrehung. Basel:
2006;26(5):1485–500.
Schwabe; 1953.
26. Millar AJW, Rode H, Cywes S. Malrotation and vol-
8. Grob M, Stockmann M, Bettex M.  Lehrbuch der
vulus in infancy and childhood. Semin Pediatr Surg.
Kinderchirurgie. Stuttgart: Georg Thieme; 1957.
2003;12:229–36.
9. Clark LA, Oldham KT. Malrotation. In: Ashcraft KW,
27. Taybi H, Lachman R.  Radiology of syndromes,

Murphy JP, Sharp RJ, et  al., editors. Pediatric sur-
metabolic disorders, and skeletal dysplasias. 3rd ed.
gery. 3rd ed. Philadelphia: Saunders Company; 2000.
Chicago, IL: Yearbook Medical Publishers; 1990.
p. 425–42.
p. 825–6.
10. Warner BW. Malrotation. In: Oldham KT, Colombani
28. Yoo SJ, Park KW, Cho SY, et  al. Definitive diagno-
PM, Foglia RP, editors. Surgery of infants and chil-
sis of intestinal volvulus in utero. Ultrasound Obstet
dren: scientific principles and practice. Philadelphia:
Gynecol. 1999;13(3):200–3.
Lippincott-Raven; 1997. p. 1229–40.
29. Chung JH, Lim GY, We JS. Fetal primary small bowel
11. Donnellan WL, Kimura K. Malrotation, internal her-
volvulus in a child without intestinal malrotation. J
nias, congenital bands. In: Donnellan WL, Burrington
Pediatr Surg. 2013;48(7):e1–5.
JD, Kimura K, editors. Abdominal surgery of infancy
30. Lesieur E, Lecompte JF, Gorincour G, et al. Prenatal
and childhood. New  York: Harwood Academic
diagnosis of complete nonrotation of fetal bowel with
Publishers; 1996. p. 1–27.
ultrasound and magnetic resonance imaging. Diagn
12. Durkin ET, Lund DP, Shaaban AF, et al. Age-related
Interv Imaging. 2016;97(6):687–9.
differences in diagnosis and morbidity of intestinal
31. Orzech N, Navarro OM, Langer JC. Is ultrasonogra-
malrotation. J Am Coll Surg. 2008;206:658–63.
phy a good screening test for intestinal malrotation? J
13. Pierro A, Ong EG. Malrotation. In: Puri P, Hollwart
Pediatr Surg. 2006;41:1005–9.
ME, editors. Pediatric surgery. New  York, Berlin,
32. Ford EG, Senac MO Jr, Srikanth MS, et  al.

Heidelberg: Springer-Verge; 2004. p. 197–201.
Malrotation of the intestine in children. Ann Surg.
14. Ferrero L, Ben Ahmed Y, Philippe P, et al. Intestinal
1992;215:172–8.
malrotation and volvulus in neonates: laparoscopy
33. Pickhardt PJ, Bhalla S. Intestinal malrotation in ado-
versus open laparotomy. J Laparoendosc Adv Surg
lescents and adults: spectrum of clinical and imaging
Tech. 2017;27(3):318–21.
features. Am J Roentgenol. 2002;179(6):1429–35.
15. Aiken JJ, Oldham KT. Malrotation. In: Ashcraft KW,
34. Berdon WE, Baker DH, Bull S, et al. Midgut malro-
Holcomb GW, Murphy JP, editors. Pediatric surgery.
tation and volvulus. Which films are most helpful?
New York: Elsevier Saunders; 2005. p. 435–77.
Radiology. 1970;96(2):375–84.
27  Intestinal Malrotation and Volvulus 383

35. Long FR, Kramer SS, Markowitz RI, et al. Intestinal formed even if the bowel shows signs of ischemia.
malrotation in children: tutorial on radiographic diag- J Laparoendosc Adv Surg Tech A. 2009;19(Suppl
nosis in difficult cases. Radiology. 1996;198:775–80. 1):S167.
36. Katz ME, Siegel MJ, Shackelford GD, et al. The posi- 49. Firor HV, Steiger E.  Morbidity of rotational abnor-
tion and mobility of the duodenum in children. Am J malities of the gut beyond infancy. Cleve Clin Q.
Roentgenol. 1987;148:947–51. 1983;50:303–9.
37. Torres AM, Ziegler MM. Malrotation of the intestine. 50. Bax NM, van der Zee DC.  Laparoscopic treatment
World J Surg. 1993;17:326–31. of intestinal malrotation in children. Surg Endosc.
38. Gaines PA, Saunders AJ, Drake D. Midgut malrotation 1998;12:1314–6.
diagnosed by ultrasound. Clin Radiol. 1987;38(1):51– 51. Frantzides CT, Cziperle DJ, Soergel K, et  al.

3. Erratum in: Clin Radiol 1987;38(5):558 Laparoscopic Ladd procedure and cecopexy in the
39. Loyer E, Eggli KD. Sonographic evaluation of supe- treatment of malrotation beyond the neonatal period.
rior mesenteric vascular relationship in malrotation. Surg Laparosc Endosc. 1996;6:73–5.
Pediatr Radiol. 1989;19(3):173–5. 52. Mazziotti MV, Strasberg SM, Langer JC.  Intestinal
40. Pracros JP, Sann L, Genin G, et al. Ultrasound diagno- rotation abnormalities abnormalities without vol-
sis of midgut volvulus: the “whirlpool” sign. Pediatr vulus: the role of laparoscopy. J Am Coll Surg.
Radiol. 1992;22(1):18–20. 1997;185:172–6.
41. Veyrac C, Baud C, Prodhomme O, et al. US assess- 53. Bass KD, Rothenberg SS, Chang JH. Laparoscopic
ment of neonatal bowel (necrotizing enterocolitis Ladd’s procedure in infants with malrotation. J
excluded). Pediatr Radiol. 2012;42(Suppl 1):S107–14. Pediatr Surg. 1998;33(2):279–81.
42. Tackett JJ, Muise ED, Cowles RA.  Malrotation:
54. Fraser JD, Aguayo P, Sharp SW, et  al. The role of
current strategies navigating the radiologic diag- laparoscopy in the management of malrotation. J
nosis of a surgical emergency. World J Radiol. Surg Res. 2009;156(1):80–2.
2014;6(9):730–6. 55. Ooms N, Matthyssens LE, Draaisma JM, et  al.

43. Chao HC, Kong MS, Chen JY, et al. Sonographic fea- Laparoscopic treatment of intestinal malrotation in
tures related to volvulus in neonatal intestinal malro- children. Eur J Pediatr Surg. 2016;26:376–81.
tation. J Ultrasound Med. 2000;19(6):371–6. 56. Bax NM, van der Zee DC.  Intestinal malrota-

44. Kalfa N, Zamfir C, Lopez M, et  al. Conditions
tion. In: Bax NM, Georgeson KE, Rothenberg
required for laparoscopic repair of subacute volvulus SS, et  al., editors. Endoscopic surgery in infants
of the midgut in neonates with intestinal malrotation: and children. Berlin, Heidelberg: Springer; 2008.
5 cases. Surg Endosc. 2004;18:1815–7. p. 299–304.
45. Weinberger E, Winters WD, Liddell RM, et  al.
57. Hagendoorn J, Vieira-Travassos D, van der Zee D.
Sonographic diagnosis of intestinal malrotation in Laparoscopic treatment of intestinal malrotation
infants: importance of the relative positions of the in neonates and infants: retrospective study. Surg
superior mesenteric vein and artery. Am J Roentgenol. Endosc. 2011;25:217–20.
1992;159:825–8. 58. Schey WL, Donaldson JS, Sty JR.  Malrotation of
46. Millar AJW, Rode H, Brown RA, et  al. The deadly bowel: variable patterns with different surgical con-
vomit: malrotation and midgut volvulus. Pediatr Surg siderations. J Pediatr Surg. 1993;28:96–101.
Int. 1987;2:172–6. 59.
Mehall JR, Chandler JC, Mehall RL, et  al.
47.
Nasir AA, Abdur-Rahman LO, Adeniran JO. Management of typical and atypical intestinal malro-
Outcomes of malrotation in children. Afr J Paediatr tation. J Pediatr Surg. 2002;37:1169–72.
Surg. 2011;8:8–11. 60. Kidd J, Jackson R, Wagner CW, et al. Intussusception
48. Adikibi BT, Strachan CL, Mackinlay GA.  Neonatal following Ladd’s procedure. Arch Surg. 2000;135(6):
laparoscopic Ladd’s procedure can safely be per- 713–5.