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INTESTINAL MALROTATION
Dr.T.KIRAN KUMAR
FINAL YEAR POST GRADUATE
GENERAL SURGERY
CONTENTS
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Definition
History
Embryology
Rotational disorders
Presentation
Diagnosis
Management
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DEFINITION
Malrotation is a congenital abnormal position of the bowel
within the peritoneal cavity and usually involves both the small
and the large bowel.
HISTORY
 Intestinal development –Earliest descriptions by Mall in 1898.
 Later expanded by Frazer and Robins in 1915.
 1928- Dott translated preliminary embryologic observations
into problems encountered clinically.
 1932 ,Ladd described the evaluation and surgical treatment of
malrotation.
EMBRYOLOGY
Development of midgut begins with the
differentiation of the primitive intestinal
tract
4th week of gestation
Foregut
Hindgut
Midgut
• Most accepted model of midgut maturation involves
Herniation
Rotation
Retraction
Fixation
Disproportional growth and elongation of
midgut (4th gestational week)
Herniation into extraembryonic coelom
3 separate 90 degrees turns, all in
counter clock wise direction around
superior mesenteric artery
.
The first 90 degrees rotation outside the
abdomen
.
2nd 90 degrees during the return of the intestine into
abdominal cavity(10th gestational week)
.
Duodenojejunal junction passes posterior to superior
mesenteric artery
Last rotation in the abdomen
 Primitive intestine has thus completed a 270 degrees counter
clock wise rotation .
Duodenojejunal junction
becomes fixed in the left
upper abdomen
cecum is anchored in
the right lower
quadrant.
ROTATIONAL DISORDERS
 Clinical disorders may arise when intestinal rotation fails to
occur or is incomplete.
 Genetic mutations may predipose the host to malrotation.
 Mutations in the gene BCL6 resulting in absence of left sided
expression of its transcript lead to reversed cardiac
orientation ,defective ocular development and malrotation.
NONROTATION :
 Failure of normal intestinal 270degrees counter clock wise
rotation around Superior mesenteric artery.
Duodenojejunal
limb
 SIGNIFICANT RISKS
 Midgut volvolus
 Extrinsic duodenal obstruction
cecocolic
limb
INCOMPLETE ROTATION
 Normal rotation has been arrested at or near 180 degrees.
CECUM IN RIGHT
UPPER ABDOMEN
 Obstructing peritoneal bands will present.
REVERSE ROTATION
PRESENTATION
Presents mainly in
childhood. Incidence:
1 in 6000 live births
Small proportion of adults
- Acute or chronic symptoms
of intestinal obstruction.
- Intermittent and recurrent
abdominal pain
75% of patients- during
the first month of life
Another 15% with in 1
year.
INCIDENCE
Most adult diagnosis of Malrotation
are made in asymptomatic patients
;either on imaging investigations for
unrelated conditions or at operations
for other pathology
Incidence in adults is
approximately
0.00001% to 0.19%.
 Incidental diagnosis is becoming increasingly common.
 The true diagnosis is fraught with immense difficulty because
- Presents with non-specific symptoms .
- Adult Surgeons usually have low index of suspicion.
Associated anomalies
Associated anomalies
In percentage
Intestinal atresia
5-26
Imperforate anus
0-9
Cardiac anamolies
7-13
Duodenal web
1-2
Meckels diverticulum
1-4
Hernia
0-7
Trisomy 21
3-10
PRESENTATION IN ADULTS:
 Presents in numerous ways and the symptoms are nonspecific.
 The clinical diagnosis in adolescents and adults is difficult
because it is rarely considered on clinical grounds.
 Many patients remain asymptomatic and the diagnosis is
discovered incidentally during investigations or laparotomy for
other unrelated problems in adult life.
 Adults with a rotational abnormality of the gut usually present
differently to paediatric patients.
 Two distinct patterns of adult presentations have been
reported in the literature:
Acute
Chronic
 Chronic presentation is more common in adults.
 This is characterised by intermittent crampy abdominal pain,
bloating, nausea and vomiting over several months or years.
 The symptoms may be highly nonspecific.
 However, the range of clinical presentations, underlines the
need for a high index of suspicion of midgut malrotation,
when investigating the cause of intermittent and varying
abdominal symptomatology in a healthy adult .
 Diagnostic delays are common in this group of patients
because of the nonspecific nature of the presentations.
 The pathophysiology of these chronic symptoms
- o pressio effe t of Ladd s a ds.
 The other group of symptomatic adults typically present
with symptoms of acute bowel obstruction.
 May or may not report a previous history of abdominal
symptoms.
 occasionally have symptoms and signs of an impending
abdominal catastrophe.
 Acute presentation may be due to
Volvulus of the midgut or ileocaecum
-.
Internal herniation aused y Ladd s a ds
-.
Identified when affected by other common abdominal
diseases
Clinical features of malrotation in neonates:
• Bilious vomiting
Cardinal •
sign
•
•
Other •
signs
Late
malrotation must be the presumed diagnosis until proven otherwise
Abdominal pain ,
Distension,
Hypovolemic shock.
• Hematemesis,
• Melena from progressive mucosal ischemia
signs
Finally
• Mesenteric vascular compromise rapidly leads to peritonitis , sepsis
shock and death
DIAGNOSIS
Ultrasound
scan USS
Plain
abdominal
radigraphy
Computed
tomography
scan CT
IDENTIFIED USING
Mesentric
arteriography
Magnetic
resonance
imaging scan
MRI
CONVENTIONAL
PLAIN
RADIOGRAPHY
• Neither sensitive nor specific .
• Right-sided jejunal markings and the absence of a
stool-filled colon in the right lower quadrant may
be suggestive, leading to further investigation.
ABDOMINAL
COLOUR
DOPPLER USS
• Reveal malposition of the SMA, raising the suspicion
of gut malrotation with or without the abnormal
location of the hollow viscus .
• Reported gold standard for diagnosis of gut
UPPER
GASTROINTESTINAL
CONTRAST STUDY
malrotation particularly in the paediatric
age group
• Shows duodenum and dudenojujenal
flexure located to the right of spine
• Shows abnormally located ileocaecum and right colon
CONTRAST
ENEMA WITH
UGI STUDY
• Findings may be non specific
• Normal study does not exclude the possibility of gut
malrotation
MALROTATION
• Previously used but now rarely indicated
MESENTRIC
ANGIOGRAPHY
• Shows the abnormal relationship between and
detect the patency of mesentric vasculature
• Increasingly used
COMPUTED
TOMOGRAPHY
• Now considered the investigation of choice
• Accuracy is 80%
 CT and MRI scans show
- SMV to be in an anomalous position;
posterior and to the left of the SMA.
- The abnormal anatomical
arrangements of the midgut with the
duodenum not crossing the spine.
-
hirlpool appeara e (first
described by Fisher)
- Internal herniation secondary to
Ladd s a ds.
 The role of mesenteric angiogrphy has been superseded by the
CT scan which has the overall advantage in detecting the
-Abnormal location of the midgut .
- Reversed mesenteric anatomical
relationship .
- Other intra-abdominal anomalies
associated with malrotation .
MANAGEMENT
 Symptomatic midgut malrotation - requires surgical
intervention.
 Management of asymptomatic patients is more controversial.
 Choi et al reviewed 177 patients over a 35-year period.
- Found that asymptomatic patients had a low risk of
intestinal volvulus.
- Elective surgery is not necessary.
- Requires close follow-up.
 However, it is increasingly argued that all suitable patients
with intestinal malrotation should undergo surgical correction
regardless of age .
 Several case series have recommended that
- It is impossible to predict which patients will develop
catastrophic complications
- So ele ti e Ladd s pro edure should e perfor ed i all
patients with intestinal malrotation.
- The potential risks of the procedure need to be weighed
against the benefits.
The surgical management of intestinal
malrotation was first described by
William Ladd in 1936 and this remains
the mainstay of treatment.
 Six key elements in operative correction of malrotation
1. Entry into abdominal cavity and evisceration(open)
2. Counterclock wise detorsion of the bowel
3. Division of ladd s bands
4. Broadening of small intestine mesentry
5. Incidental appendicectomy
6. Placement of small bowel along the right lateral gutter and
colon along the left lateral gutter.
 The origi al Ladd s pro edure as des ri ed for the
paediatric population group and the full components of this
procedure may not be offered in the adult group.
Complications after surgery
Recurrent
volvolus is low
Post operative intussception has
been noted in 3.1% of all patients
who underwent ladd procedure
10% of patients may
develop an adhesive
small bowel
obstruction requiring
laporotomy after the
procedure
Laparoscopic procedure
 There are recent reports of the use of the laparoscopic
approach in the surgical treatment of intestinal malrotation.
 The technique appears to be safe and effective when
performed by experienced laparoscopic surgeons, especially
in the absence of volvulus .
 First proposed by van der zee s i 1995.
 Four port technique provides adequate visualisation
 First goal is to determine if malrotation is actually present and
thus confirm preoperative imaging.
 Detorsion.
 Expeditious dissection may progress by identifying 2nd portion
of duodenum and excising restricting peritoneal bands.
 Next, reduction of volvolus if present , mobilisation of
duodenum , jejunum and incision of anterior mesenteric
leaflet.
 Lastly appendicectomy.
Comparision of laporoscopic and open procedures
In a review of patients undergoing open and laparoscopic
ladd procedure ,comparative results were favorable for the
laparoscopic group for
 Resumption of oral intake (1.8 days vs 2.7 days)
 Shorter hospitalisation (4 days vs 6.1 days)
 Requirements for iv narcotics on postoperative day .
 Operative time (194 min vs 143 min)
 Conversion to an open operation occurred in 25%.