INTESTINAL MALROTATION

Intestinal malrotation

Incidence :

•       1 in 500 live births.

•       Approximately 50%of patient with duodenal atresiaa and 33% patient with jejunoileal atresia have maalrotation.

•        Younger patients have higher rates of morbidity and mortality.

•       Infants range from 2 to 24%. Male to female ratio is 2:1

•       No sex discrimination seen in patients with older than one year.

•       40% of malrotattion cases develops during 1^st week of life.

•       50% of cases diagnosed during 1^st month of life, 75% in the 1^st year of life. Remaing 25% will be detected during 1 to 18 years  of life incase of other surgical procedures or at atopsy.

•       50% of cases diagnosed during 1^st month of life, 75% in the 1^st year of life. Remaing 25% will be detected during 1 to 18 years  of life incase of other surgical procedures or at atopsy.

 A report of 25 years' experience demonstrated congenital cardiovascular disease in 27.1% of patients with intestinal malrotation; those patients had a morbidity rate of 61.1% after intestinal malrotation surgery.²

Causes:

•       The exact causes is unknown.

•       It is not associated with a particular gene, but  there is some evidence of recurring in families.

Clinical features.

•       Some time it is asymptomatic

•       Vomitus,

•       Crampy abdominal pain.

•       Abdominal distention.

•       Passage of blood and mucus in the stool

Result of malrotation:

•       the small bowel is found predominantly on the right side of the abdomen

•       the cecum is displaced (from its usual position in the right lower quadrant) into the epigastrium - right hypochondrium

•       the ligament of Treitz is displaced inferiorly and rightward

•       fibrous bands (of Ladd) course over the horizontal part of the duodenum (DII), causing intestinal obstruction.

•       the small intestine has an unusually narrow base, and therefore the midgut is prone to volvulus (a twisting that can obstruct the mesenteric blood vessels and cause intestinal ischemia).

Associated conditions

•       This can lead to a number of disease manifestations such as:

•       Acute midgut volvulus

•       Chronic midgut volvulus

•       Acute duodenal obstruction

•       Chronic duodenal obstruction

•       Internal herniation

Diagnostic evaluation:

History

•       The history of present illness varies in patients with intestinal malrotation according to acute or chronic presentation, as well as according to type of rotational defect.

Acute midgut volvulus

–      Most patients present in the first year of life.

–      The primary presenting sign of acute midgut volvulus is sudden onset of bilious emesis.

Chronic midgut volvulus

–      Chronic midgut volvulus is due to intermittent or partial twisting that results in lymphatic and venous obstruction.

–      Multiple case reports show that 2 of the main presenting features are recurrent abdominal pain and malabsorption syndrome.

–      Several patients presented with acute midgut volvulus, but further history revealed they had had chronic symptoms with misdiagnoses.

–      Other clinical features include recurrent bouts of diarrhea alternating with constipation, intolerance of solid food, obstructive jaundice (1 case),³ and gastroesophageal reflux.

Acute duodenal obstruction

–      This anomaly is usually recognized in infants and is due to compression or kinking of the duodenum by peritoneal bands (Ladd bands).

–      Patients present with forceful vomiting, which may or may not be bile-stained, depending on location of the obstruction with respect to the entrance of the common bile duct (ampulla of Vater).

Chronic duodenal obstruction

–      The typical age at diagnosis ranges from infancy to preschool-age.

–      The most common symptom is vomiting, which is usually bilious.

–      Patients may also have failure to thrive and intermittent abdominal pain (frequently diagnosed as colic).

Internal herniation

–      Internal herniation usually has a chronic picture.

–      Patients have recurrent abdominal pain, which may progress from intermittent to constant.

–      They experience vomiting as well as constipation at times.

–      They are often diagnosed with psychosocial problems.

Physical examination:

•       Physical examination findings may vary depending on the type of rotational defect. Acute and chronic presentations also differ.

•       Acute midgut volvulus

–      Abdominal distention is frequently present, and the infant appears in acute pain.

–      As vascular compromise persists, intraluminal bleeding may occur, which leads to blood per rectum and sometimes hematemesis.

–      Abdominal guarding is usually present and prevents palpation of intestinal loops.

–      Acute midgut volvulus conti.. 

–      As symptoms persist, the infant may develop signs of shock,

including poor perfusion,

decreased urine output, and hypotension.

–      Patients also have signs of peritonitis, including abdominal tenderness and discoloration of the skin.

Chronic midgut volvulus

–      Physical examination results may be completely normal if the patient presents during a period when the obstruction is relieved.

–      If partial twisting is present at the time of examination, the patient may have signs and symptoms equivalent to those of acute midgut volvulus.

–      Abdominal tenderness and guarding is usually present, as well as abdominal distention.

Acute duodenal obstruction

–      Abdominal distention and gastric waves may be present.

–      Passage of meconium or stool can be present.

–      These patients usually do not have signs of peritonitis or shock unless volvulus is also present distal to the obstruction.

Chronic duodenal obstruction

–      Physical examination results may be completely normal at the time of presentation.

–      Abdominal distention and tenderness may be present.

–      Diagnosis is usually made by history and enough suspicion to obtain radiologic studies; physical examination findings are very unreliable.

–      -Intravenous (IV) fluids to help prevent dehydration dopamine at an infusion rate of 3 mcg/kg/min intravenously (IV) and continue it postoperatively even if the patient is not hypotensive.

–      -If the patient is unstable, do not delay surgical intervention for upper GI and laboratory studies. Quick surgical intervention, not prolonged medical management, produces the best results if midgut volvulus is suspected

–      Your child may also be given and antibiotics to prevent infection.

Duodenal obstruction

–      After the volvulus is reduced or if no volvulus was present, identify any extrinsic obstruction to the duodenum.

–      If peritoneal bands crossing the duodenum are found, ligate them with careful attention to protecting the superior mesenteric vessels. The bands may also obstruct the ileum or the jejunum and sometimes run to the gallbladder and liver.

Post operative care and Consultations

•       Pediatric surgeon: The only definitive treatment for malrotation is surgical in nature.

•       Dietary/intravenous nutrition team: In hospitals where available, include a nutritionist in the medical team to monitor total parenteral nutrition to ensure optimal levels are being achieved.

•       Physical therapist

–      A physical therapist can help with range of motion exercises and strength conditioning while patients are bedridden.

–      Frequent repositioning also helps prevent decubitus pressure and head molding (in infants).

•       Occupational therapist

•       An occupational therapist also helps with range of motion exercises.

•       Occupational therapists can also assist with splinting of extremities that contain central lines to prevent contractures.

•       Speech therapist

•       A speech therapist can help with oral stimulation while patients are not being fed. This stimulation can prevent feeding aversion and dyscoordination when oral feeds are restarted.