Gastro: Intestinal Malrotation Flashcards
What is Intestinal Malrotation?
Can some live with it unoticed?
is a congenital anatomical anomaly which results from an abnormal rotation of the gut as it returns to the abdominal cavity during embryogenesis.
Some individuals live their entire life with malrotated bowel without symptoms, the abnormality does predispose to midgut volvulus and internal hernias, with the potential for life threatening complications.
How does Malrotation happen (Embriogenesis) and its full pathology?
As fetus develop, as the straight tube of the intestine starts to move as it elongates into the umbilical cord, which supplies nutrients. Near end of of 1st trimester, they move from umbilical cord and return to abdomen, if it doesn’t turn 270 degree after moving to the abdomen forming a duodeno-jejunal flexure normally located left to the midline at the level of L1 vertebral body and the terminal ileum located in the right iliac fossa. This results in a broad mesentery running obliquely down from the DJ flexure to the ceecum, and prevents rotation around the superior mesenteric artery (SMA) .Malrotation occurs. 1 out of 6000 live births. Some are born with additional problems in GI, heart, and spleen.
What ends up happening?
Small intestine ends up on right side of abdomen. The cecum is displaced into the epigastrium and not its regular position in the right lower quadrant. The ligament of Treitz Is displaced inferiorly and rightward.
fibrous bands (of Ladd) course over the vertical portion 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).
Some may never have a problem.
What Problems Can It Cause?
In a condition called volvulus, since there was malrotation, the mesentary has a short root. Now the bowel twists on itself, cutting off the blood flow to the tissue and causing the tissue to die. Symptoms of volvulus,
including pain and cramping, are often what lead to the diagnosis of malrotation.
Bands of tissue called Ladd’s bands may form, obstructing the first part of the small intestine (the duodenum).
Obstruction caused by volvulus or Ladd’s bands is a potentially life threatening problem. The bowel can stop working and intestinal tissue can die from lack of blood supply if any obstruction isn’t recognized and treated.
Volvulus, especially, is a medical emergency, with the entire small intestine in jeopardy.
What is Intestinal “Nonrotation”?
•nonrotation is a subtype of malrotation in which the small bowel is mainly located in the right hemiabdomen and the caecum in the left hemiabdomen.
What Are the Signs of Intestinal Malrotation?
The clinical presentation of malrotation often correlates to the age of presentation.
Which is most prevalent in children, in adults?
An intestinal blockage can prevent the proper passage of food. Some of the earliest signs of malrotation and volvulus is abdominal pain and cramping, which happen when the bowel can’t push food past the blockage.
•In the infant the most common presentation is with a midgut volvulus. For adults, episodes of spontaneously resolving duodenal obstruction. This is thought to be due to kinking of the duodenum by Ladd bands rather than a volvulus.
Signs of Intestinal Malrotation 2
A baby with cramping might:
Pull up the legs and cry stop crying suddenly. Behave normally for 15 to 30 minutes repeat this behavior when the next cramp happens.
Infants also may be fussy, lethargic, or have trouble pooping.
Signs of Intestinal Malrotation 3
Vomiting is another symptom of malrotation, and it can help the doctor determine where the obstruction is. Vomiting that happens soon after the baby starts to cry often means the blockage is in the small intestine; delayed vomiting usually means it’s in the large intestine. The vomit may contain bile (which is yellow or green) or may resemble feces.
Other symptoms of malrotation and volvulus can include:
a swollen abdomen that’s tender to the touch
diarrhea and/or bloody poop (or sometimes no poop at all)
fussiness or crying in pain, with nothing seeming to help
rapid heart rate and breathing
little or no pee because of fluid loss
fever
How Is a Blockage Diagnosed?
With acutely ill patients, consider emergency surgery laparotomy if there is a high index of suspicion.
Upper gastrointestinal series is the modality of choice for the evaluation of malrotation as it will show an abnormal position of the duodeno-jejunal flexure (ligament of Treitz).
In cases of malrotation complicated with volvulus, it demonstrates a corkscrew appearance of the distal duodenum and jejunum. In cases of obstructing Ladd bands, it will reveal a duodenal obstruction.
In equivocal cases, contrast enema, may be helpful by showing the cecum at an abnormal location.
It is usually discovered near birth, but in some cases is not discovered until adulthood.[2] In adults, the “whirlpool sign” of the superior mesenteric artery can be useful in identifying malrotation.[3]
How Is a Blockage Diagnosed by Radiography?
What does ultrasound show?
What about CT?
Plain radiography may demonstrate signs of duodenal obstruction with dilatation of the proximal duodenum (right side jejunal markings) and stomach but it is often non-specific. May be abscence of stool filled colon in right lower quadrant.
•May show and inversion in the SMA/SMV relationship with the SMA on the right and the SMV on the left. Large bowel predominantly on left and small bowel predominantly on the right
Contrast Enema for diagnosis?
has historically also been used, the theory being that in malrotation the large bowel will also be malrotated. Unfortunately …….
in ~25% (range 20-30%) of cases with malrotation the caecum is normally located. The converse is also true, with position of the caecum in normal individuals being variable . Very rarely, the caecum may be malrotated and the small bowel in a normal position. So its not a good indicator.
How Is Intestinal Malrotation Treated? 1
Treating significant malrotation almost always requires surgery. The timing and urgency will depend on the child’s condition. If there is already a volvulus, surgery must be done right away to prevent damage to the bowel.
Any child with bowel obstruction will need to be hospitalized. A tube called a
nasogastric (NG) tube is usually inserted through the nose and down into the stomach to remove the contents of the stomach and upper intestines. This keeps fluid and gas from building up in the abdomen. The child may also be given intravenous (IV) fluids to help prevent dehydration and antibiotics to prevent infection.
How Is Intestinal Malrotation Treated? 2
During the surgery, which is called a Ladd procedure, the intestine is straightened out, the Ladd’s bands are divided, a widening of the mesenteric base. the small intestine is folded into the right side of the abdomen, and the colon is placed on the left side.
Because the appendix is usually found on the left side of the abdomen when there is malrotation (normally, the appendix is found on the right), it is removed. Otherwise, should the child ever develop appendicitis, it could complicate diagnosis and treatment.
How Is Intestinal Malrotation Treated? 3
If it appears that blood may still not be flowing properly to the intestines, the doctor may do a second surgery within _48 hour_s of the first. If the bowel still looks unhealthy at this time, the damaged portion might be removed.
If the child is seriously ill at the time of surgery, an ileostomy or colostomy usually will be done. In this procedure, the diseased bowel is completely removed, and the end of the normal, healthy intestine is brought out through an opening on the skin of the abdomen (called astoma). Fecal matter (poop) passes through this opening and into a bag that is taped or attached with adhesive to the child’s belly.
How Is Intestinal Malrotation Treated? 4
In young children, depending on how much bowel was removed, the ileostomy or colostomy is often a temporary condition that can later be reversed with another operation.
Most of these surgeries are successful, although some kids have recurring problems after surgery. Recurrent volvulus is rare, but a second bowel obstruction due to adhesions (scar tissue build-up afterany type of abdominal surgery) could happen later.
Children who had a large portion of the small intestine removed can have too little bowel to maintain adequate nutrition (a condition known as short bowel syndrome). They might need intravenous (IV) nutrition for a time after surgery (or even permanently if too little intestine remains) and may require a special diet afterward.
