A study carried out by the Cleveland Clinic describes a new surgical procedure that successfully treated patients with congenital intestinal malrotation. The study, published in Annals of Surgery, also defines the presentation of the disease in children and adults, identifies patients at risk for bowel loss, and assesses long-term outcomes after different surgical interventions.
Also called intestinal malrotation, congenital intestinal malrotation is an anomaly resulting from partial or complete failure of rotation and correction of the primitive intestine during embryonic development. In the United States, for every 500 births, at least one will experience malrotation.
The anomaly manifests itself in the neonatal period and 90% of diagnoses are made up to the first year of life. In some patients, malrotation is discovered incidentally as part of the assessment of another problem.
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What happens is that, during pregnancy, the baby's intestines normally rotate 270 degrees counterclockwise and then lock into position. With intestinal malrotation, however, this embryonic process is defective and is sometimes associated with poor development of the intestine, abdominal wall, and the nerve supply of the intestine.
As a result, babies, children, and adults with the disorder can suffer from a variety of gastrointestinal symptoms, ranging from abdominal pain, nausea and bloating, to food intolerance and altered bowel habits.
The twisted intestines - known as volvulus - often result in the loss of most of the small intestine and half of the colon, with the need for long-term intravenous nutrition. Some patients may ultimately need a life-saving bowel or multi-visceral transplant.
Based on his 30 years of experience in digestive surgery and transplantation, Kareem Abu-Elmagd, M.D, Ph.D. - director of the Cleveland Clinic's Center for Rehabilitation and Intestinal Transplantation and lead author of the study - has developed a novel intestinal malrotation correction surgery to alleviate gastrointestinal symptoms as well as prevent the need for intestinal transplantation.
"During the procedure, the digestive organs are reorganized and fixed in their proper anatomical locations, which prevents the intestines from twisting and resolves digestive symptoms with improved quality of life," said Dr. Abu-Elmagd.
The operation that has traditionally been used to treat intestinal malrotation is called the Ladd procedure, which distorts the intestines and relieves intestinal obstruction by dividing adhesive bands between the duodenum and colon. However, despite the relief of obstruction with some temporary improvement in the patient's symptoms, Ladd's procedure does not prevent the bowel from suffering recurrent future sprains, which can cut off the blood supply and be fatal at any age.
The study was carried out with 500 patients. Of this group, 41% were referred after losing bowel due to volvulus and the remaining 59% had an intact bowel with a wide variety of digestive symptoms. Bowel transplantation was necessary for patients with massive bowel loss. The new operation (called the "Kareem procedure") was performed on patients with intact bowel and disabling symptoms.
The study also revealed that patients at higher risk of losing their bowel had a history of congenital anomalies, including the gastrointestinal tract and abdominal wall, and were men - characteristics that had not been previously reported in the medical literature. In addition, 21% of patients who had a previous Ladd procedure developed another volvulus.
Also, it was concluded that bowel transplantation is effective in saving the lives of pediatric patients who have suffered bowel loss.
The survival of these children reached 63% at 10 years and 54% at 20 years. Babies had the best survival with up to 64% at 20 years. Most survivors had a better quality of life. (Most of the patients who received intestinal and multi-visceral transplants as part of the study were children, including babies.)
"This study shows the favorable results of intestinal transplantation in saving the lives of babies with bowel loss," said Dr. Abu-Elmagd. "These encouraging results should be shared with parents to guide their decision-making process along with their clinical care team."
The study also shows that more awareness of intestinal malrotation is needed to proactively treat the disorder and prevent its potentially fatal complications.
"Neglected symptoms or misdiagnosis can result in a delay in receiving life-changing care," said Dr. Abu-Elmagd. "Therefore, it is important to diagnose the congenital disorder early in life - possibly with greater efforts to establish perinatal screening programs - so that patients can receive timely effective surgical treatment to prevent volvulus and save their bowels."
In this study, the Kareem procedure was performed in 80 patients with intact bowel and disabling symptoms - 92% were adults and 8% were children. Soon after surgery, digestive symptoms improved significantly. With up to 10 years of follow-up, most of these patients can eat normally and with better quality of life. None of the patients developed volvulus after the Kareem procedure.
"The new operation is safe and effective and can be performed in patients of all ages with intestinal malrotation, especially in the presence of disabling symptoms and in those who presented volvulus after Ladd's disease", said Dr. Abu-Elmagd. "It is my hope that this procedure can become part of surgical training for pediatric and adult surgeons."
A feasibility study evaluating a laparoscopic approach to safely performing the procedure is ongoing at the Cleveland Clinic.
Sources: News room - Cleveland Clinic