Alagille syndrome surgery

Jump to navigation Jump to search

Alagille syndrome Microchapters

Home

Patient Info

Overview

Historical Perspective

Pathophysiology

Epidemiology & Demographics

Risk Factors

Causes

Differentiating Alagille syndrome from other Diseases

Natural History, Complications & Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Alagille syndrome surgery On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Alagille syndrome surgery

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Alagille syndrome surgery

CDC on Alagille syndrome surgery

Alagille syndrome surgery in the news

Blogs on Alagille syndrome surgery

Directions to Hospitals Treating Alagille syndrome

Risk calculators and risk factors for Alagille syndrome surgery

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Although Alagille syndrome may be treated through pharmacological therapies, surgical therapy may also be necessary. Surgical procedures include angioplasty, liver transplants, and biliary diversion.

Surgery

Corrective surgery is sometimes needed to repair heart defects associated with Allagile Syndrome. Also, because the pulmonary arteries are often narrow in Alagille patients, a catheterization process similar to angioplasty may be used to widen the arteries to reduce pressure on the heart's pumping valves. In moderate to severe cases, stents may be placed in the arteries to increase their diameter. Transplantation of the liver has been a successful alternative to medication in severe cases. However, liver transplantation from donor parents should always be preceded by genetic testing because cases have been known where donor mother was also found to have alagille syndrome post transplant owing to the autosomal dominant inheritance pattern of the syndrome. Death from graft failure, neurological, and cardiac complications is significantly higher in patients with Alagille Syndrome than patients with Biliary Atresia [1].

Recently, a procedure called partial biliary diversion has been used to significantly reduce pruritus, jaundice, and xanthomas caused by poor bile flow. A portion of the bile produced by the liver is directed through a surgically created stoma into a plastic pouch on the patient's lower right abdomen. The pouch is periodically drained as it fills with bile.

The Kasai procedure, although appropriate for children with biliary atresia, does not benefit children with Alagille Syndrome and actually appears to worsen outcome [2].

References

  1. Arnon R, Annunziato R, Miloh T, Suchy F, Sakworawich A, Sogawa H; et al. (2010). "Orthotopic liver transplantation for children with Alagille syndrome". Pediatr Transplant. 14 (5): 622–8. doi:10.1111/j.1399-3046.2009.01286.x. PMID 20070561.
  2. Kaye AJ, Rand EB, Munoz PS, Spinner NB, Flake AW, Kamath BM (2010). "Effect of Kasai procedure on hepatic outcome in Alagille syndrome". J Pediatr Gastroenterol Nutr. 51 (3): 319–21. doi:10.1097/MPG.0b013e3181df5fd8. PMID 20601899.

Template:WH Template:WS