Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management
| Autor: | Kevin Wymer, Mohan S. Gundeti, Blake B. Anderson, Ashley A. Wilkens |
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| Rok vydání: | 2016 |
| Předmět: |
Male
Intestinal pseudo-obstruction medicine.medical_specialty Voiding cystourethrogram Colon medicine.medical_treatment Urinary Bladder Gastroenterology 03 medical and health sciences Ileostomy 0302 clinical medicine Internal medicine Humans Medicine Abnormalities Multiple Intermittent Urethral Catheterization Survival rate Gastrostomy 030219 obstetrics & reproductive medicine medicine.diagnostic_test business.industry Intestinal Pseudo-Obstruction Infant Newborn Infant General Medicine Megacystis Microcolon medicine.disease Combined Modality Therapy Surgery Cystostomy Treatment Outcome 030220 oncology & carcinogenesis Pediatrics Perinatology and Child Health Female Parenteral Nutrition Total business Hypoperistalsis |
| Zdroj: | Journal of Pediatric Surgery. 51:1565-1573 |
| ISSN: | 0022-3468 |
| DOI: | 10.1016/j.jpedsurg.2016.06.011 |
| Popis: | Introduction Megacystis microcolon intestinal hypoperistalsis (MMIHS) is a rare disorder characterized by distended nonobstructed bladder, microcolon, and decreased intestinal peristalsis. MMIHS has a particularly poor prognosis; however, when appropriately managed, survival can be prolonged. Study design A systematic review (1996–2016) was performed with the key words "megacystis microcolon intestinal hypoperistalsis syndrome." In addition, a case series of four patients is presented as well as algorithms for the diagnosis and treatment of MMIHS. Results 135 patients with MMIHS were identified in the literature. 73% (88/121) of the patients were female, 65% underwent diagnostic biopsy (64/99), and 63% (66/106) were identified with prenatal imaging. The majority of patients were treated with TPN as well as gastrostomy or ileostomy and CIC, however 15% (18/116) received multivisceral or intestinal transplant, and 30% (22/73) had a vesicostomy. The survival rate was 57% (68/121). Conclusion Appropriate management of MMIHS patients is crucial. An enlarged, acontractile bladder in a child with bowel motility problems should be considered diagnostic. Bladder distension can be managed with CIC or vesicostomy in addition to prophylactic antibiotics if frequent urinary tract infections are present. These patients often require gastrostomy or ileostomy as well as total parenteral nutrition. This management has led to significant improvement in survival rates. |
| Databáze: | OpenAIRE |
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