Hirschsprung disease (HD), like most surgically correctable congenital anomalies, is now survivable well into adulthood. Patients are, therefore, presenting later in life with problems that were previously infrequently encountered by colorectal surgeons or gastroenterologists. The goals of our review are to describe the current state of care transition for this patient population, identify the specific needs for these patients, and propose guidelines which could inform a future model of transition from the pediatric to adult setting.
This is a review of the current state of care transition and long-term outcomes for patients with HD.
While these patients report a generally good quality of life, the majority suffer from some degree of lifelong gastrointestinal complication, regardless of the type of operative repair. A more formalized transition of care will provide a guide for pediatric surgeons and patients, alleviate colorectal surgeon and gastroenterologist concerns, and provide better long-term care for these patients.
*Department of Surgery, Yale University School of Medicine, 333 Cedar Street, New Haven, Connecticut, 06510
†Center for Colon and Pelvic Reconstruction, Department of Pediatric Surgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, Ohio, 43205
‡Department of Colon and Rectal Surgery, The Ohio State University Medical Center, Columbus, Ohio
§University at Buffalo Jacobs School of Medicine and Biomedical Sciences, State University of New York, 219 Bryant Street, Buffalo, New York, 14222
||Primary Children's Hospital, University of Utah School of Medicine, 100 N Mario Capecchi Drive, Salt Lake City, Utah, 84113.
Address correspondence and reprint requests to Amanda W. Harrington, M.D., Surgical Critical Care Fellow, Boston Children's Hospital (e-mail: email@example.com), c/o Doruk E. Ozgediz, M.D., Msc, Yale University School of Medicine, Department of Surgery, Section of Pediatric Surgery, PO Box 208064, New Haven, CT 06520-8064 (e-mail: firstname.lastname@example.org).
Received 30 August, 2018
Accepted 13 April, 2019
Financial Disclosures: The authors have no financial relationships relevant to this article to disclose. No external funding was used.
Prior Presentations: This work has never been presented.
Author Contributions: Dr. Harrington contributed through conception and design of the work, acquisition, analysis and interpretation of the data, as well as drafting and revisions of the manuscript. Drs. Gasior, Einarsdottir, Rothstein, Rollins and Ozgediz contributed through conception and design of the work, analysis and interpretation of the data, revisions of the manuscript and final approval of the version to be published. All authors can recognize the specific contributions of co-authors and all are accountable for all aspects of the work in ensuring that questions related to the accuracy were appropriately investigated and resolved. All authors have confidence in the integrity of the contributions of their co-authors.
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The authors report no conflicts of interest.