Defining the Need for Transitional Care From Pediatric to Adult Surgery for Young Adult Patients With Surgically Corrected Congenital Anomalies

A child born with a congenital birth defect today has a much higher probability of reaching adulthood than in the past. Significant advances in neonatal resuscitation and surgical management have improved prognoses for diseases such as congenital diaphragmatic hernia, esophageal atresia, and imperforate anus. As these children grow into adolescence, new challenges emerge as results from long-term studies that show ongoing morbidity among adolescent and adult survivors demonstrate a clear need for structured and formal transitional care.^1,2 Unanswered, however, are the questions of how and when the transition of care should take place. Few quantitative data exist about patients’ transition to adult care and their long-term follow-up. A growing body of literature suggests that there are long-term sequelae of many surgically corrected congenital anomalies, such as metaplastic changes in patients with esophageal atresia repair and chest wall deformities in patients undergoing thoracotomy in infancy. Survivors of congenital surgical disease are those who will benefit most from standardized approaches to transitional care. Our study seeks to quantify the need for transitional surgical care for pediatric patients with surgical anomalies in the coming decades.

The institutional review board at the University at Buffalo, State University of New York, deemed our study to be exempt from approval because the research does not include any patient contact or records. Seven correctable thoracic and gastrointestinal pediatric surgical anomalies (omphalocele, congenital diaphragmatic hernia, Hirschsprung disease, esophageal atresia/tracheoesophageal fistula, myelomeningocele, anorectal malformation, and gastroschisis) were analyzed, and their incidences were extrapolated for the time period from 2030 to 2050. These anomalies were chosen because they are relatively common, survivable congenital anomalies that often require long-term care. The initial year of contribution was arbitrarily set at 2015 to provide a contemporary analysis of the burden of care that exists. The year 2030 would then represent the first year of patients reaching 15 years of age, when transition of care should begin. Incidences were calculated using the following method:

[New Patients Starting at 15 Years of Age by Disease]_year = (Disease Prevalence) × (Projected Annual Live Births_year) × (Disease-Specific Survival Rates) × (Actuarial Survival Rates by Age).^3-6

The cumulative burden of disease projected for year is

More than 173 000 patients living with some of these surgically corrected thoracic and gastrointestinal anomalies will have entered the pool of patients who will benefit from a coordinated surgical transition plan by 2050 (Figure). Gastroschisis and anorectal malformations comprise the largest subgroups of patients.

The incidence of young adults living with surgically correctable thoracic and gastrointestinal anomalies will trend upward over the next few decades. This group of patients is at risk for late complications related to their congenital anomalies and surgical procedures. The care burden among patients in this group will undoubtedly be variable, from minimal to complex and significant. More research is necessary to elucidate the spectrum of required care, focusing on morbidity, quality of life, and health care costs. The creation of formal transitional care plans and follow-up may mitigate unfavorable long-term consequences and decrease overall health care costs. Adult care professionals should partner with the pediatric team in this process to inform and plan for the care of newly transitioned complex patients. There has never been a more opportune time than now to address the need for transitional care from pediatric to adult surgery.

Corresponding Author: David H. Rothstein, MD, MS, University at Buffalo, State University of New York and Women and Children’s Hospital of Buffalo, 219 Bryant St, Buffalo, NY 14222 (drothstein@kaleidahealth.org).

Published Online: December 16, 2015. doi:10.1001/jamasurg.2015.4417.

Author Contributions: Drs Zhao and Rothstein had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Study concept and design: All authors.

Acquisition, analysis, or interpretation of data: Zhao, Chiu, Rothstein.

Drafting of the manuscript: Zhao, Rothstein.

Critical revision of the manuscript for important intellectual content: All authors.

Statistical analysis: Zhao, Rothstein.

Administrative, technical, or material support: Chiu.

Study supervision: Dasgupta, Rothstein.

Conflict of Interest Disclosures: None reported.