The impact of variation in access to care on the
management of Hirschsprung disease
, Saud Al-Shanafey
, Mohammad Zamakhshary
, Wafa Al-Jarbou
, Mohammed Breakeit
, Safi Qutob
College of Medicine, King Saud University, Riyadh, Saudi Arabia
King Abdulaziz Medical City, Riyadh, Saudi Arabia
King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
Received 18 January 2012; accepted 26 January 2012
Introduction: Variation in access to care has a significant impact on the disease management process
and outcomes. Variable access to care might have similar effects on the management of Hirschsprung
disease (HD). However, such variation has not been highlighted.
Materials and Methods: All patients referred to 3 academic centers (ACs) with HD were reviewed.
Patient presentations, referral patterns, timing, and type of surgical intervention were compared between
patients born in AC and those born in non-AC. Babies born with major congenital anomalies (MCAs) or
total colonic HD were excluded.
Results: Between 1998 and 2011, 129 patients were identified. After excluding 30 patients, 99 were
split into 20 inborn patients (AC) and 79 outborn patients. Outborn patients more often presented with
constipation (95% vs 65%, P = .001), whereas inborn patients presented with feeding intolerance or
vomiting (75% vs 39%, P = .004). Outborn patients were diagnosed and had their pull-through (PT) at
an older median age (in days) of 186 (1-2621) vs 4.5 (1-451) (P = .001) and 345 (11-2757) vs 92
(3-928) (P = .001), respectively. Moreover, inborn patients were more likely to undergo primary PT
(75% vs 46%, P = .02) and avoid bowel diversion (95% vs 66%, P = .02).
Conclusion: Variation in access to care can have a significant impact on the quality of care
delivery in HD. Limited access to AC is associated with staged PT in addition to delay in the
diagnosis and management.
© 2012 Elsevier Inc. All rights reserved.
Despite the increases in health care spending, access to
modern pediatric surgical care is still limited by the number
and distribution of qualiﬁed surgeons as well as the
availability of other ancillary services such as neonatal
care, pediatric anesthesia, and pediatric pathology services.
Hirschsprung disease (HD) is a frequent congenital disease
managed by pediatric surgeons. Its symptoms can manifest
early as neonatal intestinal obstruction or late as chronic
constipation. During the past 3 decades, the surgical
management of HD has passed through a remarkable
evolution. One-stage primary pull-through (PPT) [1-3],
Corresponding author. Department of Surgery (37), King Khalid
University Hospital, PO Box 7805, Riyadh 11472, Saudi Arabia.
E-mail addresses: firstname.lastname@example.org, email@example.com
0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved.
Journal of Pediatric Surgery (2012) 47, 952–955