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A SINGLE CENTER’S EXPERIENCE WITH ISOLATED UNICORONAL CRANIOSYNOSTOSIS RECONSTRUCTION: LONG-TERM OUTCOMES OF 182 PATIENTS OVER 35 YEARS
10:30 am - 10:40 am
Presenter: J. Thomas Paliga, BA, Children's Hospital of Philadelphia

Authors: J. Thomas Paliga (1), Ari Wes (2), Jesse Goldstein (3), Linton Whitaker (4), Scott Bartlett (1), Jesse Taylor (5)

Institutions:
(1) Children's Hospital of Philadelphia, Philadelphia, PA, (2) The Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA, (3) University of North Carolina, Chapel Hill, Chapel Hill, NC, (4) Children's Hosp. of Philadelphia, Philadelphia, PA, (5) The Children's Hospital of Philadelphia and Perelman School of Medicine, Philadelphia, PA
First Author:
J. Thomas Paliga, BA
Children's Hospital of Philadelphia
Background/Purpose:
Assess long-term outcomes of patients with isolated unicoronal synostosis treated at our institution over a thirty-five year period.
Methods/Description:
A retrospective review was performed of patients with unicoronal synostosis from 1977 to 2012. Patients were excluded with prior outside intervention, or diagnosis of syndromic or multisutural synostosis. Demographic, operative/post-operative data and outcomes were analyzed with chi-squared and Fisher's exact test for categorical data and Wilcoxon rank-sum and Kruskal-Wallis rank for continuous data.
Results:
Over 35 years, 182 patients were treated for unicoronal synostosis and 156 met inclusion criteria. The patient population was predominantly female(65%,n=101) with synostosis predominantly on the right(60%,n=93). Patients presented with supraorbital retrusion(95%,n=148), orbital dysmorphology(71%,n=110), compensatory bossing (51%,n=79), nasal root deviation(38%,n=59), occipital irregularity(12%,n=18), and midface asymmetry(8%,n=12). Primary intervention included 55(35%) unilateral fronto-orbital advancements(FOA) with unilateral frontal craniotomy, 54(35%) incomplete bilateral FOA with bilateral frontal craniotomy, 42(27%) unilateral FOA with bilateral frontal craniotomy, and 5(3%) bilateral FOA with bilateral frontal craniotomy at a mean age of 0.98±1.0 years. There were 3 acute surgical complications(3%). Mean follow up was 5.9±5.0 years, and in patients with >=1 year follow-up(n=129), 57(44%) required second intervention. At definitive intervention, 70(54%) patients were Whitaker class I, 6(5%) class II, 50(39%) class III, and 3(3%) class IV. Recurrence of the preoperative abnormality was noted in 71(55%) patients at latest follow-up. Patients with >=5 years follow-up were more often class III(p<.001) and less often class I(p<.001) and developed supraorbital retrusion following primary intervention more often than their counterparts with <5 year follow-up (p<.001).
Conclusions:
In the largest outcome evaluation of isolated unicoronal synostosis, we critically evaluate outcomes to help shape expectations that may be useful when counseling patients and families.