Evolution in Epidemiology of Craniosynostosis

at Miami Children’s Hospital

F. Paul Marji MD
Research Fellow: Miami Children’s Hospital, Miami FL
UPMC Children’s Hospital Craniofacial Surgery Dept, Pittsburgh PA
Surgery Resident, Georgetown University Hospital, Washington DC
Financial Disclosure or Conflicts of Interest:
None
 Introduction
• Cranial Sutures: composed of fibrous tissue which connect the cranial bones as they
verge during craniofacial development.

• Fulfill crucial roles during infancy:

• Skull plasticity and mobility necessary to advance through the birth canal.
• Enabling rapid expansion of the cranial vault during early brain growth.

• Major sutures: sagittal, coronal, metopic and lambdoid.

Sara Baretto et. al.Identification of stiffness-induced signalling mechanisms in cells from

patent and fused sutures associated with craniosynostosis
 Background
• Craniosynostosis: Premature partial or complete fusion of one or
more of the cranial sutures
• Relatively common: 1 in 2,000 to 2,500 live births

• Can cause alterations in craniofacial morphology, functional abnormalities

• Can be of an isolated suture, multiple sutures, or associated with syndromes

(Apert, Crouzon, Muenke and Pfeiffer, among others).

• The frequency of the subtype of suture affected varies greatly:

• sagittal suture classically most commonly involved in nonsyndromic cases
(40-55%), coronal synostosis (20-25%), metopic synostosis (5-10 percent),
lambdoid synostosis most rare subtype (0-5%).
• In recent years - gradual change in the distribution of these four main subtypes of
nonsyndromic craniosynostosis has been seen at several institutions, increase in
metopic. 1-5
 Objective
• Explore subtypes frequencies of craniosynostosis as it pertains to cases
treated at Miami Children’s Hospital over the last 3 decades.

• To elucidate the relative proportional changes in suture fusion subtypes

over this time period.
 Methods
• Retrospective chart review of pediatric patients surgically treated by the
Department Plastic Surgery from December 1980 to December 2013 for
craniosynostosis.

• Single suture, multiple suture, or syndromic craniosynostosis cases were

included.
• Nonsurgical candidates for craniosynostosis were excluded.

• Frequencies and relative proportions were computed along with 95%

confidence intervals for four major time intervals: before 1986, 1986-1995,
1996-2005, and after 2005.

• Additional data pertaining to variables of patient gender, age at primary

procedure, surgical procedure, and overall long-term surgical outcomes
were also investigated.
 Results
Table 1a: Demographics Features of Patients Treated

Table 1b: Demographics Features of

Patients Treated
Results
Results
 Conclusions
• A decrease in the proportion of syndromic cases has not previously been described, and the
exact causal relationship is likely multifactorial. An increase in the portion of sagittal cases
seen may be due to higher detection rates or even changing incidences. Future work on
elucidating the causes of theses changes is warranted.

• Did not observe a statistically significant increase in relative proportion of metopic CS

 Limitations
• Sample Size, low power

• Some patients underwent care by neurosurgical dept.

 References
1) Selber J, Reid RR, Chike-Obi CJ, Sutton LN, Zackai EH, McDonald-McGinn D, et al. The changing epidemiologic spectrum of single-suture synostoses. Plast Reconstr Surg.
2008 Aug;122(2):527-33.

2) Van der Meulen J, van der Hulst R, van Adrichem L, Arnaud E, Chin-Shong D, Duncan C, et al. The increase of metopic synostosis: a pan-European observation. J Craniofac
Surg. 2009 Mar;20(2):283-6.

3) Di Rocco F, Arnaud E, Renier D. Evolution in the frequency of nonsyndromic craniosynostosis. J Neurosurg Pediatr. 2009 Jul;4(1):21-5.

4) Lee HQ, Hutson JM, Wray AC, Lo PA, Chong DK, Holmes AD, et al. Changing epidemiology of nonsyndromic craniosynostosis and revisiting the risk factors. J Craniofac Surg.
2012 Sep;23(5):1245-51.

5) Singer S, Bower C, Southall P, Goldblatt J. Craniosynostosis in Western Australia, 1980-1994: a population-based study. Am J Med Genet. 1999 Apr 23;83(5):382-7.