Cranial base deviation in hemifacial microsomia by craniometric analysis.

Although facial asymmetry in hemifacial microsomia (HFM) is well documented in the literature, no studies have concentrated on the morphology of the cranial base. This study aimed to evaluate the endocranial morphology in patients with HFM. Consecutive patients with unilateral HFM treated at a craniofacial center from 2000 to 2012 were included.

Complications in 54 frontofacial distraction procedures in patients with syndromic craniosynostosis.

Patients with syndromic craniosynostosis manifest midfacial hypoplasia often treated by midfacial advancement. Benefits of midfacial advancement by distraction osteogenesis have been well studied; little is known about the perioperative morbidity of these procedures, specifically relating to device selection. This study compares the perioperative complications between semiburied- and halo-type distraction osteogenesis of the midface.

Perioperative complications associated with intracranial procedures in patients with nonsyndromic single-suture craniosynostosis.

Within the diagnosis "craniosynostosis," there is a subset of patients who present with isolated, nonsyndromic, single-suture involvement. This study evaluates perioperative complications in this specific subset of patients over 4 decades at a single institution. To do so, we performed a retrospective review on consecutive patients undergoing correction of single-suture synostosis from May 1977 to January 2013 at a tertiary pediatric craniofacial center.

New-onset craniosynostosis after posterior vault distraction osteogenesis.

The aims of this study were to document the incidence of new-onset craniosynostosis (NOC) after posterior vault distraction osteogenesis (PVDO), to determine risk factors for the development of NOC, and to deduce the cranial ramifications of NOC. An institutional review board-approved retrospective review of all patients who underwent PVDO at the Children's Hospital of Philadelphia was performed. Demographics, perioperative data, as well as preoperative and postoperative three-dimensional computed tomographic scans were analyzed.

Premature closure of the spheno-occipital synchondrosis in Pfeiffer syndrome: a link to midface hypoplasia.

The spheno-occipital synchondrosis (SOS) is a critical component of midfacial and cranial base growth. Premature closure has been associated with midface hypoplasia in animal models and syndromic craniosynostosis subpopulations with Apert and Muenke syndromes. To link premature SOS closure and midface hypoplasia in patients with Pfeiffer syndrome, a retrospective case-control study was performed in patients treated at a large craniofacial center between 1982 and 2012 diagnosed with Pfeiffer syndrome.

The impact of a parent liaison at a major craniofacial center.

The psychosocial impact of craniofacial disfigurement affects both the developing child and his/her family. The Facial Reconstruction Center at the Children's Hospital of Philadelphia has employed a Parent Liaison (PL) to provide psychosocial support to families and has been an invaluable resource in this regard. We hypothesize that a PL impacts the overall outcome of the surgery by building trust between the parents and medical institution, and increasing satisfaction.

Monobloc minus Le Fort II for single-stage treatment of the Apert phenotype.

Treatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia.

Posterior vault distraction with midface distraction without osteotomy as a first stage for syndromic craniosynostosis.

Syndromic craniosynostosis (SC) may cause intracranial hypertension, exorbitism, midfacial hypoplasia associated with airway obstruction, and class III malocclusion. Current treatment strategies for SC involve expansion of the cranial vault and orbits, and midfacial advancement to relieve symptoms and sequelae of obstructive sleep apnea. We present a case of a 12-week-old female patient with Saethre-Chotzen syndrome who presented with bicoronal craniosynostosis, severe turribrachycephaly, midface hypoplasia, and a history of obstructive sleep apnea (apnea-hypopnea index = 14).

Monobloc minus Le Fort II for single-stage treatment of the Apert phenotype.

Treatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia.