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. A retrospective review was performed on all patients with syndromic craniosynostosis who underwent midface distraction with semiburied- or halo-type external distractors. Demographic information and operative/postoperative course were reviewed. Complications were categorized as hardware-related, infectious, and either as major (requiring additional intervention) or minor (requiring medication only). Chi-squared and Fisher exact test were used to compare variables.From 1999 to 2012, a total of 54 patients underwent midface distraction osteogenesis, including 23 patients with Apert syndrome, 19 patients with Crouzon syndrome, 10 patients with Pfeiffer syndrome, and 2 patients with other craniofacial syndromes. Thirty-three patients underwent a total of 34 subcranial Le Fort III distraction procedures and 21 underwent 21 monobloc distraction procedures. The mean age during surgery was 8.0 (range, 4.0-17.7) years, whereas the mean time between distractor placement and removal was 102.9 days. Thirty procedures were performed with external halo-type distractors (18 Le Fort III and 12 monobloc distractions), whereas 25 were performed with buried midface distractors (16 Le Fort III and 9 monobloc distractions). There were no significant differences in diagnoses or interventions between the distraction devices. Of the 19 distractor-related complications, there were a total of 10 (18.2%) in the halo group including 5 (9.1%) requiring separate operative intervention as well as 9 (16.4%) in the buried distractor group including 6 (10.1%) requiring separate operative intervention. Major infections were more common in the buried distractor group (n = 8) compared with the halo distractor group (n = 3) (P = 0.048). There were 4 (7.3%) patients in the halo group who had malposition or transcranial pin migration related to postoperative positioning or falls and required operative repositioning. Frontofacial distraction is an important technique in patients with syndromic craniosynostosis. Higher rates of halo displacement requiring surgery are offset with lower rates of infections compared with buried distractors.

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. The patients were grouped according to severity on the basis of the Kaban-Pruzansky classification: mild (0-1), moderate (2a), and severe (2b-3). Skull base angulation and transverse craniometric measures were recorded and then compared with those of age-matched controls. A total of 30 patients (14 males, 16 females) averaging 7.5 years of age (range, 1.1-15.7 y) were included. Four patients were classified as mild; 12, as moderate; and 14, as severe. The mean cranial base angle was found to be between 179 and 181 degrees with no significant difference between the severity groups (P = 0.57). The mean cranial base angle did not differ significantly in the patients compared with the controls(179.6 vs 180.0; P = 0.51) No significant differences between the affected and unaffected sides in the patients were found in distances from the midline to hypoglossal canal, internal acoustic meatus, lateral carotid canal, medial carotid canal, foramen ovale, and rotundum. There were no significant differences in transverse measurements between the severity classes using the same landmarks (P = 0.46, P = 0.30, P = 0.40, P = 0.25, P = 0.57, and P = 0.76, respectively). The cranial base axis is not deviated in the patients with HFM compared with the age-matched controls, and there exists little difference in endocranial morphologic measurements with increasing severity of HFM. These data are interesting, given the role of the cranial base in facial growth and the varying hypotheses regarding the mechanism of disease in HFM.

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. Suture patency preoperatively and postoperatively was recorded.Thirty patients underwent PVDO for suspected increased intracranial pressure and/or severe turribrachicephaly from 2008 to 2013. Twenty-four patients had syndromic diagnoses. The average age at the time of PVDO was 2.03 years. Distraction distances ranged from 19 to 40 mm, with an average of 28.7 mm. Among the 19 patients who had patent lambdoid sutures before PVDO, new-onset lambdoid fusion was seen in 17 patients after PVDO (89.5%), whereas the suture remained open in 2 patients (10.5%). New-onset lambdoid fusion was not significantly associated with age at distraction (P = 0.28), sex (P = 0.47), length of distraction (P = 0.93), or diagnosis (P = 0.61). Similarly, new-onset sagittal synostosis was not associated with age at distraction (P = 0.06), sex (P = 0.64), length of distraction (P = 0.83), or diagnosis (P = 0.25). None of the patients who developed NOC had characteristic head shape changes such as mastoid bulges or scaphocephaly. New-onset lambdoid and sagittal synostoses occur frequently after PVDO. Although the diagnosis of NOC is obvious radiographically, the clinical importance of the diagnosis morphometrically, neurodevelopmentally, and in cranial growth has yet to be fully investigated.

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. Demographic information, operative details, and perioperative course were collected. Complications were categorized as either major or minor. A χ(2) test and Fisher exact test were used to compare all categorical variables. Continuous variables were analyzed using Wilcoxon rank-sum and Kruskal-Wallis tests.Seven hundred forty-six patients underwent surgical correction of nonsyndromic craniosynostosis. Of these, there were 307 (41.2%) sagittal, 201 (26.9%) metopic, and 238 (31.9%) unicoronal. Thirty-four patients had complications (4.6%). Eight were considered major (1.1%), including one postoperative mortality in a patient with hypoplastic left-sided heart syndrome. Minor complications occurred in 26 patients (3.5%) and included subgaleal hematoma (n = 3), seroma (n = 4), and superficial wound infection (n = 5). Metopic and sagittal suture involvement was significantly associated with a higher complication rate (P = 0.04). A child with isolated single suture synostosis and any comorbidity had a significantly greater risk of any complication (P < 0.001; odds ratio, 3.8) and specifically an increased risk of major complication (P = 0.031; odds ratio, 6.0). Subclassification of patients by time period yielded no statistically significant changes in perioperative morbidity. To conclude, these data allow us to counsel families more accurately with regard to morbidity and mortality and may potentially serve as a benchmark for future quality improvement work.

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. At least 1 computed tomography (CT) scan was required to assess SOS patency. Age-/sex-matched control CT scans were also assessed for SOS patency. Three independent reviewers with high interrater reliability (κ = 0.88) graded SOS patency as open, partially closed, or completely closed. Wilcoxon rank sum test compared the Pfeiffer patients with control subjects. A total of 63 CT scans in 16 patients with Pfeiffer syndrome, all with midface hypoplasia, and 63 age-/sex-matched control scans, none of whom had midface hypoplasia, met inclusion criteria. Earliest partial SOS closure in patients with Pfeiffer syndrome was seen at 5 days compared with control subjects at 7.07 years. Earliest age at complete fusion was 2.76 years in the Pfeiffer cohort and 12.74 years in control subjects. Average age at partial closure was significantly younger (4.99 ± 3.33 years; n = 31 scans) in patients with Pfeiffer syndrome compared with control subjects (10.92 ± 3.53 years) (P = 0.0005), whereas average age at complete closure (11.90 ± 7.04 years) was not significantly different than that in control subjects (16.07 ± 3.39 years). Although definitive causality cannot be concluded, a strong correlation exists between midface hypoplasia and premature SOS closure in 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. An anonymous satisfaction survey was sent to families of craniofacial patients treated between January 1976 and June 2012. All patients who had undergone at least 1 craniofacial procedure had addresses on file and were included in this study. Statistical analyses were performed using the Mann-Whitney U test.During the study, 441 surveys were mailed to families meeting the inclusion criteria. A total of 151 families returned completed surveys (34.2%), and 121 surveys were included for analysis (27.4%). In rating overall satisfaction, families who met with the PL had statistically higher scores than those who had not (P = 0.0011). Parents who met with the PL preoperatively reported greater satisfaction in time spent answering questions (P = 0.0029) and the perception that questions were adequately answered (P = 0.0039). No statistical difference was observed in postoperative preparedness between families that did and did not meet the PL. The results demonstrate that the PL is beneficial in the education, experience, and satisfaction of families treated at a large Craniofacial Center. The PL complements the surgeon's treatment of the physical by adding psychosocial support.

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. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences. Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.

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). Posterior vault distraction and simultaneous transfacial pin placement under 3D CT surgical navigation was planned to concurrently relieve intracranial pressure and address airway obstruction by distraction of the midface without osteotomy. An uncomplicated surgery was completed in 290 minutes with an estimated blood loss of 340 mL. Midface distraction was initiated on postoperative day 1 at a rate of 1 mm per day for 21 days, which translated to 13 mm of midfacial advancement. Posterior vault distraction was initiated on postoperative day 5 at a rate of 1 mm per day for 25 days with a resultant 25 mm of posterior vault expansion. Transfacial pin and external distractors were removed after 6 weeks, and posterior vault distractors were removed after 8 weeks of consolidation. Craniometric comparison of 3D-CT scan demonstrated an increase in cranial vault volume of 47%, and the patient was able to be weaned from her home oxygen requirement. This is the first report of simultaneous posterior vault distraction with midfacial advancement across open facial sutures using surgical navigation as an initial stage in treating syndromic craniosynostosis.

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. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences.Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.