Onset and Resolution of Chiari Malformations and Hydrocephalus in Syndromic Craniosynostosis following Posterior Vault Distraction.

BACKGROUND: Patients with syndromic craniosynostosis have an increased incidence of progressive hydrocephalus and Chiari malformations, with few data on the relative benefit of various surgical interventions. The authors compare the incidence and resolution of Chiari malformations and hydrocephalus between patients undergoing posterior vault distraction osteogenesis (PVDO) and patients undergoing conventional cranial vault remodeling. METHODS: Patients with syndromic craniosynostosis who underwent cranial vault surgery from 2004 to 2016 at a single academic hospital, with adequate radiographic assessments, were reviewed. Demographics, interventions, the presence of a Chiari malformation on radiographic studies and hydrocephalus requiring shunt placement were recorded. Mann-Whitney U and Fisher's exact tests were used as appropriate. RESULTS: Forty-nine patients underwent PVDO, and 23 patients underwent cranial vault remodeling during the study period. Median age at surgery (p = 0.880), sex (p = 0.123), and types of syndrome (p = 0.583) were well matched. Patients who underwent PVDO had a decreased incidence of developing Chiari malformations postoperatively compared with the cranial vault remodeling cohort (2.0 percent versus 17.4 percent; p = 0.033). Not surprisingly, no significant difference was found between the groups with regard to the incidence of postoperative hydrocephalus requiring shunt placement (PVDO, 4.1 percent; cranial vault remodeling, 4.3 percent; p = 0.999). CONCLUSIONS: As expected, PVDO did not significantly affect intracranial hydrodynamics to the extent that hydrocephalus shunting rates were different for patients with syndromic craniosynostosis. However, PVDO was associated with a reduced risk of developing a Chiari malformation; however, prospective evaluation is needed to determine causality. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Risk Factors for Perioperative Respiratory Failure following Mandibular Distraction Osteogenesis for Micrognathia: A Retrospective Cohort Study.

BACKGROUND: The frequency of respiratory events in the perioperative period, and optimal duration of intubation during early mandibular distraction osteogenesis activation, are poorly understood. This study assesses potential risk factors associated with perioperative respiratory events, particularly the need for reintubation, following mandibular distraction osteogenesis surgery. METHODS: A retrospective review was conducted for infants (younger than 1 year) undergoing mandibular distraction osteogenesis for tongue-based airway obstruction between November of 2010 and December of 2017. Univariate and multivariate analyses of sentinel events and outcomes were performed. RESULTS: Ninety infants (median age, 35 days) were included (50 percent were syndromic). Twenty-seven subjects (30 percent) experienced a respiratory event requiring intervention before discharge, including 14 subjects who failed initial extubation. Subjects extubated earlier than postoperative day 5 failed extubation more frequently (33%) compared to those extubated later (9%; p = 0.005). Respiratory events occurred more frequently when extubation was attempted at distraction lengths of 5 mm or less (42 percent) compared to greater than 5 mm (21 percent; p = 0.032). Logistic regression modeling showed that syndromic status (OR, 14.8) and secondary airway anomaly (OR, 6.1) were significant predictors for respiratory events, whereas greater length of distraction at the time of extubation was protective (OR, 0.8; p < 0.05). CONCLUSIONS: Postoperative intubation of at least 5 days with associated mean distraction of 5 mm appears to be associated with successful extubation trial following mandibular distraction osteogenesis surgery. Patients with congenital syndromes and secondary airway anomalies are more likely to experience perioperative respiratory events. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Perioperative Outcomes of Secondary Frontal Orbital Advancement After Posterior Vault Distraction Osteogenesis.

BACKGROUND: Posterior cranial vault distraction osteogenesis (PVDO) has gained popularity as the initial intervention in patients with syndromic craniosynostosis. Patients may require secondary frontal orbital advancement (FOA) following PVDO, but little is known about the perioperative risks associated with this staged management. The purpose of this study is to compare the perioperative morbidity profile of secondary FOA (study) to that of primary FOA (control). METHODS: A retrospective review was conducted for patients with syndromic or complex craniosynostosis undergoing FOA between 2004 and 2017. Univariate and multivariate analysis of demographic and perioperative data were performed. RESULTS: Forty-three subjects met inclusion criteria, 17 in the study cohort and 26 in the control cohort. The 2 cohorts were similar with regards to diagnosis and suture involvement, as well as weight-adjusted estimated blood loss, blood transfusion volume, and length of hospital stay (P > 0.050). Secondary FOA procedures required longer operating time (231 ±â€Š58 versus 264 ±â€Š62 min, P = 0.031) and anesthesia time (341 ±â€Š60 versus 403 ±â€Š56 min, P = 0.002). The secondary FOA cohort had a significantly greater proportion of procedures with difficult wound closure (19% versus 59%, P = 0.008). Two subjects in the study cohort developed a wound dehiscence, compared with 1 subject in the control cohort (P = 0.552). Frontal orbital advancement as a secondary procedure after PVDO was a predictor variable in multivariate analysis for wound difficulties (odds ratio 8.6, P = 0.038). CONCLUSION: Syndromic and complex craniosynostosis may safely be managed with initial PVDO followed by FOA, with some increased wound closure difficulty.

Retrospective Review of the Complication Profile Associated with 71 Subcranial and Transcranial Midface Distraction Procedures at a Single Institution.

BACKGROUND: This study characterizes the perioperative morbidity of a large cohort of subjects with syndromic craniosynostosis who underwent transcranial or subcranial midface distraction. METHODS: Demographic and perioperative data were compared between those who underwent transcranial or subcranial midface distraction osteogenesis between July of 1999 and December of 2017. Univariate analysis was conducted using chi-square and Fisher's exact tests for categorical variables and the Mann-Whitney U test for continuous variables. Multivariate analysis was conducted using logistic regression modeling. Complications were graded using the Clavien-Dindo classification. RESULTS: Sixty-four subjects underwent a total of 71 midface distraction procedures. There was a total of 28 complications (39 percent). The trans cranial cohort had a significantly higher frequency of complications (58 percent) compared with the subcranial cohort (29 percent; p = 0.017), with a significantly greater proportion of infection-related complications in the transcranial cohort (80 percent versus 54 percent; p = 0.028). Transcranial complications included cranial contamination, whereas most subcranial cohort infections were superficial or limited facial abscesses. The only significant predictor variable for complications in a multivariate analysis was whether the osteotomy approach was transcranial as opposed to subcranial, with an odds ratio of 5.80 (p = 0.013). CONCLUSIONS: Complication rates in midface distraction remain high, with transcranial procedures having significantly higher complication rates, infection-related complications, and notably greater severity of complications. Although the goals of surgery often dictate choice of osteotomy, the risks associated with transcranial procedures must be thoroughly understood by surgeon and patient alike. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

A Standardized Perioperative Clinical Pathway for Uncomplicated Craniosynostosis Repair Is Associated With Reduced Hospital Resource Utilization.

BACKGROUND: Hospital resource overutilization can significantly disrupt patient treatment such as cancelling surgical patients due to a lack of intensive care unit (ICU) space. The authors describe a clinical pathway (CP) designed to reduce ICU length of stay (LOS) for nonsyndromic single-suture craniosynostosis (nsSSC) patients undergoing cranial vault reconstruction (CVR) in order to minimize surgical disruptions and improve patient outcomes. METHODS: A multidisciplinary team implemented a perioperative CP including scheduled laboratory testing to decrease ICU LOS. Hospital and ICU LOS, interventions, and perioperative morbidity-infection rate, cerebrospinal fluid (CSF) leaks, and unplanned return to the operating room (OR)-were compared using Mann-Whitney U, Fisher exact, and t tests. RESULTS: Fifty-one ICU admissions were managed with the standardized CP and compared to 49 admissions in the 12 months prior to pathway implementation. There was a significant reduction in ICU LOS (control: mean 1.84 ±â€Š0.93, median 1.89 ±â€Š0.94; CP: mean 1.15 ±â€Š0.34, median 1.03 ±â€Š0.34 days; P < 0.001 for both). There were similar rates of hypotension requiring intervention (CP: 2, control: 1; P = 0.999), postoperative transfusion (CP: 3, control: 0; P = 0.243), and artificial ventilation (CP: 1, control: 0; P = 0.999). Perioperative morbidity such as infection (CP: 1, control: 0; P = 0.999), return to the OR (CP: 1, control: 0; P = 0.999), and CSF leak (no leaks; P = 0.999) was also similar. CONCLUSION: Implementation of a standardized perioperative CP for nsSSC patients resulted in a significantly shorter ICU LOS without a measured change in perioperative morbidity. Pathways such as the one described that improve patient throughput and decrease resource utilization benefit craniofacial teams in conducting an efficient service while providing high-quality care.

Nasal Obstruction in Children With Cleft Lip and Palate: Results of a Cross-Sectional Study Utilizing the NOSE Scale.

OBJECTIVE: To characterize the epidemiology and risk factors for nasal obstruction among subjects with cleft lip and/or cleft palate (CL/P) utilizing the well-validated Nasal Obstruction Symptom Evaluation (NOSE) survey. DESIGN: Retrospective cross-sectional study. SETTING: Cleft Lip and Palate Program, Children's Hospital of Philadelphia. PATIENTS, SUBJECTS: One thousand twenty-eight surveys obtained from 456 subjects (mean age: 10.10 (4.48) years) with CL/P evaluated between January 2015 and August 2017 with at least 1 completed NOSE survey. INTERVENTIONS: Nasal Obstruction Symptom Evaluation surveys completed at each annual visit. MAIN OUTCOME MEASURES: Composite NOSE and individual symptom scores. RESULTS: Sixty-seven percent of subjects had nasal obstruction at some point during the study period, with 49% reporting nasal obstruction at latest follow-up. subjects aged 14 years and older reported the most severe symptoms ( P = .002). Subjects with cleft lip and alveolus (CL+A) and unilateral cleft lip and palate (CLP) reported more severe nasal blockage than other phenotypes ( P = .021). subjects with a history of either posterior pharyngeal flap (PPF) or sphincter pharyngoplasty (SP) had significantly higher NOSE scores than subjects with no history of speech surgery ( P = .006). There was no significant difference ( P > .050) in NOSE scores with regard to history of primary tip rhinoplasty, nasal stent use, or nasoalveolar molding. CONCLUSIONS: There are more severe nasal obstructive symptoms among subjects older than 14 years of age, with CL+A or unilateral CLP, and with a history of PPF or SP. Future studies utilizing the NOSE are needed to evaluate and address this prevalent morbidity in the CLP population.

Early Mandibular Distraction in Craniofacial Microsomia and Need for Orthognathic Correction at Skeletal Maturity: A Comparative Long-Term Follow-Up Study.

BACKGROUND: Controversy exists regarding the treatment of mandibular hypoplasia in craniofacial microsomia patients, notably the role of mandibular distraction osteogenesis. The authors compared the need for orthognathic surgery in skeletally mature craniofacial microsomia subjects who either did (study group) or did not (control group) undergo early mandibular distraction osteogenesis. METHODS: A retrospective review was conducted of all craniofacial microsomia patients evaluated between January of 1993 and March of 2017. This study included patients with a Kaban-Pruzansky grade I to III mandible, and who were at least 14 years old at the time of the latest follow-up. RESULTS: Thirty-eight subjects met inclusion criteria: 17 who underwent mandibular distraction osteogenesis and 21 who did not (mean age, 18.95 ± 2.82 years versus 17.95 ± 2.14 years, respectively; p = 0.246). The degree of mandibular deformity was matched (distraction, 29.4 percent Kaban-Pruzansky grade IIb and 5.9 percent grade III; no distraction, 23.8 percent grade IIb and 9.5 percent grade III; p = 0.788). No significant difference was noted between the distraction and no-distraction cohorts with regard to need for orthognathic surgery [distraction, n = 10 (58.8 percent); no distraction, n = 8 (38.1 percent); p = 0.203]. CONCLUSIONS: The results seem to suggest that there is no significant difference in orthognathic surgery rates at skeletal maturity between craniofacial microsomia subjects who underwent early mandibular distraction osteogenesis and those who did not. Subjects who undergo distraction may still ultimately require orthognathic surgery to correct facial asymmetry. Additional studies are required to determine the optimal timing and technique of distraction, the importance of orthodontic management during and after distraction, and the early psychosocial benefits of improved facial symmetry. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Influence of Repaired Cleft Lip and Palate on Layperson Perception following Orthognathic Surgery.

BACKGROUND: Facial scarring and disharmony caused by clefting are associated with psychosocial stress, which may be improved by orthognathic surgery. The authors examine how clefting influences change in layperson perception of a patient following orthognathic surgery. METHODS: One thousand laypersons were recruited through Mechanical Turk to evaluate patient photographs before and after orthognathic surgery. Nineteen patients-five with unilateral and five with bilateral clefting-were included. Respondents assessed six personality traits, six emotional expressions, and likelihood of seven interpersonal experiences on a scale from 1 to 7. RESULTS: Changes in all aspects of social perception after the procedure differed significantly between cleft versus noncleft cohorts (p < 0.01 for all). Respondents evaluated the change for the cleft cohort compared with the noncleft cohort as more trustworthy, friendly, sad, and afraid; more likely to feel lonely, be teased or bullied by others, or feel anxious around others; less angry, disgusted, threatening, dominant, intelligent, happy, and attractive; and less likely to have romantic relationships, friends, or be praised by others. For unilateral versus bilateral cleft cohorts, change in social perception was significantly different in four of the 19 items (p < 0.05 for all). Social perception change for the unilateral cohort was less surprised, sad, dominant, or happy compared with the bilateral cohort (p < 0.05 for all). CONCLUSIONS: Despite significant improvements in social perception following orthognathic surgery, cleft patients benefit less than noncleft patients. These findings may be useful to counsel postsurgical expectations for cleft patients undergoing orthognathic surgery.

Surgical Management and Outcomes of Pierre Robin Sequence: A Comparison of Mandibular Distraction Osteogenesis and Tongue-Lip Adhesion.

There is a paucity of literature directly comparing tongue-lip adhesion versus mandibular distraction osteogenesis in surgical treatment of patients with Pierre Robin sequence. This study comprehensively reviews the literature for evaluating airway and feeding outcomes following mandibular distraction osteogenesis and tongue-lip adhesion. A search was performed using the MEDLINE and Embase databases for publications between 1960 and June of 2017. English-language, original studies subjects were included. Extracted data included prevention of tracheostomy (primary airway outcome) and ability to feed exclusively by mouth (primary feeding outcome). A total of 67 studies were included. Ninety-five percent of subjects (657 of 693) treated with mandibular distraction osteogenesis avoided tracheostomy, compared to 89% of subjects (289 of 323) treated with tongue-lip adhesion. Eighty-seven percent of subjects (323 of 370) treated with mandibular distraction osteogenesis achieved full oral feeds at latest follow-up. Seventy percent of subjects (110 of 157) treated with tongue-lip adhesion achieved full oral feeds at latest follow-up. The incidence of second intervention for recurrent obstruction ranged from 4 to 6 percent in mandibular distraction osteogenesis studies, compared to a range of 22 to 45 percent in tongue-lip adhesion studies. Variability of patient selection, surgical techniques, outcomes measurement methods, and follow-up length across studies precluded meta-analysis of the data. Both mandibular distraction osteogenesis and tongue-lip adhesion are effective alternatives to tracheostomy for patients who fail conservative management and improve feeding. Mandibular distraction osteogenesis may be superior to tongue-lip adhesion in long-term resolution of airway obstruction and avoidance of gastrostomy, but is associated with notable complications.

Evaluation of parental and surgeon stressors and perceptions of distraction osteogenesis in pediatric craniofacial patients: a cross-sectional survey study.

PURPOSE: There is a paucity of literature on how limitations of distraction osteogenesis (DO) are perceived by physicians and parents of pediatric patients. Specifically understanding which features of DO are most concerning to these two groups may better inform parent education, as well as direct improvements in distraction protocols and devices. METHOD: Parents/guardians of patients (between January 2016 and October 2017) being treated with craniofacial distraction were recruited to complete a survey regarding level of stress (1 = not stressful, 9 = maximally stressful) associated with eight features of DO. Craniofacial surgeons completed a survey asking them to report (1) their personal level of stress and (2) their perceptions of parental stress regarding these same eight features of DO. RESULTS: Thirty-five parents and 15 craniofacial surgeons completed the survey. The risk of the device getting infected was perceived as most stressful by parents (5.5 ± 2.3) followed by the device sticking through the skin (4.9 ± 2.6) and the second operation for removal (4.7 ± 2.3). These same three features also elicited the highest level of stress among surgeons. Surgeon-perceived parental stress regarding turning of the distractor (5.8 ± 1.5) was significantly higher than parent self-reported stress (4.2 ± 2.8, p = 0.042). CONCLUSIONS: Both parents and surgeons perceive risk of device-associated infection, the protrusion of the device through the skin, and the requirement of a second operation for removal as the most stressful drawbacks of distraction. Infection reduction protocols, less obtrusive devices, and devices that do not require removal are potential targets for stress reduction.

Posterior Vault Distraction Osteogenesis in Nonsyndromic Patients: An Evaluation of Indications and Safety.

PURPOSE: The purpose of this study was to evaluate the indications, safety, and short-term outcomes of posterior vault distraction osteogenesis (PVDO) in patients with no identified acrocephalosyndactyly syndrome (study) and to compare those to a syndromic cohort (controls). METHODS: Demographic and perioperative data were recorded and compared across the study and control groups for those who underwent PVDO between January 2009 and December 2016. Univariate analysis was conducted using χ and Fisher exact tests for categorical variables, and Mann-Whitney U test for continuous variables. RESULTS: Sixty-three subjects were included: 19 in the nonsyndromic cohort, 44 in the syndromic cohort. The cohorts had similar proportion of subjects exhibiting pansynostosis (42.1% of nonsyndromic versus 36.4% of syndromic, P = 0.667). The nonsyndromic cohort was significantly older (4.04 ±â€Š3.66 years versus 2.55 ±â€Š3.34 years, P = 0.046) and had higher rate of signs of raised intracranial pressure (68.4% versus 25.0%, P = 0.001) than the syndromic cohort. There was no significant difference in perioperative variables or rate of complications (P > 0.05). The mean total advancement distance achieved was similar, 27 ±â€Š6 mm in the nonsyndromic versus 28 ±â€Š8 mm in the syndromic cohort (P = 0.964). All nonsyndromic subjects with signs of raised intracranial pressure demonstrated improvement at an average follow-up of 22 months. CONCLUSION: As in the syndromic patient, PVDO is a safe and, in the short-term, effective modality for cranial vault expansion in the nonsyndromic patient. The benefits and favorable perioperative profile of PVDO may therefore be extended to patient populations other than those with syndromic craniosynostosis.