Large Vertex Encephaloceles: Management and Outcomes.

BACKGROUND: Complex vertex and posterior encephaloceles containing brain tissue have uncertain prognosis and high operative risk. Patients may not be offered operative intervention depending on local and regional specialist expertise. The authors present their experience treating 5 such pediatric patients. METHODS: This is a retrospective review of the surgical assessment, planning, and technique of cranial repairs, as well as surgical outcomes and developmental follow-up regarding adaptive functioning for patients presenting for second opinion for encephalocele of the cranial vertex after having been deemed too high risk at another institution. RESULTS: Five consecutive patients presented between January 2014 and June 2016. One patient was not offered repair. Of 4 patients who underwent reconstruction, average age at time of repair was 2.7 months (range, 0.9-6.7). One presented with ruptured encephalocele, whereas the remaining 3 underwent drainage of the encephalocele (average volume of 1200 mL) at time of surgical resection. Operative time averaged 3.7 hours (range, 2.2-5.3). There were no deaths. One patient had a single seizure postoperatively. Two patients required placement of permanent shunt for hydrocephalus. Two patients completed developmental evaluations, both of whom exhibited delays in adaptive functioning relative to same-aged peers. CONCLUSIONS: Patients with large, complex encephalocele warrant evaluation by an experienced high-volume tertiary care pediatric craniofacial center. The decision to proceed with surgical management should include an interdisciplinary team of surgeons, anesthesiologists, neurologists, and social work. Further study of developmental outcomes in both operated and unoperated patients is necessary to better understand risks and benefits of reconstruction.

Perioperative Pain Management After Primary Palate Repair: A 3-Surgeon Retrospective Study.

OBJECTIVE: Pain management strategies following palatoplasty vary substantially. Despite efforts to reduce narcotic utilization, specific analgesic regimens are typically guided by surgeon preference. Our aim was to define analgesic variables that affect postoperative narcotic use and time to resumption of oral intake. DESIGN: This is a retrospective review from 2015 to 2018. PATIENTS: Nonsyndromic patients undergoing primary palate repair. MAIN OUTCOMES MEASURES: Analgesic variables included: local anesthetic, pterygopalatine ganglion nerve block, palatal pack, and postoperative use of ketorolac, dexamethasone, and nursing-controlled analgesia (NCA) opioid dosing. Proxy measures for pain included time to resumption of oral intake and morphine equivalence (mg/kg/h) administered. RESULTS: Veau phenotypes for the 111 patients included were: I (28%), II (19%), III (33%), IV (16%), and submucous (4%). Age, weight, local anesthetic, and postoperative use of ketorolac, dexamethasone, and palatal pack had no effect on either proxy measure (P > .05). Postoperative narcotic usage was significantly lower in patients who had an intraoperative suprazygomatic peripheral nerve block and significantly higher when NCA was utilized (P < .05). Neither variable had a significant impact on time to resumption of oral intake (P > .05). CONCLUSION: Several perioperative analgesic strategies lead to comparable postoperative consumption of narcotic and time to resume oral intake. The authors advise careful consideration of NCA due to the potential for increased narcotic utilization that we found in our institution. Based on our promising findings, further studies are warranted to assess risks, benefits, and costs of performing peripheral nerve blocks at the time of palatoplasty.

Cleft Lip and Palate in Infants With Prenatal Opioid Exposure.

OBJECTIVE: This study examines phenotypic presentation and perioperative outcomes of cleft-related procedures for infants with cleft lip and/or palate (CL/P) and prenatal opioid exposure. DESIGN: This is a retrospective review of infants with prenatal opioid exposure treated for CL/P from 2008 to 2018. SETTING: Patients cared for at a tertiary center from 2008 to 2018. PATIENTS/PARTICIPANTS: Eighteen patients with documented prenatal opioid exposure and CL/P had primary repairs in our unit. MAIN OUTCOME MEASURE(S): The phenotypes of CL/P were characterized. Demographic data regarding additional exposures, as well as associated medical and social comorbidities were recorded. Outcome variables included operative delays, perioperative complications, and loss of follow-up. RESULTS: Isolated cleft palate (CP; 67%) was overrepresented among patients with prenatal opioid exposure and CL/P, as was Robin sequence (50% in isolated CP). Fifty-six percent had exposure to additional substances. A majority (67%) had other medical conditions or anomalies, and 17% had known genetic syndromes. Seventy-two percent were in state custody. Thirty-nine percent of exposed patients had delays in their planned operative dates due to medical and/or social factors. There were no postoperative readmissions following cleft procedures. Lack of follow-up was noted in 33% of patients. CONCLUSIONS: Infants with CL/P who have prenatal opioid exposure are likely to have additional medical conditions and complex social challenges.

Cleft Lip and Palate in Ectodermal Dysplasia.

OBJECTIVE: Ectodermal dysplasia (ED) comprises multiple syndromes that affect skin, hair, nails, and teeth, and sometimes are associated with orofacial clefting. The purpose of this study is to (1) identify the prevalence and characteristics of cleft lip and/or palate (CL/P) in patients with ED and (2) describe the management and outcomes. DESIGN: Retrospective review from 1990 to 2019. PATIENTS: All patients with ED treated at Boston Children's Hospital. MAIN OUTCOMES MEASURES: Prevalence of CL/P was calculated and clinical details recorded: phenotypic anomalies, cleft type, operative treatment, and results of repair. RESULTS: Of 170 patients with a purported diagnosis of ED, 24 (14%) had CL/P. Anatomic categories were bilateral CL/P (67%), unilateral CL/P (8%), and cleft palate only (25%). The most common ED syndrome (37%) was ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC). Pathogenic variants in TP63 were the most frequent finding in the 11 patients who had genetic testing. Aberrations from a typical clinical course included failure of presurgical dentofacial orthopedics, dehiscence of nasolabial adhesion, and total palatal absence requiring free-flap construction. Two patients had prolonged postoperative admission for respiratory infection. High fistula (8%) and velopharyngeal insufficiency (33%) rates reflected the predominance of bilateral complete forms. CONCLUSIONS: As in other types of syndromic CL/P, cleft phenotypic expression in ED is more severe than the general cleft population. Further studies are needed to correlate genotype and phenotype for the distinct syndromes included in the ED spectrum.

Does the Nostril Shape Change After Le Fort I Advancement in Patients With Unilateral Complete Cleft Lip?

PURPOSE: Patients with unilateral cleft lip and palate (UCLP) may require Le Fort I advancement to correct maxillary hypoplasia after reaching skeletal maturity. The underlying cleft anatomy, previous operations, and scarring can affect nostril changes after maxillary advancement. The purpose of the present study was to determine whether Le Fort I advancement affects the nostril configuration (ie, width, axis, shape) in patients with UCLP. The specific aims were to (1) compare cleft and noncleft nostrils in patients with UCLP after maxillary advancement and (2) compare the changes in nostril configuration in patients with UCLP with those in noncleft controls after Le Fort advancement. PATIENTS AND METHODS: A retrospective case-control study of nonsyndromic, skeletally mature patients with UCLP and a case-matched control group without UCLP who had undergone single-piece Le Fort I advancement with alar cinch suture from 2010 to 2014. Patients were included if they had undergone pre- and postoperative 3-dimensional photogrammetry without intervening nasal revision. Three-dimensional anthropometry was used to evaluate changes in nostril axis and width, soft triangle angle, columellar show, and nasal width after orthognathic correction. RESULTS: The present study included 19 patients with UCLP (11 males; mean age, 18.0 years) and 19 noncleft controls (11 males; mean age, 18.7 years; P = .276). The mean sagittal advancement in the patients with UCLP and noncleft controls was 7.5 mm and 6.3 mm, respectively (P = .143). On average, the nostrils widened, the soft triangles flattened, and the columellar show increased. No significant difference was found in the changes to the nostril configuration between the cleft and noncleft sides in the patients with UCLP. No significant differences were found in the nostril changes between patients with UCLP and noncleft controls. CONCLUSIONS: Baseline nostril asymmetry is not altered by Le Fort osteotomy in patients with UCLP because both nostrils respond similarly to the deforming forces of maxillary advancement. Similarly, no differences were found in the nostril changes between the cleft and noncleft controls. These findings can aid proper surgical planning for cleft nasal revisions.

Cost and Satisfaction Implications of Using Telehealth for Plagiocephaly.

Patients with deformational plagiocephaly are often referred for evaluation by a plastic surgeon. During the early COVID-19 pandemic, visits were performed predominantly via telehealth. This study compares costs, satisfaction, and technological considerations for telehealth and in-person consultations for plagiocephaly. Methods: This prospective study evaluated telehealth and in-person consultation for plagiocephaly between August 2020 and January 2021. Costs were estimated using time-driven activity-based costing (TDABC) and included personnel and facility costs. Patient-borne expenses for travel were assessed. Post-visit questionnaires administered to patients' families and providers measured satisfaction with the consult and technical issues encountered. Results: Costing analysis was performed on 20 telehealth and 11 in-person consults. Median total personnel and facility costs of providing in-person or telehealth consults were comparable (P > 0.05). Telehealth visits saved on the cost of clinic space but required significantly more of the provider's time (P < 0.05). In-person visits had an additional patient-borne travel cost of $28.64. Technical difficulties were reported among 25% (n = 5) of telehealth consults. Paired provider and patient experience questionnaires were collected from 17 consults (11 telehealth, six in-person). Overall satisfaction with care did not differ significantly between consult types or between the provider and patient family (P > 0.05). Conclusions: Costs of providing in-person and telehealth plagiocephaly consultations were comparable, whereas patients incur greater costs when coming in person. Practices that treat patients with plagiocephaly may wish to consider expanding their virtual consult offerings to families desiring this option. Long-term outcome studies are necessary to evaluate the efficacy of both visit types.

Philtral Ridge Projection in Repaired Unilateral Cleft Lip: Three-Dimensional Anthropometry.

BACKGROUND: Cleft surgeons try to construct a philtral ridge during primary repair of a cleft lip, but rarely document the results. The authors used three-dimensional photogrammetry to measure projection of philtral ridges after closure of the common forms of unilateral labial cleft. METHODS: This is a retrospective study of patients with unilateral complete, incomplete (moderate and severe), and microform cleft lip repaired by one surgeon from 2000 to 2013. Cleft type determined the technique for building a philtral ridge. The relative elevation of the ridge on the cleft versus noncleft side was measured on three-dimensional childhood photographs at two locations along the philtrum: just above the Cupid's bow and at the midlabial level. RESULTS: Thirty-four patients were evaluated at a mean age of 9.25 years. All cleft types exhibited greater projection at the philtral midlabial level compared with the Cupid's bow level. The authors found a trend toward a more prominent cleft side philtral ridge in microforms. In incomplete cleft lips, there was slightly greater philtral ridge projection in severe forms repaired after a preliminary nasolabial adhesion compared with those repaired in a single stage. There was similar projection of the cleft side ridge in two-stage complete and single-stage repaired incomplete lips. CONCLUSIONS: It is possible to construct a philtral ridge that is as prominent as the noncleft side in all types of unilateral cleft lip. Increased muscle thickness may explain the slightly increased philtral ridge projection in patients with an incomplete cleft repaired in two stages compared to one-stage closure. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Evaluating Surgical Decision-making in Nonsyndromic Sagittal Craniosynostosis Using a Digital 3D Model.

Surgical correction of craniosynostosis addresses potentially elevated intracranial pressure and the cranial deformity. In nonsyndromic sagittal synostosis, approximately 15% of patients have elevated intracranial pressure. The decision to operate therefore likely reflects a combination of aesthetic goals, prevention of brain growth restriction over time, surgeon training and experience, and parental expectations. This study examines clinical factors that influence surgical decision-making in nonsyndromic sagittal synostosis. METHODS: An online survey sent to craniofacial and neurosurgeons presented 5 theoretical patients with varying severities of sagittal synostosis. For each cephalic index, 4 separate clinical scenarios were presented to assess influences of parental concern and developmental delay on the decision to operate. RESULTS: Fifty-six surveys were completed (response rate = 28%). Participants were predominantly from North America (57%), had over 10 years of experience (75%), and performed over 20 craniosynostosis procedures annually (50%). Thirty percent of respondents indicated they would operate regardless of head shape and without clinical and/or parental concern. Head shape was the greatest predictor of decision to operate (P < 0.001). Parental concern and developmental delay were independently associated with decision to operate (P < 0.001). Surgeons with more experience were also more likely to operate across all phenotypes (OR: 2.69, P < 0.004). CONCLUSIONS: Surgeons responding to this survey were more strongly compelled to operate on children with nonsyndromic sagittal craniosynostosis when head shape was more severe. Additional factors, including parental concern and developmental delay, also influence the decision to operate, especially for moderate phenotypes. Geographic and subspecialty variations were not significant.

Time-Driven, Activity-Based Costing of Presurgical Infant Orthopedics: A Critical Component of Establishing Value of Latham Appliance and Nasoalveolar Molding.

BACKGROUND: Value-based health-care reform requires assessment of outcomes and costs of medical interventions. In cleft care, presurgical infant orthopedics is still being evaluated for clinical benefits and risks; however, the cost of these procedures has been largely ignored. This study uses robust accounting methods to quantify the cost of providing two types of presurgical infant orthopedics: Latham appliance treatment and nasoalveolar molding. METHODS: This is a prospective study of patients with nonsyndromic cleft lip and/or palate who underwent treatment with presurgical infant orthopedics from 2017 to 2019 at two academic centers. Costs were measured using time-driven activity-based costing. Personnel costs, facility costs (operating room, clinic, and inpatient ward), and equipment costs were included. Travel expenses were incorporated as an estimate of direct costs borne by the family, but indirect costs (e.g., time off from work) were not considered. RESULTS: Twenty-three patients were treated with Latham appliance treatment and 14 were treated with nasoalveolar molding. For Latham appliance treatment, average total cost was $7553 per patient ($1041 for personnel, $637 for equipment, $4871 for facility, and $1004 for travel over 6.5 visits). Unilateral and bilateral costs were $6891 and $8860, respectively. For nasoalveolar molding, average cost totaled $2541 ($364 for personnel, $151 for equipment, $300 for facility, and $1726 for travel over 13 visits); $2120 for unilateral and $3048 for bilateral treatment. CONCLUSIONS: The major difference in cost is attributable to operative placement of the Latham device. Travel cost for nasoalveolar molding is often higher because of frequent clinical encounters required. Future investigation should focus on whether outcomes achieved by presurgical infant orthopedics justify the $2100 to $8900 expenditure for these adjunctive procedures.