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.

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.

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.