A Comparison of Weight Gain Following Mandibular Distraction Osteogenesis and Tongue-Lip Adhesion in the Treatment of Robin Sequence.

ABSTRACT: Obstructive respiratory stress and feeding difficulties in infants with Robin sequence (RS) may result in poor weight gain or loss. Following failure of conservative treatment, surgical options include mandibular distraction osteogenesis (MDO) and tongue-lip adhesion (TLA). Whilst both techniques have demonstrated to improve airway patency and feeding behavior, an advantage of either in restoring weight growth remains unknown. This study aimed to improve procedural selection by examining weight gain following MDO and TLA. A retrospective chart review was performed for 17 RS patients that had undergone MDO and 25 that had received TLA. The mean body weight in both groups was below the 50th population percentile at birth and fell further in the period before surgery. A mixed model analysis demonstrated that postoperative weight gain depended on the progression of time and preoperative weight. Conversely, biological sex, congenital comorbidities, method of feeding, the respective cleft team, and the type of surgery did not significantly influence the evolution of postoperative body weight. In conclusion, both MDO and TLA were able to restore weight growth in infants affected by RS, though a clear superiority of either technique could not be established.

Atypical Outcomes of Nasal and Lip Appearance After Unilateral Cleft Lip Repair: Judgment by Professionals, Patients, and Laypeople.

OBJECTIVE: To gain more insight into the assessment of "atypical" nasal and lip appearance outcomes compared to "typical" appearance outcomes after unilateral cleft lip and palate (UCLP) repair, when judged by professionals, patients with repaired UCLP, and laypeople. DESIGN: An online survey containing 3 series of photographs with various degrees of "typical" and "atypical" nasal and lip appearance outcomes after UCLP repair was sent to 30 professionals, 30 patients with repaired UCLP, and 50 laypeople in 2 countries. Participants were instructed to rank the photographs from excellent to poor based on overall appearance. Mean rank positions of photographs were analyzed and differences in mean rank score between "typical" and "atypical" results were assessed using a T-test. Agreement of ranking between the 3 groups was assessed with an analysis of variance analysis. SETTING: Amsterdam UMC, location VUmc, Netherlands and Boston Children's Hospital, Boston, USA. PATIENTS: Photographs of 6- to 18-year-old patients with repaired UCLP. RESULTS: "Atypical" appearance outcomes were ranked significantly less favorably (small nostril: P = 0.00; low vermillion border: P = 0.02; whistling deformity: P = 0.00) compared to "typical" outcomes. Difference between professionals, patients and laypeople in rank positioning the photographs was not statistically significant (P = 0.89). CONCLUSIONS: Noses with a smaller nostril and lips containing a whistling deformity were perceived as poorer outcome compared to the "typical" results. Professionals, patients, and laypeople are in agreement when assessing these outcomes.

Development of the Submental Nasal Appearance Scale for the Assessment of Repaired Unilateral Complete Cleft Lip: A Pilot Study.

OBJECTIVE: To develop the "Submental Nasal Appearance Scale" (SNAS), which is an easy-to-use objectified tool to represent a cleft surgeon's standard for assessment of the nasal appearance from the submental perspective. DESIGN: Eighty-five photographs of patients with unilateral complete cleft lip and palate were selected and cropped, displaying the submental view. Sixty-one photographs were used to develop 5 sets of reference photographs. Three cleft surgeons graded 24 photographs with these sets and subjectively graded the overall nasal appearance as well. Internal agreement for both methods was calculated, as well as correlation between them. The SNAS was created, by only using the combination of sets that showed the highest reliability and correlation. SETTING: Boston Children's Hospital, Boston, Massachusetts. PATIENTS: Six- to 9-year-old patients with unilateral complete cleft lip and palate. RESULTS: The intrarater and interrater reliability was 0.84 and 0.79, respectively, for the SNAS and 0.76 and 0.62, respectively, for the overall appearance assessment. The correlation was 0.74 between the methods. CONCLUSIONS: The SNAS is a reliable tool that reflects a cleft surgeon's standard and could be used independently or in combination with existing rating scales using the frontal and/or lateral view, for assessment after cleft lip repair.

Permanent tooth agenesis in non-syndromic Robin sequence and cleft palate: prevalence and patterns.

OBJECTIVES: Partial tooth agenesis is frequently observed in Robin sequence. Tooth anomalies are increasingly considered as an extended phenotype of the cleft palate population. The study objective was to compare the prevalence and patterns of tooth agenesis in a group of patients with non-syndromic Robin sequence (ns-RS) and a group with non-syndromic cleft palate (ns-CP). MATERIALS AND METHODS: The panoramic radiographs of 115 ns-RS and 191 ns-CP patients were assessed for agenesis of the permanent dentition (excluding third molars) and the patterns recorded using the Tooth Agenesis Code. RESULTS: Partial tooth agenesis was observed in 47.8% of ns-RS and 29.8% of ns-CP patients with a greater prevalence in the mandibula than in the maxilla, particularly in ns-RS. The teeth most frequently absent in both groups were the mandibular second premolars and maxillary lateral incisors. Tooth agenesis was bilateral in two-thirds of affected ns-RS patients and one-half of ns-CP patients. In ns-RS, bilateral agenesis of the mandibular second premolars was more frequently observed in female than that in male patients. Completely symmetrical patterns of hypodontia were found in around 45% of ns-RS patients with tooth agenesis compared to 35% in ns-CP. No association was found between the extent of the palatal cleft and the severity of hypodontia. CONCLUSION: Tooth agenesis is more prevalent in ns-RS than that in ns-CP, demonstrates a much greater predilection for the mandible in ns-RS, and bears no relation to the extent of the palatal cleft. CLINICAL RELEVANCE: When compared to ns-CP, additional developmental disturbances are likely involved in the etiology of tooth agenesis in ns-RS. Future research could help identify the underlying genetic traits and aid in classifying patients in those with and without expected tooth agenesis in order to facilitate orthodontic management strategies.

Clinical Evaluation of the Articulinx Intercarpometacarpal Cushion for the First CMC Joint: A Feasibility Study.

Background The Intercarpometacarpal Cushion (ICMC; Articulinx, Cupertino, CA, USA) is an implantable spacer designed as a less invasive surgical treatment for osteoarthritis (OA) of the first carpometacarpal joint (CMC-1). Description of Technique Following local anesthesia and exposure of the joint capsule the ICMC, attached to a needle and suture tethers, is guided into the joint space under fluoroscopic visualization through a dorsal approach. The needle is pulled through the thenar eminence to the opposite side of the hand and, once proper device placement is confirmed, cut free and the joint capsule closed. Patients and Methods Eight female patients (median age 56 years; range, 42-83) were treated and followed for 6 to 24 months. Safety of the implant procedure was evaluated intraoperatively. Pain, joint function, and strength were evaluated at 6 weeks, 3, 6, 12 and 24 months with a Visual Analog Scale (VAS) for pain, the QuickDASH inventory, Canadian Occupational Performance Measure (COPM), and pinch and grip strength measurements. Results At 2 years (n = 6), mean VAS pain scores decreased from 6.3 (± 1.5) to 2.2 (± 1.1) (p < 0.001), mean QuickDASH scores improved from 47 (± 15) to 31 (± 11) (p < 0.10), mean COPM performance scores improved from 5.0 (± 1.2) to 5.5 ( ± 1.3) (p = NS). Mean pinch and grip strength measurements also improved compared with baseline. No serious adverse events occurred. Two device removals occurred, associated with a traumatic event and Stage IV OA with device displacement, at 6 and 9 months respectively. Conclusion The ICMC can be implanted safely. Effectiveness needs to be confirmed in future studies.