Nasal Bifidity: An Unusual Pediatric Congenital Anomaly and Review of the Literature.

Congenital nasal anomalies are rare, with an estimated incidence of 1/20,000 to 40,000 live births. Hyperplasia and duplication anomalies are the most uncommon, comprising about 1% of reported cases. The authors present the case of a 6-year-old girl who presented to our institution with an isolated congenital bifid nasal septum. Parents reported a visibly abnormal nose since birth, and it had been continuously monitored by the parents and pediatrician. She demonstrated no history of difficulty breathing or other nasal concerns and was otherwise growing and developing normally. On physical examination, she was breathing comfortably through her bilateral nasal airways. Her nasal examination revealed a widened mid-vault with deep dorsal grooving and a bifid tip. Magnetic resonance imaging demonstrated an isolated bifid nasal septum without other facial malformation or intracranial extension. She underwent an open septorhinoplasty. Intraoperatively, the authors identified an anomalous dorsal nasal bone extension with a resultant bifidity in the body and caudal portions of the septum. Ostectomy and cartilaginous repositioning allowed for an autogenous reconstruction without the need for grafting. She subsequently recovered well without postoperative complications and continues to have improved nasal appearance with maintenance of function. A review of recent literatures revealed 2 other cases that are similar in presentation. The authors proposed that embryologically there might have been a change in expression of bone morphogenetic protein in the frontonasal area leading to caudal extension of the nasal bone. This in turn interferes with the fusion of nasal septum resulting in the bifid septum and dual dome morphology.

Pleomorphic Adenoma of the Eyebrow: An Unexpected Finding and Review of Unusual Periorbital Soft Tissue Lesions.

ABSTRACT: Soft tissue lesions of the eyebrow are common and are usually diagnosed as dermoid cysts, pilomatrixomas, inclusion cysts, or basal cell carcinomas. Pleomorphic adenomas are benign glandular lesions most commonly found in the parotid gland or salivary glands, and less commonly found in the lacrimal gland. Here the authors report the case of a 35-year-old male who presented with a prominent soft tissue lesion of his right upper brow. This had been present for several months and had not substantially changed in size. Operative excision was performed via a supratarsal crease incision. The mass was located in a deep, periosteal plane, similar to a dermoid cyst. Final pathology demonstrated a 1.1 × 1.0 × 0.9 cm pleomorphic adenoma without defining characteristics of either the lacrimal gland or the minor adnexal glands of the skin or subcutaneous tissue. The authors reviewed the current literature on unusual lesions of the periorbital and brow regions, and the authors present aggregate data regarding epidemiology, diagnosis, and management.

Orthotic Management of Deformational Plagiocephaly: Consensus Clinical Standards of Care.

OBJECTIVE: To establish consensus on definitive, actionable standards for the management of deformational plagiocephaly. DESIGN: Three-stage Delphi Survey process based on best practice statements obtained through literature review. SETTING: Electronic survey delivery. PARTICIPANTS: Review panel of 10 multidisciplinary subject matter experts (SMEs); survey panel of 30 cranial orthotists. RESULTS: Fifty-four best practice statements were accepted in four categories: diagnosis, presentation and severity, initiating treatment, and management principles. CONCLUSIONS: Clinical practice can be guided en route to robust evidence as to the efficacy of various plagiocephaly management strategies, in pursuit of definitive standards.

Clinical outcomes of the Furlow Z-plasty for primary cleft palate repair.

OBJECTIVE: To review the clinical outcomes following the Furlow Z-plasty for primary cleft palate repair. The primary objective was to determine if the presence of an associated sequence or syndrome (i.e., Pierre Robin sequence), age at palate repair, cleft type, or surgeon experience influenced speech outcomes after a Furlow Z-plasty. DESIGN: The outcomes of 140 patients who underwent palate repair were analyzed retrospectively. Speech evaluations were performed to score the severity of hypernasality, nasal escape, articulation errors, and velopharyngeal insufficiency. RESULTS: The mean age at latest evaluation was 4 years 9 months (age range 2 years old to 12 years old and 4 months). Of the 140 patients, 83% had no evidence of hypernasality, 91% had no presence of nasal escape, and 69% had no articulation errors. Overall, 84% of patients had no evidence of velopharyngeal insufficiency. Secondary posterior pharyngeal flap to correct velopharyngeal insufficiency was required in only 2.1% of patients. The formation of an oronasal fistula occurred in only 3.6% of patients. Nonsyndromic patients with Pierre Robin sequence and syndromic patients did just as well as purely nonsyndromic patients in terms of velopharyngeal insufficiency, hypernasality, and nasal escape. Syndromic patients were more likely to make mild-to-moderate articulation errors. In addition, age at palate repair, cleft type, and surgeon experience had no statistically significant effect on speech results. CONCLUSIONS: The Furlow Z-plasty yielded excellent speech results in our patient population with minimal and acceptable rates of fistula formation, velopharyngeal insufficiency, and the need for additional corrective surgery.

Midmaxillary internal distraction osteogenesis: ideal surgery for the mature cleft patient.

BACKGROUND: Distraction osteogenesis is a recent technique often used for maxillary advancement to correct skeletofacial deformities in cleft patients. Conventional orthognathics falls short of achieving adequate stable results. Osteodistraction with Le Fort I osteotomy may create velopharyngeal incompetence. The authors propose a new technique for distracting only the anterior maxilla to improve aesthetics and occlusion while preserving existing speech patterns, using skeletally fixated intraoral devices. METHODS: Seven patients with cleft lip and palate aged 15 years 11 months to 26 years 5 months were selected. All osteotomies included horizontal anterior maxillary osteotomies created to the first molar and vertical osteotomies created between the second premolar and the first molars. Patients were evaluated preoperatively and postoperatively by a speech pathologist by means of fiberoptic video nasoendoscopy. RESULTS: Absolute bony anteroposterior movement ranged from 8 to 18.0 mm (average, 11.29 mm). Changes in skeletal facial profile (N- A-Pg) demonstrated approximately 18.61 degrees of correction in the conversion toward convex profiles. Patients were advanced to stable class I or mild class II relationships, and open bites were closed without development of hypernasal speech. CONCLUSIONS: This novel technique permits significant anterior movements, allowing dramatic improvements in functional and facial aesthetic outcomes. Furthermore, intraoral appliances allowed greater acceptance in this age group. Disadvantages include complicated orthodontic setup and surgical procedure, as well as the cost of occasionally necessary dental implants. No postoperative relapse was seen at an average follow-up of 33 months. This approach renders comfortable yet effective patient care, yielding optimum results while circumventing shortcomings of conventional techniques.