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Published on: September 19, 2015
Binderoid complete cleft lip/palate
John B Mulliken1, Ram Burvin, Bonnie L Padwa
1Division of Plastic and Oral Surgery, Craniofacial Centre, Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. john.mulliken@tch.harvard.edu
Plastic and Reconstructive Surgery
|March 7, 2003
Summary
A distinct subset of infants with complete cleft lip/palate exhibit nasolabiomaxillary hypoplasia and orbital hypotelorism. This study defines their features, distinguishing them from holoprosencephalic spectrum disorders.
Area of Science:
- Craniofacial Surgery
- Pediatric Plastic Surgery
- Genetics and Developmental Biology
Background:
- A subset of infants with complete cleft lip/palate present with unique craniofacial anomalies, including nasolabiomaxillary hypoplasia and orbital hypotelorism.
- These features distinguish them from typical cleft presentations and other developmental syndromes.
Purpose of the Study:
- To define the clinical and radiographic characteristics of infants with complete cleft lip/palate, nasolabiomaxillary hypoplasia, and orbital hypotelorism.
- To clarify the classification, terminology, and operative management for this specific patient group.
Main Methods:
- Review of 695 patients with various cleft lip/palate forms.
- Identification and detailed analysis of 15 patients with complete cleft lip/palate, nasolabiomaxillary hypoplasia, and orbital hypotelorism.
- Clinical examination, radiographic analysis (including cephalometry), and review of surgical records.
Main Results:
- 15 patients identified (5% of complete cleft lip/palate cases), with equal distribution between unilateral and bilateral forms; female-to-male ratio of 2:1.
- Consistent features included hypoplastic nasal structures, underdeveloped midface, orbital hypotelorism (intercanthal distance 1.5 SD below normal), maxillary retrusion, and Class III malocclusion.
- Dental anomalies were noted, with absent incisors in unilateral cases and a single-toothed premaxilla in bilateral cases.
Conclusions:
- Patients with complete cleft lip/palate, nasolabiomaxillary hypoplasia, and orbital hypotelorism represent a distinct entity, not part of the holoprosencephalic spectrum or Binder anomaly.
- Proposed term: "cleft lip/palate".
- Early recognition is crucial for parental counseling and requires modifications in surgical and orthodontic management.
