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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
PubMed
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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.

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  • 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.