Syndromes Codexery

Binder's syndrome

Rare developmental disorder of nose and jaw.

Binder's syndrome

Binder's syndrome, also called maxillo-nasal dysplasia, is a rare developmental condition that mainly affects the front part of the upper jaw and the nose. Its exact causes are not known. The condition is marked by visible facial differences, which result from underdevelopment of the cartilage in the nasal septum and the premaxilla. In people with this syndrome, the anterior nasal spine is completely missing. There are also related abnormalities in the way the muscles of the upper lip and nasal floor attach, as well as in the cervical spine. Typical features include a flat, underdeveloped midface, a short nose with a low nasal bridge, and an underdeveloped upper jaw. The lower jaw often appears to protrude, with excess vertical growth in the front, leading to a Class III bite (where the lower teeth sit in front of the upper teeth). The frontal sinus is small, and the overall facial structure is unbalanced.

Early treatment is recommended. This often involves applying backward-to-forward traction to the upper jaw. Around age eight, surgery may be done to reattach the nasolabial muscles to the front edge of the nasal cartilage. For severe cases, many individuals choose plastic surgery or orthodontic treatment to improve appearance.

Field
Medical genetics, craniofacial disorders
Known for
Maxillo-nasal dysplasia with hypoplasia of nasal septum and premaxilla
Type
Developmental disorder
Rarity
Rare

Lore & Background

Binder's syndrome is a developmental disorder that primarily affects the anterior part of the maxilla and nasal complex. The causes remain unclear, and it is considered a rare condition. The syndrome involves hypoplasia of variable severity of the cartilaginous nasal septum and premaxilla, including complete total absence of the anterior nasal spine. Associated anomalies include abnormal muscle insertions of the upper lip and nasal floor, as well as cervical spine involvement.

Reader's Guide

Binder's syndrome is significant as a rare craniofacial disorder with distinct morphological features. Its legacy lies in the specific pattern of midfacial hypoplasia, including a flat nasal bridge, short nose, underdeveloped upper jaw, and Class III skeletal profile with reverse overjet. Treatment is encouraged as early as possible, involving posteroanterior traction on the maxilla and, around age 8, reinsertion of nasolabial muscles. Severe cases often undergo plastic surgery or orthodontic treatment for cosmetic reasons. The syndrome's uncertain etiology and need for multidisciplinary management continue to inform clinical practice.

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