CODAS Syndrome

A very rare syndrome combining multiple malformations involving head and skeleton. CODAS is an acronym for cerebral ocular dental auricular skeletal anomalies.

Only three cases have been reported. Inheritance is undetermined; all three cases appear to be sporadic. Two girls and one boy have been affected, two of them are of Mennonite ancestry. Consanguinity was not reported in any of the cases.

All laboratory investigations including karyotype, metabolic screening, and studies of cholesterol biosynthesis and peroxisomes yielded normal results, and the underlying defect has not been identified. However, the features of the disease suggest that a collagen gene defect is involved. Clinical signs may involve the head and neck (microcephaly, overfolded ears with dysplastic helices, flat nose with vertically grooved nasal tip, short philtrum, abnormal enamel projections, gingiva hypertrophy), the central nervous system (developmental delay), the eyes (congenital cataracts, ptosis, nystagmus), the skeleton (delayed ossification of upper and lower extremities, odontoid hypoplasia, deformed vertebral bodies with coronal clefts, scoliosis, radiologic signs of spondyloepiphyseal dysplasia, generalized abnormalities of the ilium, joint dislocations, hypoplastic pectoral muscles, generalized hypotonia, restricted pronation and supination), and the skin (alopecia).

Anesthesia in this condition has not been described. Odontoid abnormalities may be associated with instability of the cervical spine. Ascertain stability of the cervical spine both clinically and radiologically, but also assess the range of motion of the cervical spine (other vertebral anomalies). Perform direct laryngoscopy and tracheal intubation either with in-line stabilization or primarily with the fiberoptic bronchoscope. Careful intraoperative positioning is required to prevent joint dislocations. Developmental delay may limit patient cooperation. Sedative and/or anxiolytic premedication and the presence of the primary caregiver during induction of anesthesia may be helpful.