Anesthesia of a patient with Dubowitz syndrome -A case report-

Abstract

Dubowitz syndrome is a rare autosomal recessive disorder that leads to growth retardation (intrauterine, postnatal), mental retardation, a peculiar face, microcephaly, behavioral problems and eczema. The peculiar face of individuals with Dubowitz syndrome includes sparse hair and eyebrows, low-set ears, blepharophimosis, bilateral ptosis, a flat nasal bridge with a broad nasal root and micrognathia. Airway management of such individuals might be difficult due to craniofacial anomalies, such as micrognathia, cleft palate, tooth problems and craniocervical anomalies. In addition, anesthetic management may be complicated by other systemic illnesses. We report the uneventful anesthetic management of a 16-year-old girl with Dubowitz syndrome who underwent a total abdominal hysterectomy after a pelvic examination under general anesthesia. We report this case of Dubowitz syndrome with a review of the relevant literature.

Keywords: Airway management; Congenital anomaly; Dubowitz syndrome.

Fig. 1

The picture shows characteristic facial morphology of the patient with Dubowitz syndrome (microcephaly, sparse hair and eyebrows, sloping forehead, low-set ear, flat nasal bridge, micrognathia).

Fig. 2

The picture shows chronic eczema in hands and foots of the patient with Dubowitz syndrome.