Korean J Anesthesiol.  2010 May;58(5):495-499. 10.4097/kjae.2010.58.5.495.

Anesthesia of a patient with Dubowitz syndrome: A case report

• Lee MK
• Lee YS

• KMID: 1766270
• DOI: http://doi.org/10.4097/kjae.2010.58.5.495

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.

Keyword

Airway management; Congenital anomaly; Dubowitz syndrome

MeSH Terms

Adolescent
Airway Management
Anesthesia
Anesthesia, General
Blepharophimosis
Cleft Palate
Ear
Eczema
Eyebrows
Facies
Growth Disorders
Gynecological Examination
Hair
Humans
Hysterectomy
Intellectual Disability
Microcephaly
Tooth
Eczema
Facies
Growth Disorders
Intellectual Disability
Microcephaly