Dental Management of a 14-Year-Old with Cockayne Syndrome under General Anesthesia

Abstract

Cockayne's syndrome is a rare, autosomal recessive disorder characterized clinically by cachectic dwarfism, cutaneous photosensitivity, loss of adipose tissue, mental retardation, skeletal and neurological abnormalities, and pigmentary degeneration of the retina. Dental caries is a common finding. Dental rehabilitation of a 14-year-old male with Cockayne's syndrome is presented.

Anesthesia for a cesarean section on a pregnant patient with Cockayne syndrome: case report

“…Patients have fast progressing abnormalities, and the prognosis is very poor, leading to premature death. 4 On the other hand, CS Type III (Mild or Atypical) is diagnosed later in childhood and patients present mild clinical characteristics, normal intellectual development, growth and reproductive function. No correlation has been shown between genotype and phenotype in CS.…”

“…At the preoperative assessment of CS patients some clinical conditions related to early aging, such as high blood pressure, renal and hepatic dysfunction, progressive cataract, diabetes mellitus and myocardial ischemia can be found. 4 , 5 …”

Perioperative Care of a Pediatric Patient With Cockayne Syndrome

“…There are several perioperative concerns to consider when [11][12][13][14][15][16][17][18][19][20]. As with any anesthetic care, appropriate preoperative preparation begins with a thorough history and physical examination.…”