ABSTRACT
A deletion 8p syndrome is a relatively uncommon congenital disease characterized by mental retardation associated with multiple malformation that make anesthetic management a challenge. Anesthetic management of a patient with deletion 8p syndrome may pose a serious problem mainly from difficult tracheal intubation, aspiration complication and cardiac malformation. We experienced a case of 10 year-old boy with a deletion 8p syndrome who underwent appendectomy under the general anesthesia. Intubation was performed by video glidescope after unsuccessful attempt with Macintosh laryngoscope. A high arched palate, short neck, poor patient cooperation due to mental retardation and occasional autistic behaviour made airway management difficult. This case should alert anesthesiologists to the greater difficulties of managing patients with deletion 8p syndrome.
Subject(s)
Humans , Airway Management , Anesthesia, General , Appendectomy , Chromosome Deletion , Chromosomes, Human, Pair 8 , Intellectual Disability , Intubation , Laryngoscopes , Neck , Palate , Patient ComplianceABSTRACT
A male baby with intrauterine growth retardation had a short neck, small hands and feet, hypospadia, both grade I hydronephrosis, type II atrial septal defect, and moderate valvular pulmonary stenosis. The routine chromosome and banding analyses revealed a 46,XY,rec(8)del(8)(p21)dup(8) (q24.1)inv(8)(p21q24.1)pat chromosome constitution. His mother has normal chromosomes, but the father had 46,XY,inv(8)(p21q24.1). Also his uncle had an inv(8) chromosome constitution. We used lymphocytes and examined 40 mitotic cells. All mitotic cells showed deletion of 8p21-->pter and duplication of 8q24.1-->qter. Because 8p21 involves secretion of macrophage and lymphocyte against cancer cells, long-term follow-up for cancer will be needed.