Russell-Silver syndrome: anaesthetic implications and management.

Correspondence: Dr MD Scarlett, Department of Surgery, Radiology, Anaesthesia and Intensive Care, The University of the West Indies, Kingston 7, Jamaica, West Indies. Fax: 977-6160, e-mail: [email protected] CASE REPORT A 12-year-old boy of height 130 cm and weight 26.9 kg was scheduled for elective right orchidopexy for an undescended right testis and herniotomy for right inguinal hernia. RussellSilver syndrome was diagnosed in early infancy based on the patient’s low birthweight (2.2 Kg), postnatal growth deficiency and associated physical traits. His height and weight were below the third percentile. He also had congenital heart disease (atrial septal defect – ASD), hyperactive airway disease, frequent chest infections and was maintained on salbutamol inhaler. He had frequent hypoglycaemic episodes up to the age of four years, and had one episode of seizurelike activity at age two years, for which continued treatment was not necessary. Physical examination revealed a very asthenic boy with little muscle development and subcutaneous fat, but the left side was more developed. He had a narrow elongated head, small triangular face, low set posterior-rotated ears, hypoplastic mandible, small mouth with down-turned corners and microdontia (small crowded teeth). A Mallampati score of II was given with regards to the ease of tracheal intubation. He had widely spaced nipples, lumber lordosis and scoliosis, a larger left thumb with clinodactyly of the fifth digit of both hands. The right lower limb was shorter by 1.5 cm. The genitalia and scrotum were under-developed with the undescended right testis and the right inguinal hernia. Cardiovascular system examination findings were in keeping with the ASD, which was confirmed by echocardiography and cardiac catheterization. The chest X-ray showed enlarged main pulmonary trunk, but normal pulmonary vessels, lungs and cardiac shadow. The basic haematological and chemical blood tests results were all within normal limits and hypoglycaemia was ruled out. The patient was fasted for six hours prior to surgery and intravenous 5% dextrose in 0.45% saline was administered. No premedication was given but prophylactic antibiotics (intravenous amoxicillin 1.5 g and gentamycin 40 mg) against infective endocarditis were administered one hour prior to surgery. The anaesthetic plan was to avoid tracheal intubation since the surgical procedure was expected to be of short duration and profound muscle relaxation was not needed. Preparations were made for a possible difficulty, if tracheal intubation became necessary. After the application of all monitors and pre-oxygenation, anaesthesia was induced with intravenous propofol (60 mg) and maintained with 2.0L O2/2.0L N2O/1– 2.5% halothane mixture. Spontaneous respiration was maintained via “bag-and-mask” technique and patency of the airway maintained with a size 2 oro-pharynRussell-Silver Syndrome Anaesthetic Implications and Management MD Scarlett1, MW Tha2