Perioperative Anaesthetics Management of a Girl with Byars-Jurkiewicz Syndrome for Mammoplasty

Abstract

Byars-Jurkiewicz syndrome is a cutaneous- facial -genital disorder characterized by sporadic congenitalmacrogengivae hypertrichosis, massive bilateral enlargement of breast after puberty and Kyposis. It isan extremely rare condition as only four to five patients diagnosed throughout the world till now. MissBithi, aged 12 years was admitted in plastic surgery in BSMMU with hypertrichosis on the face frombirth, huge gingival fibromatosis and bilateral excessive rapid enlargement of breast since last year whichis almost half of her 38 kg body weight. She was scheduled for mammoplasty. No abnormalities detectedin all routine tests. Small mouth opening with hypertrophied gum obscured visibility of the posteriorpharyngeal wall, thyromental distance and cervical spine mobility was found normal. General anesthesiawas planned with anticipation of profuse blood loss during procedure and ICU bed was booked for prolongedsurgery, .Difficult airway trolley was kept ready, two wide bore canula were inserted and all routinemonitoring were applied before induction. Laryngoscopic view was equivalent to Cormac Lehan grade 2.Surgery was completed uneventfully except huge bleeding and prolonged duration (8 hours). She wastransferred to ICU, mechanically ventilated overnight and extubated in the next morning and sent thepatient to the ward.

JBSA 2018; 31(1): 45-47