초록
저자는 후기 영아형 이염성 백질 이영양증으로 인해 신경학적인 퇴행이 관찰되고 섭식 장애로 인한 다발성 우식을 보이는 환아를 전신마취 하 치료하였기에 문헌 고찰과 함께 보고하는 바이다. 저작기능 장애로 인한 영양 결핍으로 환아는 신체 발육이 매우 저하되었으며 긴 식사 시간으로 다발성 치아 우식에 이환된 상태였다. 협조도 부족 및 치료 범위 등을 고려하여 전신마취 하에 치과 치료를 시행하였고 정기적인 검진을 시행 중이다. 진행성, 신경성 퇴행질환의 특징을 이해하고 환아의 상태를 고려한 구강 건강 관리 및 보호자 교육을 제공하는 것이 필요할 것으로 사료된다.
Metachromatic leukodystrophy (MLD) is a progressive and degenerative neurological disease caused by a deficiency of the catabolic enzyme arylsulfatase A. Deficiency of arylsulfatase A results in accumulation of sulfatide in the white matter of the peripheral and central nervous system and it occurs demyelination as a result. The patient gradually goes through mental and motor failure. General symptoms of MLD include gait disturbance, mental deterioration, muscle rigidity and impaired swallowing. Inheritance of the disease is autosomal recessive. We report a dental caries treatment of a 3-year old boy with MLD. The patient underwent hematopoietic stem cell transplantation (HSCT) to slow the progression of the disease. He was suffered from difficulties of mastication and swallowing from the degenerative neurological symptom. He was ingesting food by both oral feeding and tubal feeding after he took percutaneous endoscopic gastrostomy (PEG). The cause of multiple caries was mainly presumed as patient's prolonged time of meal. The treatment was performed under general anesthesia considering patient's incompliance. Severely affected lower primary molars were treated with pulp treatment and restored with stainless steel crown. Others were restored with composite resin. There were no postoperative complications. MLD is life threatening progressive disease and also has an impact on unfavorable condition for oral health. Routine home oral care and periodic professional dental care should be emphasized to the caregiver of patient considering the susceptibility of dental caries. Not only the medical care, but periodic dental office visit would benefit the quality of life of the patient.
