Sialorrhea in children with mucopolysaccharidosis type II (a review of several clinical cases)

Abstract

Sialorrhea in children is a complex medical problem and leads to the development of a large number of complications. Sialorrhea also negatively affects the social aspects of the patients’ lives and their families. All this leads to a deterioration in the quality of life both in the child and his relatives. In mucopolysaccharidosis (MPS) type II, the main cause of sialorrhea is pseudobulbar syndrome, which forms gradually as the neurodegenerative process progresses. This can reach a significant degree of manifestation, leading to continuous salivation, the need for constant change of clothes and evacuation of saliva from the mouth and pharynx. One of the most effective modern approaches in the treatment of sialorrhea in children is the use of botulinum toxin type A (BTA) preparations. Unlike cerebral palsy, in which the practice of treating sialorrhea with BTA drugs is already becoming routine, therapy of this problem in patients with MPS is not widespread. This publication presents three clinical cases of successful therapy of chronic sialorrhea in patients with MPS type II. All patients were observed at the National Medical Research Center for Children’s Health of the Russian Federation Ministry of Health for many years. The procedure was performed in all patients according to the recommended standard scheme — injection of the product BTA — Xeomin (international name incobotulinumtoxinA) into the parotid and submandibular salivary glands on both sides under ultrasound control, depending on the child weight. To assess the severity of sialorrhea and its course after injections of Xeomin, “The Drooling Impact Scale” was used. The results of our observations show the use of the drug Xeomin to allow obtaining a significant effect in reducing the severity of sialorrhea in MPS patients type II for at least 3 months. Using this product in the recommended doses and administration regimen, effective and safe treatment can be carried out, which, with repeated injections, can be used to achieve long-term improvement in chronic sialorrhea in children with MPS type II.