Oral Rehabilitation in Patient With Hereditary Sensory and Autonomic Neuropathy (HSAN) Type V: Clinical Report

TL;DR

This paper reports on the oral health and treatment of a 9-year-old girl with a rare inherited nerve disorder called HSAN-V.

Contribution

The study provides a detailed clinical report on oral rehabilitation strategies for a pediatric HSAN-V patient.

Findings

HSAN-V can lead to significant oral complications requiring specialized prosthodontic care.

Early intervention and tailored oral rehabilitation can improve quality of life in HSAN-V patients.

Oral lesions and absent corneal reflexes are notable features in this condition.

Abstract

Hereditary sensory and autonomic neuropathies (HSANs) are rare inheritable syndromes of unknown etiology. They typically appear in early childhood and are categorized into six different types based on their symptoms. HSAN-V is characterized by loss of pain and thermal perception, Charcot joints, painless fractures, scoliosis, oral lesions, and absent corneal reflexes. The HSAN-V phenotype has a propensity to progress to autoamputation, varying degrees of hypohidrosis, and moderate hyperactivity and perception delay. In this case report, we focused on oral findings, prosthodontic treatments, and oral rehabilitation of a 9-year-old girl with HSAN type V.

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