Publication:

Dental management under general anesthesia in a pediatric patient with Netherton syndrome: a case report

Netherton syndrome (NS) is a rare genodermatosis with autosomal recessive inheritance caused by mutations in the serine peptidase inhibitor Kazal type 5 (SPINK5) gene, characterized by the triad of ichthyosiform erythroderma, hair shaft abnormality, and atopic diathesis. A 5-year-old Saudi boy diagnosed with NS is reported. He presented with many food allergies, mild lymphopenia, trichorrhexis invaginata, eczema-like lesions, elevated immunoglobulin E (IgE), and exfoliative erythroderma at birth. Several carious teeth and widespread non-plaque-induced gingival inflammation were among the intraoral findings. Following preoperative multidisciplinary clearance, full dental rehabilitation under general anesthesia was carried out. Prophylactics, composite restorations, stainless steel crowns, and extractions of non-restorable teeth were included as part of the treatment. The patient's recovery from the procedure went smoothly, and both oral comfort and function improved. In conclusion, this case highlights the critical role of multidisciplinary collaboration in the safe delivery of dental rehabilitation under general anesthesia for patients with NS.