Syndromes/Craniofacial Anomalies
Hermansky-Pudlak Syndrome (HPS) is characterized by oculocutaneous albinism and bleeding diathesis. Eleven types have been identified and types 1 and 4 are the most severe forms of the disorder. Types 1, 2, and 4 are the only types associated with pulmonary fibrosis. The common types in Puerto Rico are 1 and 3, and the prevalence of HPS-1 in PR constitutes 50% of the cases worldwide. It is estimated that 1 in 1,800 Puerto Ricans are affected by HPS-1. Patients face risk of bleeding during dental procedures. This case report presents the management of a 6-year-old male with HPS-3, oculocutaneous hypopigmentation, lateral nystagmus and iron deficiency anemia before, during and after receiving dental treatment under general anesthesia due to multiple caries. Tranexamic acid was used as a premedication to stabilize the blood clots and reduce bleeding from oral mucosa. Multidisciplinary approach is important when treating a patient with a hematological condition.