Special Health Care Needs
Richa Bhatia, DDS
Pediatric Dental Resident
University of Michigan, Ann Arbor, MI
University of Michigan
Fenton, Michigan, United States
James R. Boynton, DDS, MS
Clinical Professor, Pediatric Dentistry Division Head and Graduate Program Director
University of Michigan Dental School, Ann Arbor, MI
Ann Arbor, Michigan, United States
Hydranencephaly is a congenital post-neurulation disorder affecting the central nervous system during the second trimester of pregnancy. It is characterized by destruction of the cerebral hemispheres resulting in an empty cranial cavity that is replaced with cerebrospinal fluid. Symptoms include hydrocephalus, epilepsy, respiratory failure, and poor psychomotor development, among others. Although the incidence is reported as 1 in 10,000 to 1 in 5,000, it is usually fatal before birth or shortly after, as very few children with this condition survive infancy. Most cases are therapeutically aborted - therefore, literature relating to dental treatment for these patients is limited. This case report details the dental management of a 4-year-old female patient who was consulted by the University of Michigan Pediatric Dentistry Clinic for emergency dental treatment under general anesthesia due to risk of sepsis secondary to a diagnosis of hydranencephaly. This report will discuss the clinical and radiographic dental findings, the importance of timely intervention, the treatment rendered to our patient, including prognosis and outcomes assessment, and demonstrates how coordination of treatment with multiple healthcare providers can help facilitate optimal patient care.