HATS Syndrome: Hemimaxillary Enlargement, Asymmetry of the Face, Tooth Abnormalities, and Skin Findings
Hemimaxillary enlargement, asymmetry of the face, tooth abnormalities, and skin findings (HATS syndrome) is a rare developmental disorder involving the first and second branchial arches. Physical manifestations may present at birth or during early childhood. Characteristic findings include unilateral abnormalities of the face involving the bones, teeth, gums, and skin. Among the characteristic cutaneous manifestations of HATS syndrome, Becker nevus is the most common. A variety of modalities have been utilized in the treatment of HATS syndrome, but no standardized therapy has been established. We report a case of this rare condition in a 14-year-old adolescent boy.
Practice Points
- Appropriate management and treatment of hemimaxillary enlargement, asymmetry of the face, tooth abnormalities, and skin findings (HATS syndrome) requires a multidisciplinary team including a dermatologist, dentist, radiologist, and orthopedic surgeon.
- Becker nevus is among the most common cutaneous manifestations of HATS syndrome and can be treated effectively with the Q-switched laser or the erbium:YAG laser.
The differential diagnosis includes hemifacial hyperplasia, monostotic fibrous dysplasia, and regional odontodysplasia.1 Little information is available concerning the treatment of patients with this condition.15 The reported treatment modalities include combined surgical and orthodontic treatment of unerupted teeth (premolar/canine), prosthodontic treatment, gingivoplasty, recontouring osteotomy for severe facial asymmetry, and reconstructive jaw surgery.1,6,11,15 Successful treatment of Becker nevi with the Q-switched ruby laser, erbium:YAG laser, and 755-nm alexandrite laser have been reported.22-24
Conclusion
There is a need for continued reporting of cases of HATS syndrome in addition to long-term follow-up to document the natural history of the condition and to establish the appropriate treatment.
