Orbital malformation with dysplastic infraorbital artery in neurofibromatosis type 1.

PURPOSE: To report a case with neurofibromatosis type 1 presenting as prominent enophthalmos and abnormal infraorbital artery.

CASE DESCRIPTION: A 19-year-old man with a family history of neurofibromatosis presented with prominent right enophthalmos. Computed tomography showed orbital dysplasia and enlarged inferior orbital fissure but no plexiform neurofibroma. Prominent intraoperative hemorrhage originated from several abnormal arteries in the infraorbital region during orbital reconstruction. A tortuous and dysplastic infraorbital artery was verified postoperatively by computed tomography angiography. The bleeding vessels were supposed to be the orbital branches of the dysplastic infraorbital artery.

CONCLUSIONS

The orbital malformation and enlargement of inferior orbital fissure probably resulted in an abnormal infraorbital artery. Selective artery embolization may be chosen as a preceding treatment before orbital reconstruction surgery.