Massive intratumor hemorrhage in facial plexiform neurofibroma

✍ Scribed by Tung-Chain Tung; Yu-Ray Chen; Kuo-Ting Chen; Chien-Tzung Chen; Richard Bendor-Samuel

• Publisher: John Wiley and Sons
• Year: 1997
• Tongue: English
• Weight: 432 KB
• Volume: 19
• Category: Article
• ISSN: 1043-3074
• DOI: 10.1002/(sici)1097-0347(199703)19:2<158::aid-hed13>3.0.co;2-9

✦ Synopsis

Background. Plexiform neurofibromatosis is a fea-Recklinghausen's disease (VRD), an autosomal ture of von Recklinghausen's disease. Head and neck lesions may dominant disorder with an incidence of approxiproduce varying degrees of cosmetic and functional deformity. However, life-threatening hemorrhage into facial plexiform neuromately 1 in 3000 live births. 1,2 Head and neck lefibromas has not been previously reported.

sions may present as either asymptomatic

Method: We report two patients with von Recklinghausen's masses which, in the extreme form, are pendudisease who experienced massive hemorrhage into facial neurolous and grotesque (elephantiasis neurofibrofibromas, one following a blunt injury and the other without a known initiating event.

matosa) or as space occupying tumors producing

Results: Conservative management did not stop the hemorpressure effects on the craniofacial bones and unrhage into facial neurofibromas in either instance. Surgical exploderlying structures. They usually appear shortly ration was mandated for hemorrhage control and evacuation of after birth, enlarge during childhood, but are ofblood clots. Hemostasis was attained by a combination of hypotenten not fully developed until adult life. Although sive anesthesia and chromic catgut suture ligatures.

Conclusions: These case reports demonstrate a potentially these tumors have significant neovascularization lethal complication in patients with facial plexiform neurofibroma.

with abnormal vessel wall structure, spontane-

Where a competent and experienced interventional neuroradioloous massive hemorrhage is extremely rare. Neargist is not available, surgical exploration should be undertaken fatal bleeding from a plexiform neurofibroma of to control bleeding.