J Korean Med Sci.  2010 Apr;25(4):638-640. 10.3346/jkms.2010.25.4.638.

Transcatheter Embolization of a Ruptured Internal Pudendal Artery Pseudoaneurysm in a Patient with Neurofibromatosis Type 1

• Zhang CW
• Yang ZG
• Xie XD
• Wang CH
• You C
• Li W

• KMID: 1792939
• DOI: http://doi.org/10.3346/jkms.2010.25.4.638

Abstract

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder. Pseudoaneurysms formation and rupture is an unusual complication of neurofibromatosis. To date, pseudoaneurysm of the internal pudendal artery associated with NF-1 has not been reported. In this article, we present a 62-yr-old man with NF-1 suffering from spontaneous hematoma of the perinea and scrotum. A digital substraction angiography disclosed a ruptured pseudoaneurysm of the right internal pudendal artery, which was successfully managed with transcatheter embolization.

Keyword

Neurofibromatoses; Aneurysm, False; Embolization, Therapeutic

MeSH Terms

Aneurysm, False/pathology/*therapy
Angiography, Digital Subtraction
Arteries/*pathology
Embolization, Therapeutic/*methods
Humans
Male
Middle Aged
Neurofibromatosis 1/*complications/pathology
Perineum/blood supply/pathology
Scrotum/blood supply/pathology
Treatment Outcome

Figure

• 

  Fig. 1 A 62-yr-old man with a history of neurofibromatosis type 1. (A) Physical examination revealed multiple neurofibromas all over the body, a large hematoma on the perinea and scrotum regions, the extremely swelled penis and scrotum. (B) Right internal iliac arteriography revealed a ruptured pseudoaneurysm of the right internal pudendal artery. (C) Post-embolization right internal iliac arteriography showed complete occlusion of the pseudoaneurysm with coils.

Reference

1. Li Y, O'Conell P, Breidenbach HH, Cawthon R, Stevens J, Xu G, Neil S, Robertson M, White R, Viskochil D. Genomic organization of the neurofibromatosis 1 gene (NF1). Genomics. 1995. 25:9–18.

2. Crowe FW, Schull WJ, Neel JV. A clinical, pathological and genetic study of multiple neurofibromatosis. 1956. Springfield, IL: Charles C. Thomas;181.

3. Mulvihill JJ, Parry DM, Sherman JL, Pikus A, Kaiser-Kupfer MI, Eldridge R. NIH Conference: Neurofibromatosis 1 (Recklinghausen disease) and Neurofibromatosis 2 (bilateral acoustic neurofibromatosis). An update. Ann Intern Med. 1990. 113:39–52.

4. Tatebe S, Asami F, Shinohara H, Okamoto T, Kuraoka S. Ruptured aneurysm of the subclavian artery in a patient with von Recklinghausen's disease. Circ J. 2005. 69:503–506.

5. Kipfer B, Lardinois D, Triller J, Carrel T. Embolization of a ruptured intercostal artery aneurysm in type I neurofibromatosis. Eur J Cardiothorac Surg. 2001. 19:721–723.

6. Tsutsumi M, Kazekawa K, Tanaka A, Ueno Y, Nomoto Y, Nii K, Haraoka S. Rapid expansion of benign scalp neurofibroma caused by massive intratumoral hemorrhage--case report. Neurol Med Chir (Tokyo). 2002. 42:338–340.

7. Baek SH, Kim JH, Kim JS, Han SB, Cho JS, Yoon YH, Kim L. Recurrent massive subcutaneous hemorrhage in neurofibromatosis type 1: a case report. J Korean Med Sci. 2007. 22:728–730.

8. Saitoh S, Matsuda S. Aneurysms of the major vessels in neurofibromatosis. Arch Orthop Trauma Surg. 1998. 117:110–113.

9. Rosen MP, Greenfield AJ, Walker TG, Grant P, Guben JK, Dubrow J, Bettmann MA, Goldstein I. Arteriogenic impotence: findings in 195 impotent men examined with selective internal pudendal angiography. Young Investigator's Award. Radiology. 1990. 174:1043–1048.