BALIGA ET AL

INT CHIN J DENT

Massive oral hemorrhage secondary to atypical expression of Maffucci’s
syndrome: A case report
Mohan J. Baliga, MDS, Raghu Ram Prasad, MDS, Rajesh Kumar, BP, MDS, andPaul Davies, MDS
Department of Oral & Maxillofacial Surgery, College of Dental Surgery, Kasturba
Medical College, Mangalore, South India
A healthy adult male who had massive oral hemorrhage was diagnosed to have atypical presentation ofMaffucci’s syndrome. Extensive search of the literature confirmed the reports of this syndrome involving
maxillofacial region only in two cases till date. (Int Chin J Dent 2002; 2: 60-62.)
Key words: Maffucci’s syndrome, massive oral hemorrhage.

INTRODUCTION
Maffucci’s syndrome is a congenital disorder that has been described among patients of all races with nosexual predilection.1 It is characterized by the presence of multiple enchondromas and soft tissue angiomas.
The presenting symptom of the patient may be skeletal or vascular.

Skeletal lesions appear as

dyschondroplasias where as soft tissue lesions consist of typical cavernous hemagiomas.2 The visceral
involvement by hemagiomas has been reported in 17% of the cases with Maffucci’s syndrome and only 2cases involving maxillofacial region have been so far found in literature.2,3

CLINICAL REPORT
A 50-year old man was referred from the medical unit to diagnose and manage recurrent massive oral
hemorrhage. History revealed that the patient used to develop massive oral hemorrhage which used to last for
short duration. As the frequency of hemorrhagic attacks increased, the amount of blood loss warranted
admission to the hospital. At the time of referral, the patient had received supportive treatment for thehemorrhage which had occurred 24 hours earlier. Patient was conscious and in a state of hyperdynamic
circulation. The complete blood picture and coagulation profile were found to be normal.
Intra oral examination of the patient revealed diffuse angiomatous lesions extending on both sides of the
tongue involving the ventral surface and lateral border present since childhood (Fig. 1). The lesion was softin consistency and bluish red in color. On examination of the body there was a subcutaneous angioma on theleft side of the chest wall (Fig. 2). Since the patient had experienced recurrent massive oral hemorrhage, andin order to prevent further episodes, elective bilateral lingual artery ligation was done. Biopsy of the intra

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INT CHIN J DENT

oral lesions were taken which confirmed the clinical diagnosis of cavernous hemangiomas.

Fig. 1. Bluish-red colored diffuse angiomas on the ventral surface lateral aspects of the tongue.

Fig. 2. Subcutaneous angioma on the left side of the chest wall.

DISCUSSION
Maffucci described this syndrome characterized by enchondromatosis, bone deformities, hemangiomas
and phlebolithiasis. Kast and Von Recklinghausen described it in 1859. This syndrome has been reviewed
by Canleton, Bean, Anderson, Lewis, and Ketcham.4 This syndrome should not be confused with Ollier’s
disease, Klippel-Trenaunay-Weber syndrome and Proteus syndrome.

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INT CHIN J DENTIn the above reported case, as the angiomas involved large areas of the tongue bilaterally and to prevent
further oral hemorrhage, elective bilateral lingual artery ligation was done. The patient is kept under constantobservation to rule out the possibility of malignant transformation of the lesion.

REFERENCES
1. Damjanov I, Linder J. Anderson’s pathology. 10th ed. St Louis: Mosby; 1990. p. 1712, 2543.
2. Lowell SH, Mathog RH. Head and neck manifestations of Maffucci’s syndrome. Arch Otolaryngol 1979; 105: 427-30.
3. Wolf M, Engelberg S. Recurrent oral bleeding in Maffucci’s syndrome: Report of a case, J Oral Maxillofac Surg 1993; 51:596-7.
4. Gorlin RJ, Cohen MM, Levin SL. Syndromes of the head & Neck 3rd ed. New York: Oxford University Press, Inc.; 1990.
p. 382.
5. Sun TC, Swee G, Shives TC, Unni KK. Chondrosarcoma in Maffucci’s syndrome. J Bone Joint Surg Am 1985; 67:
1214-9.

Reprint request to:
Dr. Mohan J. Baliga
Department of Oral & Maxillofacial Surgery, College of Dental Surgery
Kasturba Medical College
Mangalore 575001, Karnataka, India
TEL: 91-824 492453
E-mail: rbekal9@hotmail.com
Received on April 11, 2001. Revised on October 16, 2001. Accepted on December 22, 2001.
Copyright ©2002 by the Editorial Council of the International Chinese Journal of Dentistry.

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