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Blue rubber-bleb naevus syndrome
British Journal of Oral and Maxillofacial Surgery (1988) 26, 160-164 0 1988 The British Association of Oral and Maxillofacial Surgeons
BLUE R. F. CROSHER,
RUBBER-BLEB
NAEVUS
SYNDROME
C. W. BLACKBURN, M.B., ct,.B., B.D.s., R. C. W. DINSDALE, B.G.D., F.D.S.R.C.S.
B.D.s., F.D.s.R.c.P.s.,
F.D.S.R.C.S.
and
Charles Clifford Dental Hospital,
Wellesley Road, Sheffield, SlO 2SZ
Summary. A case is reported of a patient who presented with facial pain and was found to have phleboliths associated with venous anomalies of the face and neck. Systemic investigation revealed a microcytic, hypochromic anaemia which was the result of haemorrhage from multiple haemangiomas of the gastro-intestinal tract. The finding of anaemia together with cutaneous and intestinal haemangiomas fits with a diagnosis of blue rubber-bleb naevus syndrome.
Introduction Haemangiomas are relatively common lesions in the head and neck region but are only rarely associated with anomalies elsewhere (Watson & McCarthy, 1940; Jones & Mason, 1980). The association of phleboliths with vascular malformation is well established (Gorlin & Goldman, 1970) and there have been a number of reports of haemangiomas with phlebolithiasis (Deighan & Barton, 1956; Smith et al., 1966; Dempsey & Murley, 1970; Faber et al., 1978; Keathley et al., 1983; Ikegami & Nishijima, 1984). Phleboliths form when an intravascular thrombus becomes organised and calcified. Their presence is highly suggestive (if not pathognomonic) of a cavernous haemangioma (Parker & Frommer, 1964). It is important to distinguish phleboliths from other calcific bodies; in the head and neck region, sialography should be used to differentiate between phleboliths and salivary calculi (O’Riordan, 1974). In addition to isolated cutaneous haemangiomas, the lesions may be multiple and represent a more generalised disorder. The association of cutaneous and intestinal haemangiomas was first reported by Gascoyen (1860). Shepherd (1953) gave an extensive review of the angiomatous conditions of the alimentary tract and drew attention to the high incidence of cutaneous lesions occurring with intestinal lesions. Bean (1958) introduced the term blue rubber-bleb naevus to describe the condition of haemangiomas of the skin and gastro-intestinal tract with intestinal bleeding. Case report A 25-year-old Caucasian female presented in May 1981 with a 6 month history of an ache in her right cheek. She associated the ache with a recurrent swelling over her right mandibular ramus. No precipitating or exacerbating factors were identified and the swelling was not related to gustatory stimulus. Three years earlier she had attended her general medical practitioner because of heavy menstrual bleeding. She was found to be anaemic and was treated with oral iron supplements. Her father had died from a brain haemorrhage at the age of 53 (Received Address
for correspondence:
30 December R. F. Crosher,
1986; accepted 31, Storthwood
160
17 February
1987)
Court,
Lane,
Storth
Sheffield,
SlO 3HP
BLUE
RUBBER-BLEB
NAEVUS
SYNDROME
161
years and she has an epileptic sister. There was no other relevant medical or family history. On examination a number of hard nodules were palpable in her right cheek. She had lesions also on the left side of the dorsum of her tongue and dorsum of her left hand which had the clinical appearance of haemangiomas. Radiographs demonstrated the presence of radiopaque bodies in the right and left cheeks (Fig. l), right side of neck (Fig. 2), and apices of both lungs (Fig. 3). A right parotid sialogram was normal; none of these opacities lay within the duct system. Her haemoglobin level was 12.8 g/dl. In April 1982, four of the calcified bodies in her right cheek were removed by an intra-oral approach under endotracheal anaesthesia. Histological examination of one specimen showed an enlarged vascular channel containing a mass of concentrically arranged amorphous eosinophilic material. The ground section appearances of the calcified bodies were those of phleboliths.
Fig. Figure
1-Orthopantogram
showing
a collection
1
of phleboliths face.
(arrowed)
Fig. 2 Figure
2-Postero-anterior
Figure
3-Chest
Fig. 3
radiograph
X-ray
on the right and left sides of
of mandible and the right
showing
phleboliths
showing phleboliths side of neck. (arrowed)
(arrowed)
at the apices
in the right cheek
of the lungs
162
BRITISH
JOURNAL
OF
ORAL
&
MAXILLOFACIAL
SURGERY
Following surgery the patient remained symptom-free until March 1983 when she developed a painful swelling on the right side of her neck. A discreet, firm, bluish nodule which was tender to palpation was visible at the level of the thyroid cartilage (Fig. 4) and a radiograph demonstrated phleboliths in this area (Fig. 5). She was admitted for the exploration of the area under general anaesthesia. Deep to platysma a vascular lesion was found lying in front of the anterior border of the sternomastoid muscle. This lesion appeared to involve the superficial lobe of the submandibular salivary gland and extended to the midline superficial to sternohyoid. A specimen was obtained which comprised a large number of veins and included the right submandibular salivary gland. A number of stones were palpable within the mass. Histological examination of the specimen demonstrated recent thrombi within vessels which showed evidence of endothelial proliferation. Degenerative changes were seen in other vessel walls. A section of the salivary gland block showed a phlebolith associated with a vessel close to normal salivary gland tissue. It appeared from these findings that this patient had multiple angiomatous lesions that were susceptible to the production of thrombi which became organised into phleboliths. She was investigated further in conjunction with a consultant physician. At this time the haematological findings were:Haemoglobin 8.7 g/d1 (Range 11.5-16.5 g/dl) Serum Iron 4 umol/l (Range 9-32 umol/l) TIBC 69 umol/l (Range 4.5-75 umol/l) Serum Ferritin 5 ng/ml (Range 15-130 ng/ml)
Fig. 4 Figure
4-Clinical Figure
photograph 5-Radiograph
Fig. showing
small haemangioma
showing
phleboliths
(‘Blue rubber
5
bleb’) on right side of neck.
at the level of the hyoid
bone.
BLUE
RUBBER-BLEB
NAEVUS
SYNDROME
163
The blood film showed microcytosis and hypochromia. Three specimens of faeces were positive for occult blood. A barium enema showed two small polyps; one at the splenic flexure, the other just proximal to the hepatic flexure. Colonoscopy revealed two polypoid vascular lesions l-2 cm in diameter. One was in the rectum, the second near the splenic flexure. There were at least three smaller lesions in the mid descending colon. Gastroscopy revealed that the stomach and duodenum were normal. Selective arteriography of the mesenteric vessels showed a collection of venous spaces in the region of the hepatic flexure. Venography of the forearm vessels showed widespread tiny varicosities. Cerebral arteriography did not reveal any intracranial vascular anomalies. Our patient was treated conservatively with oral iron supplements. Bowel resection was considered inappropriate because of the widespread distribution of the lesions.
Discussion Blue rubber-bleb naevus syndrome is an uncommon disease in which haemangiomas in the skin are associated with intestinal haemorrhage from similar lesions in the gut (Bean, 1958). In making a diagnosis, blue rubber-bleb naevus syndrome needs to be distinguished from hereditary haemorrhagic telangiectasia. In each there may be iron deficiency resulting from gastro-intestinal bleeding but the skin lesions are The typical lesion of hereditary haemorrhagic telangiectasia is distinctive. punctiform, l-4 mm in diameter, consisting of a tightly woven mat of telangiectatic vessels. The lesions are usually macular but may be papular or spider like. Subungual telangiectases are characteristic and oral and facial lesions are common (Shepherd, 1953). The lesions of the blue rubber-bleb naevus syndrome are larger, usually raised, having ‘the feel and look of rubber nipples’ (Bean, 1958) and never subungual. Hereditary haemorrhagic telangiectasia is inherited as an autosomal dominant trait and the family history is important in the diagnosis yet the mode of transmission of blue rubber-bleb naevus syndrome is not clear. Berlyne and Berlyne, (1960) reported a family in which the condition was transmitted as an autosomal dominant trait through five generations, but this is not a constant finding (Rice & Fischer, 1962) and there is no clear pattern of inheritance in our case. Cutaneous haemangiomas with phlebolithiasis are a feature of both the Maffucci syndrome (Demis et al., 1983) and the Klippel-Trenaunay-Weber syndrome (Stewart & Prescott, 1976). However, the marked skeletal abnormalities of the former and the unilateral limb hypertrophy of the latter distinguish them from the blue rubber-bleb naevus syndrome. Two notable features of our case are the presenting complaint of facial pain and the finding of phleboliths. There is some debate as to whether or not phleboliths cause pain. Deighan and Barton (1956) suggest that phleboliths are painless unless tissues are compressed against them. Dempsey and Murley (1970) propound that vascular congestion or thrombophlebitis may be the cause of pain in haemangiomas. The lesions in the case presented here were both tender on palpation and spontaneously painful. This observation has been recorded in other cases of the blue rubber-bleb naevus syndrome (Fine et al., 1961; Pruzanski, 1963; Fretzin & Potter, 1965). Haemangiomas in the skin and intestine are characteristic of the blue rubberbleb naevus syndrome but other organs also may be affected. In 1962 Rice and Fischer, reported a case in which haemangiomas were found in the liver, lungs and
164
BRITISH
JOURNAL
OF
ORAL
&
MAXILLOFACIAL
SURGEKk
skeletal muscle. The phleboliths seen on the chest X-ray in this case (Fig. 3) can be considered as markers of haemangiomas in the lungs. The iron deficiency anaemia of our patient was the result of bleeding from intestinal haemangiomas and is a typical feature of the blue rubber-bleb naevus syndrome (Bean, 1958). It is therefore recommended that patients presenting with cutaneous or mucosal haemangiomas and found to be anaemic should be investigated for intestinal lesions. Acknowledgements We are indebted to Dr .I. L. Maddocks (Consultant Physician, Royal Hallamshire Hospital) for his help in the diagnosis and management of our patient. Our thanks are due to the Department of Oral Pathology, University of Sheffield; Mr R. Cousins for the photographs; Mrs P. Oldfield and Mrs H. Gibson for typing the manuscript.
References Bean, W. B. (1958). Vuscular Spiders and Related Lesions of the Skin. pp. 178-185. Springfield Illinois: Charles, C. Thomas. Berlyne, G. M. & Berlyne, N. (1960). Anaemia due to ‘blue-rubber-bleb’ naevus disease. The Lance& 2, 1275. Deighan, W. J. & Barton, E. J. (1956). Haemangioma of the buccal pad with phlebolithiasis. Journal of Oral Surgery, 14, 228. Demis, D. .I.: Dobson, R. L. & McGuire, J. (1983). Haemangiomas. Clinical Dermatology 2 (10th Revision) Unit 7-65. Philadelphia: Harper & Row. Dempsey, E. F. & Murley, R. S. (1970). Vascular malformations simulating salivary disease. British Journal of Plastic Surgery, 23, 77. Faber, R. G., Ibrahim, S. Z., St. J. Brew, D. & Hobsley, M. (1978). Vascular malformations of the parotid region. British Journal of Surgery, 65, 171. Fine, R. M., Derbes, V. J. 81 Clark, W. H. (1961). Blue rubber-blcb nevus. Archives OfDermatology, 84, 144. Fretzin, D. F. & Potter, B. (1965). Blue rubber-bleb nevus. Archives of Internal Medicine, 116, 924. Gascoyen, G. G. (1860). Case of naevus involving the parotid gland and causing death from suffocation: Naevi of the viscera. Transactions of the Pathological Society of London, 11, 267. Gorlin, R. J. & Goldman, H. M. (1970). Thoma’s Oral Pathology, (6th Edition) Vol. 2 1031. St. Louis: C. V. Mosby Company. Ikegami, N. & Nishijima, I<. (1984). Haemangioma of the buccal pad with phlebolithiasis: report of a case. Acta Medica Okayama, 38, 79. Jones, J. H. & Mason, D. K. (1980). Oral Manifestations of Systemic DBease. p. 248. LondonPhiladelphia-Toronto: W. B. Saunders Company Ltd. Keathley, C. J., Campbell, R. L. and Isbell, J. W. (19X3). Arteriovenous malformation and associated phleboliths. Oral Surgery, Oral Medicine, Oral Pathology, 56, 132. O’Riordan, B. (1974). Phleboliths and salivary calculi. British Journal of Oral Surgery, 12, 119. Parker, L. A. & Frommer, H. H. (1974). Phleboliths. Report of a case. Oral Surgery, Oral Medicine, Oral Pathology, 18, 476. Pruzanski, W. (1963). Intestinal and cutaneous haemangiomatosis. Report of a case. American Journal of Digestive diseases, 8, 200. Rice, J. S. & Fischer, D. S. (1962). Blue rubber-bleb nevus syndrome. Archives qf Dermatology, 86, 163. Shepherd, J. A. (1953). Angiomatous conditions of the gastro-intestinal tract. Brifish Journal of Surgery, 40, 409. Smith, J. F., Drake, J. & Sollee, N. (1966). Massive oral haemangioma with phlebolithiasis. Oral Surgery, Oral Medicine, Oral Pathology, 21, 83. Stewart, R. E. & Prescott, G. H. (1976). Oral Facial Genetics. pp. 63&634. St. Louis: C. V. Mosby Company. Watson, W. L. & McCarthy, W. D. (1940). Blood and lymph vessel tumours. A report of 1,056 cases. Surgery, Gynecology and Obstetrics, 71, 569.
BLUE R. F. CROSHER,
RUBBER-BLEB
NAEVUS
SYNDROME
C. W. BLACKBURN, M.B., ct,.B., B.D.s., R. C. W. DINSDALE, B.G.D., F.D.S.R.C.S.
B.D.s., F.D.s.R.c.P.s.,
F.D.S.R.C.S.
and
Charles Clifford Dental Hospital,
Wellesley Road, Sheffield, SlO 2SZ
Summary. A case is reported of a patient who presented with facial pain and was found to have phleboliths associated with venous anomalies of the face and neck. Systemic investigation revealed a microcytic, hypochromic anaemia which was the result of haemorrhage from multiple haemangiomas of the gastro-intestinal tract. The finding of anaemia together with cutaneous and intestinal haemangiomas fits with a diagnosis of blue rubber-bleb naevus syndrome.
Introduction Haemangiomas are relatively common lesions in the head and neck region but are only rarely associated with anomalies elsewhere (Watson & McCarthy, 1940; Jones & Mason, 1980). The association of phleboliths with vascular malformation is well established (Gorlin & Goldman, 1970) and there have been a number of reports of haemangiomas with phlebolithiasis (Deighan & Barton, 1956; Smith et al., 1966; Dempsey & Murley, 1970; Faber et al., 1978; Keathley et al., 1983; Ikegami & Nishijima, 1984). Phleboliths form when an intravascular thrombus becomes organised and calcified. Their presence is highly suggestive (if not pathognomonic) of a cavernous haemangioma (Parker & Frommer, 1964). It is important to distinguish phleboliths from other calcific bodies; in the head and neck region, sialography should be used to differentiate between phleboliths and salivary calculi (O’Riordan, 1974). In addition to isolated cutaneous haemangiomas, the lesions may be multiple and represent a more generalised disorder. The association of cutaneous and intestinal haemangiomas was first reported by Gascoyen (1860). Shepherd (1953) gave an extensive review of the angiomatous conditions of the alimentary tract and drew attention to the high incidence of cutaneous lesions occurring with intestinal lesions. Bean (1958) introduced the term blue rubber-bleb naevus to describe the condition of haemangiomas of the skin and gastro-intestinal tract with intestinal bleeding. Case report A 25-year-old Caucasian female presented in May 1981 with a 6 month history of an ache in her right cheek. She associated the ache with a recurrent swelling over her right mandibular ramus. No precipitating or exacerbating factors were identified and the swelling was not related to gustatory stimulus. Three years earlier she had attended her general medical practitioner because of heavy menstrual bleeding. She was found to be anaemic and was treated with oral iron supplements. Her father had died from a brain haemorrhage at the age of 53 (Received Address
for correspondence:
30 December R. F. Crosher,
1986; accepted 31, Storthwood
160
17 February
1987)
Court,
Lane,
Storth
Sheffield,
SlO 3HP
BLUE
RUBBER-BLEB
NAEVUS
SYNDROME
161
years and she has an epileptic sister. There was no other relevant medical or family history. On examination a number of hard nodules were palpable in her right cheek. She had lesions also on the left side of the dorsum of her tongue and dorsum of her left hand which had the clinical appearance of haemangiomas. Radiographs demonstrated the presence of radiopaque bodies in the right and left cheeks (Fig. l), right side of neck (Fig. 2), and apices of both lungs (Fig. 3). A right parotid sialogram was normal; none of these opacities lay within the duct system. Her haemoglobin level was 12.8 g/dl. In April 1982, four of the calcified bodies in her right cheek were removed by an intra-oral approach under endotracheal anaesthesia. Histological examination of one specimen showed an enlarged vascular channel containing a mass of concentrically arranged amorphous eosinophilic material. The ground section appearances of the calcified bodies were those of phleboliths.
Fig. Figure
1-Orthopantogram
showing
a collection
1
of phleboliths face.
(arrowed)
Fig. 2 Figure
2-Postero-anterior
Figure
3-Chest
Fig. 3
radiograph
X-ray
on the right and left sides of
of mandible and the right
showing
phleboliths
showing phleboliths side of neck. (arrowed)
(arrowed)
at the apices
in the right cheek
of the lungs
162
BRITISH
JOURNAL
OF
ORAL
&
MAXILLOFACIAL
SURGERY
Following surgery the patient remained symptom-free until March 1983 when she developed a painful swelling on the right side of her neck. A discreet, firm, bluish nodule which was tender to palpation was visible at the level of the thyroid cartilage (Fig. 4) and a radiograph demonstrated phleboliths in this area (Fig. 5). She was admitted for the exploration of the area under general anaesthesia. Deep to platysma a vascular lesion was found lying in front of the anterior border of the sternomastoid muscle. This lesion appeared to involve the superficial lobe of the submandibular salivary gland and extended to the midline superficial to sternohyoid. A specimen was obtained which comprised a large number of veins and included the right submandibular salivary gland. A number of stones were palpable within the mass. Histological examination of the specimen demonstrated recent thrombi within vessels which showed evidence of endothelial proliferation. Degenerative changes were seen in other vessel walls. A section of the salivary gland block showed a phlebolith associated with a vessel close to normal salivary gland tissue. It appeared from these findings that this patient had multiple angiomatous lesions that were susceptible to the production of thrombi which became organised into phleboliths. She was investigated further in conjunction with a consultant physician. At this time the haematological findings were:Haemoglobin 8.7 g/d1 (Range 11.5-16.5 g/dl) Serum Iron 4 umol/l (Range 9-32 umol/l) TIBC 69 umol/l (Range 4.5-75 umol/l) Serum Ferritin 5 ng/ml (Range 15-130 ng/ml)
Fig. 4 Figure
4-Clinical Figure
photograph 5-Radiograph
Fig. showing
small haemangioma
showing
phleboliths
(‘Blue rubber
5
bleb’) on right side of neck.
at the level of the hyoid
bone.
BLUE
RUBBER-BLEB
NAEVUS
SYNDROME
163
The blood film showed microcytosis and hypochromia. Three specimens of faeces were positive for occult blood. A barium enema showed two small polyps; one at the splenic flexure, the other just proximal to the hepatic flexure. Colonoscopy revealed two polypoid vascular lesions l-2 cm in diameter. One was in the rectum, the second near the splenic flexure. There were at least three smaller lesions in the mid descending colon. Gastroscopy revealed that the stomach and duodenum were normal. Selective arteriography of the mesenteric vessels showed a collection of venous spaces in the region of the hepatic flexure. Venography of the forearm vessels showed widespread tiny varicosities. Cerebral arteriography did not reveal any intracranial vascular anomalies. Our patient was treated conservatively with oral iron supplements. Bowel resection was considered inappropriate because of the widespread distribution of the lesions.
Discussion Blue rubber-bleb naevus syndrome is an uncommon disease in which haemangiomas in the skin are associated with intestinal haemorrhage from similar lesions in the gut (Bean, 1958). In making a diagnosis, blue rubber-bleb naevus syndrome needs to be distinguished from hereditary haemorrhagic telangiectasia. In each there may be iron deficiency resulting from gastro-intestinal bleeding but the skin lesions are The typical lesion of hereditary haemorrhagic telangiectasia is distinctive. punctiform, l-4 mm in diameter, consisting of a tightly woven mat of telangiectatic vessels. The lesions are usually macular but may be papular or spider like. Subungual telangiectases are characteristic and oral and facial lesions are common (Shepherd, 1953). The lesions of the blue rubber-bleb naevus syndrome are larger, usually raised, having ‘the feel and look of rubber nipples’ (Bean, 1958) and never subungual. Hereditary haemorrhagic telangiectasia is inherited as an autosomal dominant trait and the family history is important in the diagnosis yet the mode of transmission of blue rubber-bleb naevus syndrome is not clear. Berlyne and Berlyne, (1960) reported a family in which the condition was transmitted as an autosomal dominant trait through five generations, but this is not a constant finding (Rice & Fischer, 1962) and there is no clear pattern of inheritance in our case. Cutaneous haemangiomas with phlebolithiasis are a feature of both the Maffucci syndrome (Demis et al., 1983) and the Klippel-Trenaunay-Weber syndrome (Stewart & Prescott, 1976). However, the marked skeletal abnormalities of the former and the unilateral limb hypertrophy of the latter distinguish them from the blue rubber-bleb naevus syndrome. Two notable features of our case are the presenting complaint of facial pain and the finding of phleboliths. There is some debate as to whether or not phleboliths cause pain. Deighan and Barton (1956) suggest that phleboliths are painless unless tissues are compressed against them. Dempsey and Murley (1970) propound that vascular congestion or thrombophlebitis may be the cause of pain in haemangiomas. The lesions in the case presented here were both tender on palpation and spontaneously painful. This observation has been recorded in other cases of the blue rubber-bleb naevus syndrome (Fine et al., 1961; Pruzanski, 1963; Fretzin & Potter, 1965). Haemangiomas in the skin and intestine are characteristic of the blue rubberbleb naevus syndrome but other organs also may be affected. In 1962 Rice and Fischer, reported a case in which haemangiomas were found in the liver, lungs and
164
BRITISH
JOURNAL
OF
ORAL
&
MAXILLOFACIAL
SURGEKk
skeletal muscle. The phleboliths seen on the chest X-ray in this case (Fig. 3) can be considered as markers of haemangiomas in the lungs. The iron deficiency anaemia of our patient was the result of bleeding from intestinal haemangiomas and is a typical feature of the blue rubber-bleb naevus syndrome (Bean, 1958). It is therefore recommended that patients presenting with cutaneous or mucosal haemangiomas and found to be anaemic should be investigated for intestinal lesions. Acknowledgements We are indebted to Dr .I. L. Maddocks (Consultant Physician, Royal Hallamshire Hospital) for his help in the diagnosis and management of our patient. Our thanks are due to the Department of Oral Pathology, University of Sheffield; Mr R. Cousins for the photographs; Mrs P. Oldfield and Mrs H. Gibson for typing the manuscript.
References Bean, W. B. (1958). Vuscular Spiders and Related Lesions of the Skin. pp. 178-185. Springfield Illinois: Charles, C. Thomas. Berlyne, G. M. & Berlyne, N. (1960). Anaemia due to ‘blue-rubber-bleb’ naevus disease. The Lance& 2, 1275. Deighan, W. J. & Barton, E. J. (1956). Haemangioma of the buccal pad with phlebolithiasis. Journal of Oral Surgery, 14, 228. Demis, D. .I.: Dobson, R. L. & McGuire, J. (1983). Haemangiomas. Clinical Dermatology 2 (10th Revision) Unit 7-65. Philadelphia: Harper & Row. Dempsey, E. F. & Murley, R. S. (1970). Vascular malformations simulating salivary disease. British Journal of Plastic Surgery, 23, 77. Faber, R. G., Ibrahim, S. Z., St. J. Brew, D. & Hobsley, M. (1978). Vascular malformations of the parotid region. British Journal of Surgery, 65, 171. Fine, R. M., Derbes, V. J. 81 Clark, W. H. (1961). Blue rubber-blcb nevus. Archives OfDermatology, 84, 144. Fretzin, D. F. & Potter, B. (1965). Blue rubber-bleb nevus. Archives of Internal Medicine, 116, 924. Gascoyen, G. G. (1860). Case of naevus involving the parotid gland and causing death from suffocation: Naevi of the viscera. Transactions of the Pathological Society of London, 11, 267. Gorlin, R. J. & Goldman, H. M. (1970). Thoma’s Oral Pathology, (6th Edition) Vol. 2 1031. St. Louis: C. V. Mosby Company. Ikegami, N. & Nishijima, I<. (1984). Haemangioma of the buccal pad with phlebolithiasis: report of a case. Acta Medica Okayama, 38, 79. Jones, J. H. & Mason, D. K. (1980). Oral Manifestations of Systemic DBease. p. 248. LondonPhiladelphia-Toronto: W. B. Saunders Company Ltd. Keathley, C. J., Campbell, R. L. and Isbell, J. W. (19X3). Arteriovenous malformation and associated phleboliths. Oral Surgery, Oral Medicine, Oral Pathology, 56, 132. O’Riordan, B. (1974). Phleboliths and salivary calculi. British Journal of Oral Surgery, 12, 119. Parker, L. A. & Frommer, H. H. (1974). Phleboliths. Report of a case. Oral Surgery, Oral Medicine, Oral Pathology, 18, 476. Pruzanski, W. (1963). Intestinal and cutaneous haemangiomatosis. Report of a case. American Journal of Digestive diseases, 8, 200. Rice, J. S. & Fischer, D. S. (1962). Blue rubber-bleb nevus syndrome. Archives qf Dermatology, 86, 163. Shepherd, J. A. (1953). Angiomatous conditions of the gastro-intestinal tract. Brifish Journal of Surgery, 40, 409. Smith, J. F., Drake, J. & Sollee, N. (1966). Massive oral haemangioma with phlebolithiasis. Oral Surgery, Oral Medicine, Oral Pathology, 21, 83. Stewart, R. E. & Prescott, G. H. (1976). Oral Facial Genetics. pp. 63&634. St. Louis: C. V. Mosby Company. Watson, W. L. & McCarthy, W. D. (1940). Blood and lymph vessel tumours. A report of 1,056 cases. Surgery, Gynecology and Obstetrics, 71, 569.