International Journal of Paediatric Dentistry 1992; 2: 4 1-45
An unusual giant cell lesion in a child with vitamin D-resistant rickets BARBARA L. CHADWICK' & MICHAEL J . ALDRED2 'Department of Child Dental Health, University of Wales College of Medicine Vnstitute of Medical Genetics, University of Wales College of Medicine
Summary. This paper reports the presence of a focus of giant cells in a sinus tract associated with an abscessed primary tooth in a patient with vitamin D-resistant rickets. The relevance of this giant cell lesion to the systemic disorder is discussed.
Introduction Rickets unresponsive to normal doses of vitamin D was first reported by McCune [l]. This entity was characterised as vitamin D-resistant rickets (VDRR) by Albright et al. [2]. The prime features of the disorder have been summarised as being familial occurrences, lowered serum phosphate levels with decreased renal reabsorption of phosphate, as well as the unresponsiveness to physiological doses of vitamin D [3]. Classically, VDRR is inherited as an X-linked disorder [4], although alternative modes of inheritance have also been recognized [3]. Males are usually more severely affected than females, who may show only mild hypophosphataemia without any clinical stigmata [5]. Clinically, patients (especially affected males) are short in stature and often have marked lateral bowing of the legs. This does not usually become apparent until the child starts to walk [6]. Frontal bossing, craniotabes and scoliosis have also been reported [2,7]. Radiographic findings are similar to those in classical vitamin D-dependent rickets, with defective calcification of the epiphyseal cartilage seen at the distal ends of the long bones, bony protuberances at the site of major muscle attachments and pseudofractures [2,6]. Laboratory examination usually reveals slightly Correspondence to: Miss B. Chadwick, Department of Child Dental Health, University of Wales Collegeof Medicine, Heath Park, Cardiff CF4 4XY, UK.
lowered serum calcium and phosphate levels and a mild increase in alkaline phosphatase concentration. Circulating levels of immunoreactive parathyroid hormone are usually slightly higher than in control patients [3,8,9]. Dental findings were first described by Gunther et al. [ 101 and subsequently by Rushton [ 1 11 and Harris & Sullivan [ 121. Dental problems may be the reason for attendance [ 131. Both the primary and permanent dentitions are involved, the characteristic finding being draining abscesses in caries-free primary teeth [ 121; this latter sign has been regarded as pathognomonic of the disorder [ 13,141. It is probable that this is pathognomonic of rickets per se rather than vitamin D-resistant rickets as it is also seen in vitamin Ddependent rickets [ 15,161. Other reported dental findings include thin enamel [ 14,171and enamel hypoplasia [7,14]. The study of Abe et al. [18] did not confirm any significant reduction in enamel thickness, but did find evidence of hypomineralization. The dentine is markedly affected; mantle dentine is formed normally but beneath this there are masses of poorly mineralised globular dentine [ 11,121. In the region of the pulp horns tubular defects of dentine extend to the enamel-dentine junction (EDJ) [7,12]. Periapical complications occur following direct invasion of the pulp by micro-organisms through these defects. The bacteria apparently gain access as the enamel is worn away by attrition, thus exposing the underlying dentine and pulp 1141. 41
42
B. L. Chadwick & M. J. Aldred
teeth and the developing permanent first molars were considered to be larger than normal, with pulp horns extending towards the enamel-dentine junction (EDJ). In addition the primary molars and the developing permanent molars showed apical displacement of the pulpal floor (taurodontism). A radiolucent line within the dentine adjacent to the EDJ was observed in the following teeth:
I 6 4 1
Fig. 1. Pedigree of family with X-linked vitamin D-resistant rickets. 0 males 0 females affected male 0 heterozygous female
Case report The patient, a male white Caucasian, was first referred to the Department of Child Dental Health at the age of 6 years 7 months by his dental practitioner for advice regarding a chronic abscess associated with the maxillary left primary central incisor tooth. The second child of unrelated parents, he had been diagnosed at the age of 13 months as having VDRR after he had developed bow legs while learning to walk. Investigation of the family had subsequently identified his mother as being hypophosphataemic (she had mild clinical evidence of the disorder), but his older brother and maternal uncle showed no evidence of hypophosphataemia or rickets (Fig. 1). At the time of dental consultation he was taking phosphate supplements and 1-alphahydroxycholecalciferol. Clinical examination revealed an apparently healthy boy. All primary teeth were present and erupted. The pulp chamber of the maxillary left primary central incisor had been opened palatally to drain, and the mandibular right primary first molar was noted to be submerging. An orthopantomograph (Fig. 2) showed all permanent teeth, with the exception of third molars, to be present. The pulp chambers of the primary
6
(36; 44, 46)
The contrast between enamel and dentine was felt to be within normal limits and the lamina dura was visible around most of the teeth. It was decided to extract the abscessed primary incisor and the submerged primary molar. The patient was next seen 18 months later. The maxillary right primary canine had been extracted elsewhere some 5 months previously, following the development of an abscess. However, a sinus related to this abscess had failed to heal. A soft tissue swelling considered to be another sinus was present buccally in the mandibular left primary second molar region. Radiographs showed a radiolucent area between the roots of this tooth, but no radiographic abnormality could be seen in the region of maxillary right primary canine. Under local anaesthesia the mandibular left primary second molar was extracted and the soft tissue lesion associated with the tooth curetted and sent for histopathological examination. A flap was raised to examine the area around the sinus in the maxillary right primary canine region, but no bony defect was found. The sinus tract, which was found to be blind-ended and confined to the soft tissues, was excised and submitted for histopathological examination. The area healed uneventfully with no subsequent problems over a 2-year follow-up period. During that time period, the maxillary left primary canine was extracted by his general dental practitioner, because of a chronic abscess. Medically he was well, his most recent parathyroid hormone (PTH) level being 3-5 ng/l, within the normal range of 0.9-5.4 ng/l.
Histopa thology The first soft tissue specimen, associated with the extracted primary molar, was diagnosed to be a periapical granuloma. The second soft tissue
Unusual giant cell lesion
43
Fig. 2. Orthopantomograph showing the patient’s dentition at the age of 6 years 7 months, the enlarged pulp horns in molar teeth and the pulpal extensions to the enamel-dentine junction.
specimen (from the primary canine region) was covered by a normal thickness of non-keratinised stratified squamous epithelium. Within the fibrous conective tissue were large numbers of multinucleate giant cells together with capillaries and haemosiderin deposits (Fig. 3).
Discussion The essential feature of X-linked VDRR is hypophosphataemia, with both the biochemical and clinical features being more severe in affected males than in heterozygous females. The pathogenesis has not yet been resolved, as there is some debate whether it is related to renal tubular reabsorption of phosphate or to defective uptake of calcium from the gastro-intestinal tract and subsequent overactivity of the parathyroid glands (for review see McKusick [ 191). The dental features in the present case are similar to those reported in previous studies. The development of abscesses in the absence of caries is a well-documented and characteristic finding in VDRR [7,12,13], as well as in vitamin D-dependent rickets [ 151. Eruption of permanent teeth is typically retarded [ 12,20-221. Although larger than normal pulps were found in VDRR, in our case the bifurcation was considered to be apically displaced, characteristic of taurodontism- [23].- It is not clear from the literature whether this is a consistent finding in VDRR. There are numerous reports in the
Fig. 3. Haematoxylin and eosin-stained section showing giant cells 40).
literature of large pulps or pulp horns extending to the EDJ [3,12-14,20,22,24-281, but only
44
B. L. Chadwick & M. J. Aldred
Bender & Naidorf [14] specifically referred to taurodontism. Some other workers have, however, presented radiographs which might be classified in this way [22,25,28,29]. It is not clear whether taurodontism in VDRR is related to the association of the condition with the X chromosome. The radiolucent band at the EDJ was first reported by Via [ 171 and it has been suggested as being caused by large zones of poorly calcified interglobular dentine at the periphery of the dentine adjacent to, or close to, the EDJ [21]. Although in our case the enamel contrasted with the dentine radiographically, Abe et al. [ 181 demonstrated hypomineralization which might account for reports of marked abrasion and chipping of enamel. A number of treatment regimes have been proposed. Stainless steel crowning of primary molars has been suggested [30,311, but preparation may lead to pulp exposures and subsequent pulp necrosis. Pulp treatment of abscessed teeth has proved unsuccessful [22], while elective pulp extirpation has been advocated by some workers 1141. The unusual feature reported in the present case is a giant cell lesion presenting in a persistent sinus tract, at the site of extraction of an abscessed tooth. Giant cell lesions may be either peripheral, as in the giant cell epulis, or deep-seated, as in the central giant cell granuloma. Either of these two giant cell lesions of the jaws may be a manifestation of hyperparathyroidism, but do not appear to have been reported in VDRR. It is possible that this lesion reflects a mild degree of overactivity of the parathyroid glands, which would be consistent with reports of elevated levels of immunoreactive parathyroid hormone in VDRR [8,9]. Falls et al. [32] reported phosphaturia due to secondary hyperparathyroidism in VDRR, and Thomas & Fry [33] described a case of hyperparathyroidism due to adenoma of the parathyroid glands associated with bone lesions in VDRR. It has been reported that giant cell lesions are more frequently encountered in primary hyperparathyroidism than in the secondary condition [34]. Although not a previously well-reported feature of VDRR, the presence of a giant cell lesion in the jaws of VDRR patients may be the first indication of the development of hyperparathyroidism. There are two factors which suggest that there was no element of hyperparathyroidism in our case.
Firstly, no loss of lamina dura appears to have occurred and the bony architecture of the jaws showed no obvious abnormality; however, in the absence of a skeletal survey, the possible existence of pathological changes in the rest of the skeleton cannot be excluded. Secondly, the patient's most recent circulating PTH level was normal. It is possible that the sinus tract acted as a nidus for giant cell proliferation, perhaps because of persistent low-grade inflammation. Whether this related to a small fragment of retained root from the primary canine is unclear but, since no bony defect was observed on exploration, this is perhaps less likely.
Acknowledgements We are grateful to Dr K Verrier-Jones (Senior Lecturer in Paediatric Nephrology) for her advice during the preparation of this paper.
RdsumC. Cette Ctude montre la prdsence de cellules gCantes dans le sinus associCs avec une dent temporaire chez un patient souffrant d'un rachitisme resistant A la vitamine D. La relation entre cette lCsion a cellules gCantes et une pathologie de systkme est discutCe. Zussamenfassung. Diese Arbeit berichtet uber einen Patienten mit einer Vitamin D resistenten Ricketserkrankung bei welchem im Sinus ein Fokus von Riesenzellen gefunden wurden, dies in Verbindung mit einem Abzess ausgehend von einem Milchzahn. Der eventuelle Zusammenhang dieser Riesenzell-Usion mit einer systemischen Erkrankung wird besprochen. Resumen. Este articulo reporta la presencia de un foco de cClulas gigantes y trayecto sinuoso asociado con un diente temporal abscesado en un paciente con riquetsia resistente a vitamina D. La relevancia de esta lesion de cClulas gigantes con el trastorno sistCmico es discutido.
References 1 McCune DV. Refractoryrickets. The Journal ofPediutrics 1935; 6:872-873. 2 Albright F, Butler AM, Bloomberg E. Rickets resistant to
Unusual giant cell lesion
3 4 5
6
7 8 9
I0 1I
12 I3
14 15
16 17 18
vitamin D therapy. American Journal of Diseases of Children 1937; 5 4 529-547. Bixler D. Heritable disorders affecting dentin, in: Stewart RE, Prescott GH (eds), Oralfacialgenetics. St Louis: C. V. Mosby, 1976; 249-253. Christensen JF. Three familial cases of atypical late rickets. Acta Paediatrica 1941; 28: 241-270. Winters RW, Graham JB. Multiple genetic mechanisms. Pediatrics 1960; 2 5 932-933. Hirschman GH, DeLuca HF, Chan JCM. Hypophosphatemic vitamin D resistant rickets: metabolic balance studies in a child receiving 1,25 dihydroxyvitamin D3, phosphate and ascorbic acid. Pediatrics 1978; 61: 45 1-457. Witkop CJ. Manifestations of genetic diseases in the human pulp. Oral Surgery 1971; 32: 278-3 16. Reitz RE, Weinstein RL. Parathyroid hormone secretion in familial vitamin-D-resistant rickets. New England Journal of Medicine 1973; 289: 941-945. Hahn TJ, Scharp CR, Halstead LR, Haddad JG, Karl DM, Avioli LV. Parathyroid hormone status and renal responsiveness in familial hypophosphatemic rickets. Journal of Clinical Endocrinology and Metabolism 1975; 41: 926-937. Gunther L, Cohn ET, Cohn WE, Greenberg DM. Metabolism of bone salts in resistant rickets. American Journalof Diseases of Children 1943; 6 6 5 17-527. Rushton MA. Two specimens illustrating proteolysis of dentine. Proceedings of the Royal Society of Medicine 1958; 5 2 115-1 17. Hams R, Sullivan HR. Dental sequelae in deciduous dentition in vitamin D resistant rickets. Australian Dental Journal 1960; 5 200-203. Archard HO, Witkop CJ. Hereditary hypophosphatemia (vitamin D-resistant rickets) presenting primary dental manifestations. Oral Surgery, Oral Medicine and Oral Pathology 1966; 22: 184-193. Bender IB, Naidorf IJ. Dental observations in vitamin D-resistant rickets with special reference to periapical lesions. Journal of Endodontics 1985; 11: 514-520. Arnaud C, Maijer R, Reade T, Scriver CR, Whelan DT. Vitamin D dependancy: an inherited postnatal syndrome with secondary hyperparathyroidism. Paediatrics 1970; 46: 871-880. Witkop CJ, Sauk JJ. Heritable defects of enamel, in: Stewart RE, Prescott GH (eds), Oral facial genetics. St Louis: CV Mosby 1976; 218-219. Via WF. ‘Spontaneous’ degeneration of the dental pulp associated with phosphate diabetes. Oral Surgery, Oral Medicine and Oral Pathology 1967; 2 4 623-628. Abe K, Ooshima T, Lily TSM, Yasafuku Y, Sobue S. Structural deformities of deciduous teeth in patients with
I9 20 21 22 23 24
25 26 27 28 29 30
31 32 33 34
45
hypophosphatemic vitamin D-resistant rickets. Oral Surgery, Oral Medicine and Oral Pathology 1988; 6 5 191-198. McKusick VM. X-linked phenotypes, in: Mendelian inheritance in man. Baltimore: The Johns Hopkins University Press, 1988; I 3 17-1 3 19. Marks SC, Lindahl RL, Bawden JW. Dental and cephalometric findings in vitamin D resistant rickets. Journal of Dentistry for Children 1965; 32: 259-265. Pliskin ME, Brown AM, Baden EE, Kimball HG. Vitamin D resistant rickets of a young adult patient. JournalofOral Medicine 1975; 3 0 77-79. Breen GH. Prophylactic dental treatment for a patient with vitamin D-resistant rickets: report of case. Journalof Dentistry for Children 1986; 5 3 38-43. Keith A. Problems relating to the teeth of the earlier forms of prehistorical man. Proceedings of the Royal Society of Medicine 1913; 6 103- 1 19. Greenburg BG, Winters RW, Graham JB. The normal range of serum inorganic phosphorus and its utility as a discriminant in the diagnosis of congenital hypophosphatemia. Journal of Clinical Endocrinology 1960; 2 0 364-379. Gardener DE, Davis WB, Prescott GH. Hereditary hypophosphatemia. Journal of Dentistry for Children 1969; 36: 199-2 16. Ainley JE. Manifestations of familial hypophosphatemic rickets. Journal of Endodontics 1978; 4 26-28. Vasilakas GJ, Nygaard VK, DiPalma DM. Vitamin D resistant rickets. Journal of Oral Medicine 1980; 3 5 19-26. Herbert FL. Hereditary hypophosphatemia rickets. Journal of Dentistry for Children 1986; 53: 223-226. Fadavi S,Rowold E. Familial hypophosphatemic vitamin D-resistant rickets: review of the literature and report of a case. Journal of Dentistry for Children 1990; 57: 2 12-2 1 5. Cohen S, Becker GL. Origin, diagnosis and treatment of the dental manifestations of vitamin D resistant rickets: review of the literature and report of a case. Journalof the American Dental Association 1976; 9 2 120- 129. Tullock EN, Andrews FH. The association of dental abscesses with vitamin D resistant rickets. British Dental Journal 1983; 154 136-138. Falls WF, Carter NW, Rector FC, Seldin DW. Familial vitamin D-resistant rickets. Annals of Internal Medicine 1968; 68: 553-560. Thomas WC, Fry RM. Adenomas in chronic rickets. The American Journal of Medicine 1910; 4 9 404-407. Gurumurthy K, Solomon M, Butt K, Gelfman W, Moel DI. Maxillary brown tumour caused by secondary hyperparathyroidism in an infant. Journal of Pediatrics 1982; 100 245-247.
An unusual giant cell lesion in a child with vitamin D-resistant rickets BARBARA L. CHADWICK' & MICHAEL J . ALDRED2 'Department of Child Dental Health, University of Wales College of Medicine Vnstitute of Medical Genetics, University of Wales College of Medicine
Summary. This paper reports the presence of a focus of giant cells in a sinus tract associated with an abscessed primary tooth in a patient with vitamin D-resistant rickets. The relevance of this giant cell lesion to the systemic disorder is discussed.
Introduction Rickets unresponsive to normal doses of vitamin D was first reported by McCune [l]. This entity was characterised as vitamin D-resistant rickets (VDRR) by Albright et al. [2]. The prime features of the disorder have been summarised as being familial occurrences, lowered serum phosphate levels with decreased renal reabsorption of phosphate, as well as the unresponsiveness to physiological doses of vitamin D [3]. Classically, VDRR is inherited as an X-linked disorder [4], although alternative modes of inheritance have also been recognized [3]. Males are usually more severely affected than females, who may show only mild hypophosphataemia without any clinical stigmata [5]. Clinically, patients (especially affected males) are short in stature and often have marked lateral bowing of the legs. This does not usually become apparent until the child starts to walk [6]. Frontal bossing, craniotabes and scoliosis have also been reported [2,7]. Radiographic findings are similar to those in classical vitamin D-dependent rickets, with defective calcification of the epiphyseal cartilage seen at the distal ends of the long bones, bony protuberances at the site of major muscle attachments and pseudofractures [2,6]. Laboratory examination usually reveals slightly Correspondence to: Miss B. Chadwick, Department of Child Dental Health, University of Wales Collegeof Medicine, Heath Park, Cardiff CF4 4XY, UK.
lowered serum calcium and phosphate levels and a mild increase in alkaline phosphatase concentration. Circulating levels of immunoreactive parathyroid hormone are usually slightly higher than in control patients [3,8,9]. Dental findings were first described by Gunther et al. [ 101 and subsequently by Rushton [ 1 11 and Harris & Sullivan [ 121. Dental problems may be the reason for attendance [ 131. Both the primary and permanent dentitions are involved, the characteristic finding being draining abscesses in caries-free primary teeth [ 121; this latter sign has been regarded as pathognomonic of the disorder [ 13,141. It is probable that this is pathognomonic of rickets per se rather than vitamin D-resistant rickets as it is also seen in vitamin Ddependent rickets [ 15,161. Other reported dental findings include thin enamel [ 14,171and enamel hypoplasia [7,14]. The study of Abe et al. [18] did not confirm any significant reduction in enamel thickness, but did find evidence of hypomineralization. The dentine is markedly affected; mantle dentine is formed normally but beneath this there are masses of poorly mineralised globular dentine [ 11,121. In the region of the pulp horns tubular defects of dentine extend to the enamel-dentine junction (EDJ) [7,12]. Periapical complications occur following direct invasion of the pulp by micro-organisms through these defects. The bacteria apparently gain access as the enamel is worn away by attrition, thus exposing the underlying dentine and pulp 1141. 41
42
B. L. Chadwick & M. J. Aldred
teeth and the developing permanent first molars were considered to be larger than normal, with pulp horns extending towards the enamel-dentine junction (EDJ). In addition the primary molars and the developing permanent molars showed apical displacement of the pulpal floor (taurodontism). A radiolucent line within the dentine adjacent to the EDJ was observed in the following teeth:
I 6 4 1
Fig. 1. Pedigree of family with X-linked vitamin D-resistant rickets. 0 males 0 females affected male 0 heterozygous female
Case report The patient, a male white Caucasian, was first referred to the Department of Child Dental Health at the age of 6 years 7 months by his dental practitioner for advice regarding a chronic abscess associated with the maxillary left primary central incisor tooth. The second child of unrelated parents, he had been diagnosed at the age of 13 months as having VDRR after he had developed bow legs while learning to walk. Investigation of the family had subsequently identified his mother as being hypophosphataemic (she had mild clinical evidence of the disorder), but his older brother and maternal uncle showed no evidence of hypophosphataemia or rickets (Fig. 1). At the time of dental consultation he was taking phosphate supplements and 1-alphahydroxycholecalciferol. Clinical examination revealed an apparently healthy boy. All primary teeth were present and erupted. The pulp chamber of the maxillary left primary central incisor had been opened palatally to drain, and the mandibular right primary first molar was noted to be submerging. An orthopantomograph (Fig. 2) showed all permanent teeth, with the exception of third molars, to be present. The pulp chambers of the primary
6
(36; 44, 46)
The contrast between enamel and dentine was felt to be within normal limits and the lamina dura was visible around most of the teeth. It was decided to extract the abscessed primary incisor and the submerged primary molar. The patient was next seen 18 months later. The maxillary right primary canine had been extracted elsewhere some 5 months previously, following the development of an abscess. However, a sinus related to this abscess had failed to heal. A soft tissue swelling considered to be another sinus was present buccally in the mandibular left primary second molar region. Radiographs showed a radiolucent area between the roots of this tooth, but no radiographic abnormality could be seen in the region of maxillary right primary canine. Under local anaesthesia the mandibular left primary second molar was extracted and the soft tissue lesion associated with the tooth curetted and sent for histopathological examination. A flap was raised to examine the area around the sinus in the maxillary right primary canine region, but no bony defect was found. The sinus tract, which was found to be blind-ended and confined to the soft tissues, was excised and submitted for histopathological examination. The area healed uneventfully with no subsequent problems over a 2-year follow-up period. During that time period, the maxillary left primary canine was extracted by his general dental practitioner, because of a chronic abscess. Medically he was well, his most recent parathyroid hormone (PTH) level being 3-5 ng/l, within the normal range of 0.9-5.4 ng/l.
Histopa thology The first soft tissue specimen, associated with the extracted primary molar, was diagnosed to be a periapical granuloma. The second soft tissue
Unusual giant cell lesion
43
Fig. 2. Orthopantomograph showing the patient’s dentition at the age of 6 years 7 months, the enlarged pulp horns in molar teeth and the pulpal extensions to the enamel-dentine junction.
specimen (from the primary canine region) was covered by a normal thickness of non-keratinised stratified squamous epithelium. Within the fibrous conective tissue were large numbers of multinucleate giant cells together with capillaries and haemosiderin deposits (Fig. 3).
Discussion The essential feature of X-linked VDRR is hypophosphataemia, with both the biochemical and clinical features being more severe in affected males than in heterozygous females. The pathogenesis has not yet been resolved, as there is some debate whether it is related to renal tubular reabsorption of phosphate or to defective uptake of calcium from the gastro-intestinal tract and subsequent overactivity of the parathyroid glands (for review see McKusick [ 191). The dental features in the present case are similar to those reported in previous studies. The development of abscesses in the absence of caries is a well-documented and characteristic finding in VDRR [7,12,13], as well as in vitamin D-dependent rickets [ 151. Eruption of permanent teeth is typically retarded [ 12,20-221. Although larger than normal pulps were found in VDRR, in our case the bifurcation was considered to be apically displaced, characteristic of taurodontism- [23].- It is not clear from the literature whether this is a consistent finding in VDRR. There are numerous reports in the
Fig. 3. Haematoxylin and eosin-stained section showing giant cells 40).
literature of large pulps or pulp horns extending to the EDJ [3,12-14,20,22,24-281, but only
44
B. L. Chadwick & M. J. Aldred
Bender & Naidorf [14] specifically referred to taurodontism. Some other workers have, however, presented radiographs which might be classified in this way [22,25,28,29]. It is not clear whether taurodontism in VDRR is related to the association of the condition with the X chromosome. The radiolucent band at the EDJ was first reported by Via [ 171 and it has been suggested as being caused by large zones of poorly calcified interglobular dentine at the periphery of the dentine adjacent to, or close to, the EDJ [21]. Although in our case the enamel contrasted with the dentine radiographically, Abe et al. [ 181 demonstrated hypomineralization which might account for reports of marked abrasion and chipping of enamel. A number of treatment regimes have been proposed. Stainless steel crowning of primary molars has been suggested [30,311, but preparation may lead to pulp exposures and subsequent pulp necrosis. Pulp treatment of abscessed teeth has proved unsuccessful [22], while elective pulp extirpation has been advocated by some workers 1141. The unusual feature reported in the present case is a giant cell lesion presenting in a persistent sinus tract, at the site of extraction of an abscessed tooth. Giant cell lesions may be either peripheral, as in the giant cell epulis, or deep-seated, as in the central giant cell granuloma. Either of these two giant cell lesions of the jaws may be a manifestation of hyperparathyroidism, but do not appear to have been reported in VDRR. It is possible that this lesion reflects a mild degree of overactivity of the parathyroid glands, which would be consistent with reports of elevated levels of immunoreactive parathyroid hormone in VDRR [8,9]. Falls et al. [32] reported phosphaturia due to secondary hyperparathyroidism in VDRR, and Thomas & Fry [33] described a case of hyperparathyroidism due to adenoma of the parathyroid glands associated with bone lesions in VDRR. It has been reported that giant cell lesions are more frequently encountered in primary hyperparathyroidism than in the secondary condition [34]. Although not a previously well-reported feature of VDRR, the presence of a giant cell lesion in the jaws of VDRR patients may be the first indication of the development of hyperparathyroidism. There are two factors which suggest that there was no element of hyperparathyroidism in our case.
Firstly, no loss of lamina dura appears to have occurred and the bony architecture of the jaws showed no obvious abnormality; however, in the absence of a skeletal survey, the possible existence of pathological changes in the rest of the skeleton cannot be excluded. Secondly, the patient's most recent circulating PTH level was normal. It is possible that the sinus tract acted as a nidus for giant cell proliferation, perhaps because of persistent low-grade inflammation. Whether this related to a small fragment of retained root from the primary canine is unclear but, since no bony defect was observed on exploration, this is perhaps less likely.
Acknowledgements We are grateful to Dr K Verrier-Jones (Senior Lecturer in Paediatric Nephrology) for her advice during the preparation of this paper.
RdsumC. Cette Ctude montre la prdsence de cellules gCantes dans le sinus associCs avec une dent temporaire chez un patient souffrant d'un rachitisme resistant A la vitamine D. La relation entre cette lCsion a cellules gCantes et une pathologie de systkme est discutCe. Zussamenfassung. Diese Arbeit berichtet uber einen Patienten mit einer Vitamin D resistenten Ricketserkrankung bei welchem im Sinus ein Fokus von Riesenzellen gefunden wurden, dies in Verbindung mit einem Abzess ausgehend von einem Milchzahn. Der eventuelle Zusammenhang dieser Riesenzell-Usion mit einer systemischen Erkrankung wird besprochen. Resumen. Este articulo reporta la presencia de un foco de cClulas gigantes y trayecto sinuoso asociado con un diente temporal abscesado en un paciente con riquetsia resistente a vitamina D. La relevancia de esta lesion de cClulas gigantes con el trastorno sistCmico es discutido.
References 1 McCune DV. Refractoryrickets. The Journal ofPediutrics 1935; 6:872-873. 2 Albright F, Butler AM, Bloomberg E. Rickets resistant to
Unusual giant cell lesion
3 4 5
6
7 8 9
I0 1I
12 I3
14 15
16 17 18
vitamin D therapy. American Journal of Diseases of Children 1937; 5 4 529-547. Bixler D. Heritable disorders affecting dentin, in: Stewart RE, Prescott GH (eds), Oralfacialgenetics. St Louis: C. V. Mosby, 1976; 249-253. Christensen JF. Three familial cases of atypical late rickets. Acta Paediatrica 1941; 28: 241-270. Winters RW, Graham JB. Multiple genetic mechanisms. Pediatrics 1960; 2 5 932-933. Hirschman GH, DeLuca HF, Chan JCM. Hypophosphatemic vitamin D resistant rickets: metabolic balance studies in a child receiving 1,25 dihydroxyvitamin D3, phosphate and ascorbic acid. Pediatrics 1978; 61: 45 1-457. Witkop CJ. Manifestations of genetic diseases in the human pulp. Oral Surgery 1971; 32: 278-3 16. Reitz RE, Weinstein RL. Parathyroid hormone secretion in familial vitamin-D-resistant rickets. New England Journal of Medicine 1973; 289: 941-945. Hahn TJ, Scharp CR, Halstead LR, Haddad JG, Karl DM, Avioli LV. Parathyroid hormone status and renal responsiveness in familial hypophosphatemic rickets. Journal of Clinical Endocrinology and Metabolism 1975; 41: 926-937. Gunther L, Cohn ET, Cohn WE, Greenberg DM. Metabolism of bone salts in resistant rickets. American Journalof Diseases of Children 1943; 6 6 5 17-527. Rushton MA. Two specimens illustrating proteolysis of dentine. Proceedings of the Royal Society of Medicine 1958; 5 2 115-1 17. Hams R, Sullivan HR. Dental sequelae in deciduous dentition in vitamin D resistant rickets. Australian Dental Journal 1960; 5 200-203. Archard HO, Witkop CJ. Hereditary hypophosphatemia (vitamin D-resistant rickets) presenting primary dental manifestations. Oral Surgery, Oral Medicine and Oral Pathology 1966; 22: 184-193. Bender IB, Naidorf IJ. Dental observations in vitamin D-resistant rickets with special reference to periapical lesions. Journal of Endodontics 1985; 11: 514-520. Arnaud C, Maijer R, Reade T, Scriver CR, Whelan DT. Vitamin D dependancy: an inherited postnatal syndrome with secondary hyperparathyroidism. Paediatrics 1970; 46: 871-880. Witkop CJ, Sauk JJ. Heritable defects of enamel, in: Stewart RE, Prescott GH (eds), Oral facial genetics. St Louis: CV Mosby 1976; 218-219. Via WF. ‘Spontaneous’ degeneration of the dental pulp associated with phosphate diabetes. Oral Surgery, Oral Medicine and Oral Pathology 1967; 2 4 623-628. Abe K, Ooshima T, Lily TSM, Yasafuku Y, Sobue S. Structural deformities of deciduous teeth in patients with
I9 20 21 22 23 24
25 26 27 28 29 30
31 32 33 34
45
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