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Case Reports

Indian Pediatrics 2003; 40:894-896 

Oral Bisphosphonates in Polyostotic Fibrous Dysplasia

V.V. Khadilkar
A.V. Khadilkar
G.B. Maskati

From the Growth and Pediatric Endocrine Unit Hirabai Cowasji Jehangir Medical Research Institute, Jehangir Hospital, 32, Sassoon Road, Pune 411 004, India.

Correspondence to: Dr. Vaman Khadilkar, Consultant Pediatric Endocrinologist, Hirabai Cowasji Jehangir Medical Research Institute, Jehangir Hospital 32, Sassoon Road, Pune 411 004, India. E-mail: akhadilkar@vsnl.net

Manuscript received: October 23, 2002; Initial review completed: December 13, 2002; Revision accepted: February 28, 2003.


The bisphosphonates inhibit osteoclastic bone resorption and have been used as an intravenous infusion in fibrous dysplasia of the bone. Oral bisphosphonates are cheaper and also easy to administer. We report a case of polyostotic fibrous dysplasia in a three-year-old child who showed significant improvement in bone mineral density after treatment with oral alendronate.

Keywords: Bisphosphonates, Polyostotic fibrous dysplasia.

Fibrous dysplasia of the bone can involve one or several bones and is characterized by bone deformities, pain and fractures. The bony lesions of fibrous dysplasia are characterized by proliferation of fibroblast like cells that in some areas have features of osteoblasts and in other areas have features of chondrocytes. There is also an associated increase in osteoclastic bone resorption. The lesions are usually focal and may be monoostotic or polyostotic and have a radiolucent appearance. The disorder occurs with equal frequency in both sexes(1) and in some individuals may have distinctive areas of skin pigmentation and precocious puberty (McCune Albright syndrome). Fractures and skeletal deformities are common and early onset disease is usually more severe.

In the past 10 years, the bisphosphonate pamidronate has been used as an intravenous infusion in fibrous dysplasia of the bone(2). Oral bisphosphonate is a cheaper alternative and is also easy to administer, hence its applicability in Indian circumstances is more. We report a case of polyostotic fibrous dysplasia in a three-year-old child who was given oral alendronate and showed significant improvement in the bone mineral content while on this therapy.

Case Report

Three-year-old male child, fifth product of a non-consanguinous marriage presented with repeated fractures of the left femur, pain while walking and asymmetry of the lower limbs. He was fully immunized and had normal intellectual development but had delayed sitting and walking. By the time he was five years old he had 5 fractures of the left femur requiring surgical corrections. During this time his pain continued and so did the asymmetry. Clinical examination showed unequal lower extremity length, joint laxity, hyper-extensible joints, tibial deformity and genu valgum. Rest of his systemic and general examination was normal. His biochemistry showed calcium 8.5 mg/dL, serum protein 7.4 g/dL, serum albumin of 3.9 g/dL, serum phosphorus was 1.9 mg/dL, alkaline phosphatase was 310 µ/L (50-350 µ/L) and parathormone was 69.5 pg/mL (12-72 pg/mL). Renal function was normal with a blood urea of 6.4 mg/dL, creatinine of 0.3 mg/dL, serum sodium was 137.6 mmol/L and potassium was 3.1 mmol/L, uric acid was 2.7 mg/dL, CPK was 53 µ (24-190/µL). Hemo-gram was normal. Routine urine examination was normal. Radiography showed radiolucent areas in phalanges and in the metacarpals and Looser’s zones at metaphyseal ends of long bones. Abdominal and renal sonography was normal. No parathyroid mass was seen on USG. Whole body scan using Technetium 99 suggested polyostotic fibrous dysplasia localizing the distribution of the lesions to the left axial skeleton and left side of pelvis, skull and facial bones. His bone biopsy was consistent with a diagnosis of fibrous dysplasia. DEXA scan of the lumbar spine showed low bone mineral density (BMD) with a Z score of –2.51. BMD of left hip was much lower (0.241 g/cm2) as compared to that of the right hip (0.403 g/cm2). He was treated with oral alendronate in a dose of 5 mg per day. A repeat DEXA scan following 9 months of alendronate therapy showed that BMD at lumbar spine and both hips had significantly increased with a Z score of –1.68. The BMD in the lumbar spine increased by 0.067 g/cm2 accounting for almost 8% bone mass gain. BMD at right and left hip increased by 0.054 g/cm2 and 0.023 g/cm2 respectively. The next follow-up DEXA scan of the lumbar spine done after 18 months of therapy showed a further increase in BMD at lumbar spine and both hips with a Z score of –1.27. The BMD at lumbar spine increased by 0.041g/cm2 and that at the right and left hip (in the region of femoral neck) increased by 0.057 g/cm2 and 0.007 g/cm2 respectively.

Calcium and vitamin D supplementation (elemental calcium 1000 mg and vitamin D 3,500 IU/day) was also given during alendro-nate therapy. His bone pain was significantly reduced and he did not have any further fractures while on treatment with alendronate, although asymmetry was persistent. During the course of therapy no side effects were reported and therapy was tolerated well.


The main biologic action of bisphospho-nates consists of the inhibition of osteoclastic bone resorption and hence they play a major role in conditions that are characterized by an increased bone resorption(1). Primary and secondary osteoporosis, symptomatic Paget disease of bone, osteogenesis imperfecta both in children and adults and fibrous dysplasia are currently the major indications for bisphospho-nates. In the future, they might be used in rheumatoid arthritis and osteoarthritis(3).

Pamidronate is poorly absorbed and may have a local irritant effect on the upper gastrointestinal tract(1). The main published studies describe the effects of intravenous pamidronate at a daily dose of 1.5-3.0 mg/kg body weight for three days every four to six months for 1-5 years in children with severe osteogenesis imperfecta(3,4). In fibrous dysplasia it has been used by infusion (two courses per year) with good results with respect to pain and osteolytic lesion(3). The cost of the drug, its intravenous route of administration and hence the necessity for hospitalization significantly reduces the usage of the bisphosphonates in India.

Alendronate is at present indicated for use in prevention and treatment of osteoporosis and Paget’s disease. It should be used in conjunction with adequate amounts of vitamin D and calcium in the prevention of progressive loss of bone mass. As yet very few studies have looked at the use of oral alendronate in resorptive bone disorders in children(5). Our patient with fibrous dysplasia had an improvement in BMD and marked reduction in bone pain after 18 months on treatment with oral alendronate. Based on these early observations we feel that clinical trials of oral bisphosphonates should be carried out so that its safety and efficacy in children can be evaluated.

Contributors: VVK and AVK planned and conducted the study: AVK and GBM collected the data and drafted the manuscript. VVK will act as guarantor of the study.

Funding: HCJMRI, Jehangir Hospital, Pune.

Competing interests: None stated.


1. Krane S. Paget’s disease of the bone: In: Isselbacher KJ, Braundwald E, Wilson JD, Martin JB, Fauci AS, Dennis LK, (Eds). Harrison’s Principles of Internal Medicine, 13th edn. New York: McGraw-Hill Inc 1994; 2190-2192.

2. Chapurlat RD, Meunier PJ. Fibrous dysplasia of bone. Baillieres Best Res Clin Rheumatol 2000; 14: 385-398.

3. Devogelaer JP. Treatment of bone diseases with bisphosphonates, excluding osteoporosis. Curr Opin Rheumatol 2000; 12: 331-335.

4. Glorieux FH, Bishop NJ, Plotkin H, Chabot G, Lanoue G, Travers R. Cyclic administration of pamidronate in children with severe osteogenesis imperfecta. N Engl J Med 1998; 39: 947-952.

5. Camacho NP, Raggio CL, Doty SB, Root L, Zraick V, Ilg WA, et al. A controlled study of the effects of alendronate in a growing mouse model of osteogenesis imperfecta. Calcif Tissue Int 2001. 69: 94-101.


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