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Severe kyphoscoliosis associated with osteomalacia
The Spine Journal 6 (2006) 587–590
Case Study
Severe kyphoscoliosis associated with osteomalacia Takao Motosuneya, MD*, Takashi Asazuma, MD, Hiroki Yasuoka, MD, Takashi Tsuji, MD, Kyosuke Fujikawa, MD Department of Orthopaedic Surgery, National Defense Medical College, 3-2, Namiki, Tokorozawa, Saitama, 359-8513, Japan Received 7 July 2005; accepted 14 January 2006
Abstract
BACKGROUND CONTEXT: Kyphoscoliosis is one of the most frequent complications of osteomalacia, which only rarely results in severe deformity requiring surgery. To the best of our knowledge, there has been only one previous report of a spinal deformity as a complication of osteomalacia that was sufficiently severe so as to require surgical treatment. PURPOSE: To report here the case of a 27-year-old woman who experienced back pain of gradual onset accompanied by progressive scoliosis resulting in severe dyspnea. STUDY DESIGN: A case report. METHODS: She was diagnosed with hypophosphatemic osteomalacia and secondary hyperparathyroidism. She underwent posterior surgical correction and fusion from Th4–L1 using the ISOLA spinal system. RESULTS: At the last follow-up (3 year and 9 months postoperatively), her body balance was good and the dyspnea had disappeared. Plain radiographs demonstrated no loss of correction and also showed no evidence of instrumentation failure. CONCLUSIONS: We present a unique instance of a young woman with severe kyphoscoliosis who underwent posterior surgical correction/fusion with spinal instrumentation. Ó 2006 Elsevier Inc. All rights reserved.
Keywords:
Osteomalacia; Scoliosis; Kyphosis; Posterior fusion
Introduction Kyphoscoliosis is one of the most frequent complications of osteomalacia, although severe deformity that needs surgical treatment is extremely rare. To the best of our knowledge, there has been only one report of a case requiring surgical treatment for spinal deformity as a complication of osteomalacia [1]. However, that case was essentially different from our case, because it was associated with neurofibromatosis 1 (NF1). Our case was extremely unique because she suffered from an onset of adult-developed osteomalacia and was not ever diagnosed with NF1. Spinal surgery in someone with osteomalacia carries a potentially greater risk of hazards such as instrumentation failure, loss FDA device/drug status: not applicable. Nothing of value received from a commercial entity related to this manuscript. * Corresponding author. Department of Orthopaedic Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama 359-8513, Japan. Tel.: þ42-995-1663; fax: þ42-996-5208. E-mail address: [email protected] (T. Motosuneya) 1529-9430/06/$ – see front matter Ó 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.spinee.2006.01.008
of correction, and nonunion. We present a unique instance of a young woman with severe kyphoscoliosis who underwent posterior surgical correction/fusion with spinal instrumentation. Case report A 27-year-old woman experienced back pain of gradual onset first noticed in 1992. Her initial complaint was the inability to run continuously. She was examined in several hospitals without a diagnosis. In 1999 a spinal deformity was noticed and she presented to our clinic again. We followed her as an outpatient and noted that the scoliosis continued to progress and that dyspnea appeared. She was admitted to our clinic for surgical treatment of spinal deformity in January 2002. She was 4 feet 4 inches tall and weighed 67.8 pounds. She was not able to walk more than a few steps because of her inability to maintain body balance because of an off-axis shift of her center-of-gravity due to the spinal deformity. Back examination revealed thoracic
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T. Motosuneya et al. / The Spine Journal 6 (2006) 587–590
kyphoscoliosis, and the deformity of her thoracic cage was remarkable. She complained of general arthralgia with motion but experienced no rest pain or night pain. Neurological examination revealed whole limb muscle weakness, although sensory disturbance and hyperreflexia were not observed. Similar cases were not observed in her family. Serial plain radiographs of the spine showed that the kyphoscoliosis was progressive. Right thoracic Cobb angle from T5 to T11 advanced from 42 degrees to 82 degrees over the preceding 2 years 4 months, and the kyphosis angle from Th4 to Th9 was 66 (Fig. 1). Bending test showed that the scoliosis angle improved less than 30 with right bending, and the spine was defined as rigid. Plain radiographs of both hip joints showed narrowing of joint space, and an obvious varus deformity at the neck of each femur was observed (Fig. 2A). Old fractures and deformities were observed at the pubis and sacrum (Fig. 2B). Serum phosphorus and alkaline phosphatase levels were 2.2 mg/dL (normal range, 2.5–4.7 mg/dL) and 1,357 IU/L (normal range, 100–340 IU/L), respectively. Intact parathyroid hormone was 190 pg/mL (normal range, 10–65 pg/mL). Urinary type I collagen cross-linked N-telopeptide (NTx)/ Cr was 249.7 nmol BCE (bone collagen equivalents)/mmol. Creatinine (normal range, 8.3–69.9 nmol BCE/mmol_ Creatinine). The other data, both in serum and urine, were within normal limits. According to these data, osteomalacia with hypophosphatemia and secondary hyperparathyroidism were diagnosed. Her bone marrow density was extremely decreased to 0.450 g/cm2. Spirometry showed that her vital capacity deteriorated to 41.6%. In February 2002, a posterior correction and fusion using the ISOLA spinal system was performed at Th4–L1
levels. Postoperatively, the scoliosis and kyphosis angles improved to 42 and 52 , respectively (correction rate 48.8% and 21.2%) (Fig. 3). Laminar bone and marrow cells were observed in medullary cavity on histological sections from iliac bone and vertebrae. One week after the operation, she started training to stand with a hard orthosis, and at 2 weeks she attempted to ambulate with a walker. At the last follow-up (3 years and 9 months postoperatively), her body balance was remarkably improved and the dyspnea she had complained of before surgery had disappeared. Plain radiographs showed neither loss of correction nor instrumentation failure. Phosphate from her diet, as well as active vitamin D and bisphosphonate, have all also helped to treat her osteomalacia. Fortunately, her serous phosphorus and alkaline phosphatase levels have approached more normal levels (2.6 mg/dL and 309 IU/L, respectively) and her bone marrow density has improved to 0.864 g/cm2.
Discussion Osteomalacia is a failure to mineralize the newly formed organic matrix (osteoid) of bone in a normal manner. In children, the term rickets is used to indicate a disorder in which there is epiphyseal dysplasia, retardation of longitudinal growth of long bones, and a variety of skeletal deformities. The manifestations of rickets depends not only upon the severity and duration of the underlying disorder but to a great extent upon the patient’s age [2,3]. The causes of osteomalacia are classified as vitamin D deficiency, disorders of vitamin D metabolism, peripheral
Fig. 1. (A) Right thoracic Cobb angle from T5 to T11 was 82 . (B) Hypnosis angle from Th4 to Th9 was 66 .
T. Motosuneya et al. / The Spine Journal 6 (2006) 587–590
589
Fig. 2. (A) Plain radiographs of bilateral hip joints revealed irregular joint surface, and an obvious varus deformity at the neck of both femora was observed (white arrow). (B) Old fractures and deformity were observed at the pubis and sacrum (white arrow).
resistance to vitamin D, and hypophosphatemic vitamin D resistant rickets [3]. In our case, serum phosphorus level was low, calcium was normal, and parathyroid hormone was high. She was diagnosed as having osteomalacia with hypophosphatemia and secondary hyperparathyroidism. In adult-onset disease, skeletal deformities are seldom encountered except in the most severe cases. Bone pain is common in both children and adults. It is often experienced in the pelvis, the lower extremities, the spine, and the ribs.
In severe cases, bone tenderness is present upon palpation [3]. The pathognomonic radiological feature of osteomalacia is the presence of microfractures, also known as Looser zones. These narrow zones of rarefaction are often bilateral and symmetric [2,3]. Hypophosphatemic osteomalacia is treated slightly differently in children and in adults. In children, the aims of therapy with osteomalacia are to correct deformity, to minimize prolonged symptoms later in life, and to increase the
Fig. 3. (A) Postoperative scoliosis angle improved to 42 . (B) Postoperative kyphosis angle improved to 52 .
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T. Motosuneya et al. / The Spine Journal 6 (2006) 587–590
child’s ultimate height. Although it has been difficult to document this objectively, combination therapy with vitamin D and phosphate appears to help the deformity, because few patients require surgical intervention, whereas before, with the introduction of combination therapy, the majority of such patients required osteotomies [4]. However, therapy for adult patients is somewhat controversial. There is an opinion that vitamin D has been ineffective [2], whereas others still suggest that patients should take vitamin D and adequate amounts of phosphorus [3]. In our case, the patient included phosphate in her diet, as well as vitamin D and bisphosphonate, which did improve her bone density [5]. The weakened vertebral bodies allow ballooning of the nuclei pulposi into the classical biconcave ‘‘codfish’’ deformities of the intervertebral disc spaces. Kyphoscoliosis is one of the most frequent complications of osteomalacia. The odds ratio of asymmetric posture was 2.76 for children with rickets [6], but the clinical findings related to the spine are usually not as striking as they are in the long bones. These conditions seldom are allowed to progress sufficiently to cause a severe degree of kyphosis, and severe deformity that requires surgical treatment is extremely rare [7]. The association between osteomalacia and coronal plane deformity is not clear. To the best of our knowledge, there is only one previous case that needed surgical treatment for spinal deformity as a complication of osteomalacia. Abdel-Wanis et al. reported the case of coexisting NF1 and primary hyperparathyroidism and osteomalacia [1]. However, that case was essentially different from our case, because it was associated with NF1. Our case was extremely unique because the patient suffered from an onset of adult-developed osteomalacia and was not ever diagnosed with NF1. In our case, the Cobb angle was 82 , indicating a severe deformity, and the bending test indicated that the spine was defined as rigid. Furthermore, because osteomalacia-
induced osteoporosis was quite severe as well, the procedure and technique of the surgical intervention had to be performed carefully and meticulously. More specifically, we released the concave side thoroughly and corrected the deformity with a gentle maneuver. We also obtained grafted bone from both iliac bones because the bone stock was expected to be poor. There had been concern that a hook or rod might become displaced, but fortunately, instrumentation failure was not seen at the time of last follow-up. In addition, her body balance improved and the dyspnea she had complained of before surgery disappeared. However, careful follow-up is required because of the obvious varus deformity at the neck of the femur observed and because she needs crutches for walking.
References [1] Abdel-Wanis ME, Kawahara N, Tomita K. The association of neurofibromatosis 1 and spinal deformity with primary hyperparathyroidism and osteomalacia; might melatonin have a role? J Orthop Sci 2001;6:193–8. [2] Goldring SR, Krane SM, Avioli LV. Disorders of calcification: osteomalacia and rickets. In: DeGroot LJ, Jameson JL, editors. Endocrinology. 14th ed. Philadelphia: WB Saunders, 2001:1223–43. [3] Frederick RS. Metabolic bone disease: osteomalacia and rickets. In: Felig P, Baxter JD, Broadus AE, editors. Endocrinology and metabolism. New York: McGraw-Hill, 1981:1085–93. [4] Verge CF, Lam A, Simpson JM, Cowell CT, Howard NJ, Silink M. Effects of therapy in X-linked hypophosphatemic rickets. N Engl J Med 1991;325:1843–8. [5] Chattopadhyay A, Bhansali A, Mohanty SK, Khandelwal N, Mathur SK, Dash RJ. Hypophosphatemic rickets and osteomalacia in polyostotic fibrous dysplasia. J Pediatr Endocrinol Metab 2003;16:893–6. [6] Juskeliene V, Magnus P, Bakketeig L, Dailidiene N, Jurkuvenas V. Prevalence and risk factors for asymmetric posture in preschool children aged 6–7 years. Int J Epidemiol 1996;25:1053–9. [7] Hensinger RN. Hypnosis secondary to skeletal dysplasias and metabolic disease. Clin Orthop 1977;128:113–28.
Case Study
Severe kyphoscoliosis associated with osteomalacia Takao Motosuneya, MD*, Takashi Asazuma, MD, Hiroki Yasuoka, MD, Takashi Tsuji, MD, Kyosuke Fujikawa, MD Department of Orthopaedic Surgery, National Defense Medical College, 3-2, Namiki, Tokorozawa, Saitama, 359-8513, Japan Received 7 July 2005; accepted 14 January 2006
Abstract
BACKGROUND CONTEXT: Kyphoscoliosis is one of the most frequent complications of osteomalacia, which only rarely results in severe deformity requiring surgery. To the best of our knowledge, there has been only one previous report of a spinal deformity as a complication of osteomalacia that was sufficiently severe so as to require surgical treatment. PURPOSE: To report here the case of a 27-year-old woman who experienced back pain of gradual onset accompanied by progressive scoliosis resulting in severe dyspnea. STUDY DESIGN: A case report. METHODS: She was diagnosed with hypophosphatemic osteomalacia and secondary hyperparathyroidism. She underwent posterior surgical correction and fusion from Th4–L1 using the ISOLA spinal system. RESULTS: At the last follow-up (3 year and 9 months postoperatively), her body balance was good and the dyspnea had disappeared. Plain radiographs demonstrated no loss of correction and also showed no evidence of instrumentation failure. CONCLUSIONS: We present a unique instance of a young woman with severe kyphoscoliosis who underwent posterior surgical correction/fusion with spinal instrumentation. Ó 2006 Elsevier Inc. All rights reserved.
Keywords:
Osteomalacia; Scoliosis; Kyphosis; Posterior fusion
Introduction Kyphoscoliosis is one of the most frequent complications of osteomalacia, although severe deformity that needs surgical treatment is extremely rare. To the best of our knowledge, there has been only one report of a case requiring surgical treatment for spinal deformity as a complication of osteomalacia [1]. However, that case was essentially different from our case, because it was associated with neurofibromatosis 1 (NF1). Our case was extremely unique because she suffered from an onset of adult-developed osteomalacia and was not ever diagnosed with NF1. Spinal surgery in someone with osteomalacia carries a potentially greater risk of hazards such as instrumentation failure, loss FDA device/drug status: not applicable. Nothing of value received from a commercial entity related to this manuscript. * Corresponding author. Department of Orthopaedic Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama 359-8513, Japan. Tel.: þ42-995-1663; fax: þ42-996-5208. E-mail address: [email protected] (T. Motosuneya) 1529-9430/06/$ – see front matter Ó 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.spinee.2006.01.008
of correction, and nonunion. We present a unique instance of a young woman with severe kyphoscoliosis who underwent posterior surgical correction/fusion with spinal instrumentation. Case report A 27-year-old woman experienced back pain of gradual onset first noticed in 1992. Her initial complaint was the inability to run continuously. She was examined in several hospitals without a diagnosis. In 1999 a spinal deformity was noticed and she presented to our clinic again. We followed her as an outpatient and noted that the scoliosis continued to progress and that dyspnea appeared. She was admitted to our clinic for surgical treatment of spinal deformity in January 2002. She was 4 feet 4 inches tall and weighed 67.8 pounds. She was not able to walk more than a few steps because of her inability to maintain body balance because of an off-axis shift of her center-of-gravity due to the spinal deformity. Back examination revealed thoracic
588
T. Motosuneya et al. / The Spine Journal 6 (2006) 587–590
kyphoscoliosis, and the deformity of her thoracic cage was remarkable. She complained of general arthralgia with motion but experienced no rest pain or night pain. Neurological examination revealed whole limb muscle weakness, although sensory disturbance and hyperreflexia were not observed. Similar cases were not observed in her family. Serial plain radiographs of the spine showed that the kyphoscoliosis was progressive. Right thoracic Cobb angle from T5 to T11 advanced from 42 degrees to 82 degrees over the preceding 2 years 4 months, and the kyphosis angle from Th4 to Th9 was 66 (Fig. 1). Bending test showed that the scoliosis angle improved less than 30 with right bending, and the spine was defined as rigid. Plain radiographs of both hip joints showed narrowing of joint space, and an obvious varus deformity at the neck of each femur was observed (Fig. 2A). Old fractures and deformities were observed at the pubis and sacrum (Fig. 2B). Serum phosphorus and alkaline phosphatase levels were 2.2 mg/dL (normal range, 2.5–4.7 mg/dL) and 1,357 IU/L (normal range, 100–340 IU/L), respectively. Intact parathyroid hormone was 190 pg/mL (normal range, 10–65 pg/mL). Urinary type I collagen cross-linked N-telopeptide (NTx)/ Cr was 249.7 nmol BCE (bone collagen equivalents)/mmol. Creatinine (normal range, 8.3–69.9 nmol BCE/mmol_ Creatinine). The other data, both in serum and urine, were within normal limits. According to these data, osteomalacia with hypophosphatemia and secondary hyperparathyroidism were diagnosed. Her bone marrow density was extremely decreased to 0.450 g/cm2. Spirometry showed that her vital capacity deteriorated to 41.6%. In February 2002, a posterior correction and fusion using the ISOLA spinal system was performed at Th4–L1
levels. Postoperatively, the scoliosis and kyphosis angles improved to 42 and 52 , respectively (correction rate 48.8% and 21.2%) (Fig. 3). Laminar bone and marrow cells were observed in medullary cavity on histological sections from iliac bone and vertebrae. One week after the operation, she started training to stand with a hard orthosis, and at 2 weeks she attempted to ambulate with a walker. At the last follow-up (3 years and 9 months postoperatively), her body balance was remarkably improved and the dyspnea she had complained of before surgery had disappeared. Plain radiographs showed neither loss of correction nor instrumentation failure. Phosphate from her diet, as well as active vitamin D and bisphosphonate, have all also helped to treat her osteomalacia. Fortunately, her serous phosphorus and alkaline phosphatase levels have approached more normal levels (2.6 mg/dL and 309 IU/L, respectively) and her bone marrow density has improved to 0.864 g/cm2.
Discussion Osteomalacia is a failure to mineralize the newly formed organic matrix (osteoid) of bone in a normal manner. In children, the term rickets is used to indicate a disorder in which there is epiphyseal dysplasia, retardation of longitudinal growth of long bones, and a variety of skeletal deformities. The manifestations of rickets depends not only upon the severity and duration of the underlying disorder but to a great extent upon the patient’s age [2,3]. The causes of osteomalacia are classified as vitamin D deficiency, disorders of vitamin D metabolism, peripheral
Fig. 1. (A) Right thoracic Cobb angle from T5 to T11 was 82 . (B) Hypnosis angle from Th4 to Th9 was 66 .
T. Motosuneya et al. / The Spine Journal 6 (2006) 587–590
589
Fig. 2. (A) Plain radiographs of bilateral hip joints revealed irregular joint surface, and an obvious varus deformity at the neck of both femora was observed (white arrow). (B) Old fractures and deformity were observed at the pubis and sacrum (white arrow).
resistance to vitamin D, and hypophosphatemic vitamin D resistant rickets [3]. In our case, serum phosphorus level was low, calcium was normal, and parathyroid hormone was high. She was diagnosed as having osteomalacia with hypophosphatemia and secondary hyperparathyroidism. In adult-onset disease, skeletal deformities are seldom encountered except in the most severe cases. Bone pain is common in both children and adults. It is often experienced in the pelvis, the lower extremities, the spine, and the ribs.
In severe cases, bone tenderness is present upon palpation [3]. The pathognomonic radiological feature of osteomalacia is the presence of microfractures, also known as Looser zones. These narrow zones of rarefaction are often bilateral and symmetric [2,3]. Hypophosphatemic osteomalacia is treated slightly differently in children and in adults. In children, the aims of therapy with osteomalacia are to correct deformity, to minimize prolonged symptoms later in life, and to increase the
Fig. 3. (A) Postoperative scoliosis angle improved to 42 . (B) Postoperative kyphosis angle improved to 52 .
590
T. Motosuneya et al. / The Spine Journal 6 (2006) 587–590
child’s ultimate height. Although it has been difficult to document this objectively, combination therapy with vitamin D and phosphate appears to help the deformity, because few patients require surgical intervention, whereas before, with the introduction of combination therapy, the majority of such patients required osteotomies [4]. However, therapy for adult patients is somewhat controversial. There is an opinion that vitamin D has been ineffective [2], whereas others still suggest that patients should take vitamin D and adequate amounts of phosphorus [3]. In our case, the patient included phosphate in her diet, as well as vitamin D and bisphosphonate, which did improve her bone density [5]. The weakened vertebral bodies allow ballooning of the nuclei pulposi into the classical biconcave ‘‘codfish’’ deformities of the intervertebral disc spaces. Kyphoscoliosis is one of the most frequent complications of osteomalacia. The odds ratio of asymmetric posture was 2.76 for children with rickets [6], but the clinical findings related to the spine are usually not as striking as they are in the long bones. These conditions seldom are allowed to progress sufficiently to cause a severe degree of kyphosis, and severe deformity that requires surgical treatment is extremely rare [7]. The association between osteomalacia and coronal plane deformity is not clear. To the best of our knowledge, there is only one previous case that needed surgical treatment for spinal deformity as a complication of osteomalacia. Abdel-Wanis et al. reported the case of coexisting NF1 and primary hyperparathyroidism and osteomalacia [1]. However, that case was essentially different from our case, because it was associated with NF1. Our case was extremely unique because the patient suffered from an onset of adult-developed osteomalacia and was not ever diagnosed with NF1. In our case, the Cobb angle was 82 , indicating a severe deformity, and the bending test indicated that the spine was defined as rigid. Furthermore, because osteomalacia-
induced osteoporosis was quite severe as well, the procedure and technique of the surgical intervention had to be performed carefully and meticulously. More specifically, we released the concave side thoroughly and corrected the deformity with a gentle maneuver. We also obtained grafted bone from both iliac bones because the bone stock was expected to be poor. There had been concern that a hook or rod might become displaced, but fortunately, instrumentation failure was not seen at the time of last follow-up. In addition, her body balance improved and the dyspnea she had complained of before surgery disappeared. However, careful follow-up is required because of the obvious varus deformity at the neck of the femur observed and because she needs crutches for walking.
References [1] Abdel-Wanis ME, Kawahara N, Tomita K. The association of neurofibromatosis 1 and spinal deformity with primary hyperparathyroidism and osteomalacia; might melatonin have a role? J Orthop Sci 2001;6:193–8. [2] Goldring SR, Krane SM, Avioli LV. Disorders of calcification: osteomalacia and rickets. In: DeGroot LJ, Jameson JL, editors. Endocrinology. 14th ed. Philadelphia: WB Saunders, 2001:1223–43. [3] Frederick RS. Metabolic bone disease: osteomalacia and rickets. In: Felig P, Baxter JD, Broadus AE, editors. Endocrinology and metabolism. New York: McGraw-Hill, 1981:1085–93. [4] Verge CF, Lam A, Simpson JM, Cowell CT, Howard NJ, Silink M. Effects of therapy in X-linked hypophosphatemic rickets. N Engl J Med 1991;325:1843–8. [5] Chattopadhyay A, Bhansali A, Mohanty SK, Khandelwal N, Mathur SK, Dash RJ. Hypophosphatemic rickets and osteomalacia in polyostotic fibrous dysplasia. J Pediatr Endocrinol Metab 2003;16:893–6. [6] Juskeliene V, Magnus P, Bakketeig L, Dailidiene N, Jurkuvenas V. Prevalence and risk factors for asymmetric posture in preschool children aged 6–7 years. Int J Epidemiol 1996;25:1053–9. [7] Hensinger RN. Hypnosis secondary to skeletal dysplasias and metabolic disease. Clin Orthop 1977;128:113–28.