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LETTERS TO EDITOR
Year : 2018  |  Volume : 66  |  Issue : 5  |  Page : 1510-1511

A rare case of Chiari malformation secondary to Paget's disease of skull


Department of Neurosurgery, Longyan First Hospital, Fujian Medical University, Longyan, Fujian, People's Republic of China

Date of Web Publication17-Sep-2018

Correspondence Address:
Dr. Jing Lu
Department of Neurosurgery, Longyan First Hospital, Fujian Medical University, No. 105 North 91 Road, Xinluo District, Longyan, 364000, Fujian
People's Republic of China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.241388

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How to cite this article:
Lin Z, Lan F, Shen D, Guan C, Qiu P, Gao Z, Lu J. A rare case of Chiari malformation secondary to Paget's disease of skull. Neurol India 2018;66:1510-1

How to cite this URL:
Lin Z, Lan F, Shen D, Guan C, Qiu P, Gao Z, Lu J. A rare case of Chiari malformation secondary to Paget's disease of skull. Neurol India [serial online] 2018 [cited 2018 Oct 23];66:1510-1. Available from: http://www.neurologyindia.com/text.asp?2018/66/5/1510/241388




Sir,

A 76-year old man presented with increasing head circumference with otherwise normal neurological findings. Physical examination revealed a striking deformity of the skull with a maximum circumference of 68 cm. His serum alkaline phosphatase (ALP) level was elevated at 322 IU/L (normal 45–125 IU/L). The serum calcium and intact parathyroid hormone levels were within normal limits. The hemogram parameters, such as white blood cell count, red blood cell count and hemoglobin were also within the normal range. Computed tomography (CT) scans disclosed marked thickening of the bone, widening of the diploe, and diffuse thickening of the skull base [Figure 1]a and b]. Sagittal T2-weighted images revealed widening of the diploic space, distortion and flattening of the cerebral and cerebellar hemispheres, platybasia with basilar invagination, reduction of the volume of the posterior fossa, and 10 mm downward displacement of the cerebellar tonsils into the foramen magnum [Figure 1]c. Review of magnetic resonance (MR) imaging of the head obtained 3 years earlier had demonstrated mild  Chiari malformation More Details with only 4 mm cerebellar tonsillar descent into the upper cervical canal [Figure 2]. A progressive Chiari malformation secondary to skull Paget's disease was suggested. The following day, a discussion was held with the patient and his family regarding the management options. He was treated with biphosphonate to prevent progression of the neurological involvement; a long-term follow-up was required to assess the improvement in the condition.
Figure 1: CT bone windows (a and b) showed marked thickening of the bone, widening of the diploe, and diffuse thickening of the skull base. Sagittal T2 weighted images (c) revealed widening of the diploic space, distortion and flattening of the cerebral and cerebellar hemispheres, platybasia with basilar invagination, reduction of the volume of the posterior fossa, and 10 mm downward displacement of the cerebellar tonsils into the foramen magnum

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Figure 2: A sagittal T2-weighted MR image obtained from the patient at 73 years of age revealing 4 mm of cerebellar tonsillar herniation below the foramen magnum

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Paget's disease of the bone, also called as “osteitis deformans,” was first described in 1877 by Sir James Paget, and the skull was involved in approximately half of patients with the disease.[1] It is a chronically progressive monostotic or polyostotic disorder characterized by rapid bone resorption and deposition, resulting in a “mosaic” pattern of lamellar bone with extensive local vascularity and fibrous tissue in the bone marrow. It occurs rarely in the Asians and Africans, while it is a common entity among the elderly Caucasian populations of Europe, North America, and Australia.[2] The etiology of Paget's disease is not completely understood.

Skull Paget's disease should be differentiated from skull erythropoiesis secondary to congenital dyserythropoietic anemia (CDA). CDAs are a group of rare hereditary disorders characterized by ineffective erythropoiesis. MR images also show widening of the diploic space and displacement of the outer table of the involved skull. However, these changes in the skull are caused by increased medullary erythropoiesis and marrow hyperplasia in response to chronic anemia. Apart from a history of CDA, a decreased hemoglobin concentration, and a normal serum alkaline phosphatase (ALP) is the norm. In occasional cases, the trabeculae extend perpendicularly between the inner and outer skull tables through the diploe in the expanded bone marrow space, producing a “hair-on-end” appearance on X-ray radiography.[3]

Chiari malformation constitutes caudal displacement of the cerebellar tonsils below the foramen magnum, commonly described as a congenital anomaly secondary to the abnormal development of the posterior fossa.[4] Acquired Chiari malformation has been previously described after lumbar puncture/drainage, lumboperitoneal shunts, and spontaneous cerebrospinal fluid leakage as well as pseudotumor cerebri, craniosynostosis, achondroplasia, and other conditions that cause cranioencephalic disproportion.

Skull Paget's disease is a rare cause of acquired Chiari malformation. To the best of our knowledge, there were only three cases of skull Paget's disease associated Chiari malformation reported in the English literature.[5],[6],[7] [Table 1] summarizes the data of the patients detailed in each article. Despite of its relative rarity, it has been well established that the formation of acquired Chiari malformation has been attributable to the Paget's disease in the skull. Overgrowth and softening of the skull base has led to platybasia and basilar impression. The small posterior fossa predisposed to the overcrowding and subsequent herniation of the cerebellar tonsils into the upper cervical spine.
Table 1: Review of the literature of Chiari malformation caused by skull Paget's disease

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Although the signs and symptoms of congenital and acquired Chiari malformation may be indistinguishable, differentiating between the two types has important therapeutic implications. Congenital Chiari malformation is best treated with suboccipital decompression, whereas an acquired lesion may be managed by treating the primary disease first. In our case, the skull Paget's disease and the Chiari malformation were coincidentally found on neuroimaging. However, they were not two completely separate entities and their pathogenesis were associated. The Chiari malformation was noted to develop on serial images, and resulted from basilar impression, causing progressive reduction of the volume of the posterior fossa [Figure 1] and [Figure 2].{Figure 1}{Figure 2}

We present the case of an acquired Chiari malformation secondary to Paget's disease of the skull. The pathophysiological mechanisms mentioned above were thought to account for this lesion. Management of the skull Paget's disease prevented the progressive descent of the tonsillar herniation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Paget J. On a form of chronic inflammation of bones (osteitis deformans). Med Chir Trans 1877;60:37-64.  Back to cited text no. 1
    
2.
Ankrom MA, Shapiro JR. Paget's disease of bone (osteitis deformans). J Am Geriatr Soc 1998;46:1025-33.  Back to cited text no. 2
    
3.
Pérez-Jacoiste Asín MA, Ruiz Robles G. Skull erythropoiesis in a patient with congenital dyserythropoietic anaemia. Lancet 2016;387:787.  Back to cited text no. 3
    
4.
Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, et al. The natural history of the Chiari type I anomaly. J Neurosurg Pediatr 2008;2:179-87.  Back to cited text no. 4
    
5.
Iglesias-Osma C, Gomez Sanchez JC, Suquia Mugica B, Querol Prieto R, de Portugal Alvarez J. Paget's disease of bone and basilar impression associated with an Arnold-Chiari type-1 malformation. An Med Interna 1997;14:519-22.  Back to cited text no. 5
    
6.
Richards PS, Bargiota A, Corrall RJ. Paget's disease causing an Arnold-Chiari Type 1 malformation: Radiographic findings. Am J Roentgenol 2001;176:816-7.  Back to cited text no. 6
    
7.
Otsuka F, Inagaki K, Suzuki J, Ogura T, Makino H. Skull Paget's disease developing into Chiari malformation. Endocr J 2004;51:391-2.  Back to cited text no. 7
    


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