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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
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CASE REPORT
Year : 2005  |  Volume : 10  |  Issue : 1  |  Page : 50-51
 

Intramural ureteric calcification in dermatomyositis mimicking pelviureteric junction obstruction


1 Paediatric Endoscopic Surgeon and Urologist, Ahmedabad, India
2 Birmingham Children's Hospital, Birmingham, United Kingdom

Correspondence Address:
Amar Shah
Paediatric Endoscopic Surgeon and Urologist, Ahmedabad
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.16078

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   Abstract 

Juvenile dermatomyositis classically manifests as necrotising vasculitis in multiple organs. It mainly involves the vessels of skin and muscle and is associated with subcutaneous deposits of calcium. Ureteric involvement in dermatomyositis is extremely rare. The authors present a case of a 14-year-old girl with juvenile dermatomyositis with intramural ureteric calcification mimicking pelviureteric junction (PUJ) obstruction.


Keywords: Dermatomyositis, ureteric calcification, pelviureteric junction obstruction


How to cite this article:
Shah A, Thakur R, Parashar K. Intramural ureteric calcification in dermatomyositis mimicking pelviureteric junction obstruction . J Indian Assoc Pediatr Surg 2005;10:50-1

How to cite this URL:
Shah A, Thakur R, Parashar K. Intramural ureteric calcification in dermatomyositis mimicking pelviureteric junction obstruction . J Indian Assoc Pediatr Surg [serial online] 2005 [cited 2019 Sep 20];10:50-1. Available from: http://www.jiaps.com/text.asp?2005/10/1/50/16078


Juvenile onset dermatomyositis is an idiopathic inflammatory myopathy that is first detected in childhood or adolescence. It is rare and affects 1.9 children per million in United Kingdom and Ireland.[1] The estimated incidence in the United States ranged from 2.5 to 4.1 per million. The estimated annual incidence rates were 3.4 for white non-Hispanics, 3.3 for African American non-Hispanics and 2.7 for Hispanics. Girls are affected more than boys (2.3:1).[2]

It is characterized by a rash accompanying or more often preceding muscle weakness. Calcification is the most characteristic soft tissue abnormality in dermatomyositis and is manifested by calcareous deposits in the intermuscular fascial planes or subcutaneous tissue. We report a rare case of dystrophic intramural ureteric calcification causing symptomatic obstruction.


   Case Report Top


A 14-year-old girl with juvenile dermatomyositis was referred with chronic right loin pain. On clinical examination she had characteristic cutaneous eruptions and muscle weakness. There was some fullness in the right loin. An ultrasound scan showed a right hydronephrosis, with no dilatation of the ureter. A MAG3 scan showed decreased function with poor drainage on the right. She was being treated with cyclophosphamide, methotrexate, mesna, steroids and local applications. Due to the severity of her overall condition it was decided to manage the PUJ obstruction by the placement of a JJ stent. This relieved her loin pain. Repeat scans showed a reduction in hydronephrosis and improved renal drainage. Six months after the procedure she was readmitted for recurrent right loin pain. An X-ray showed calcification around the JJ stent. Replacement of the JJ stent proved impossible. Hence an emergency exploration was carried out. At operation there was narrowing of the ureter just below the PUJ. The pelviureteric junction was itself patent, but surrounded by a severe inflammatory reaction. The child underwent a dismembered pyeloplasty with excision of the upper ureteric segment. Post-operative recovery was uneventful. Histopathology of the upper ureter showed ulceration with marked fibrosis disrupting muscle coat, accompanied by abundant calcific debris in the lumen and sub epithelial stroma [Figure - 1]. Follow up scans 6 months after the procedure showed mild dilatation of the renal calyces with good drainage and function.


   Discussion Top


Juvenile dermatomyositis is a multi system disease characterised by necrotising vasculitis in multiple organs. It is probably the result of a humoral attack on the muscle capillaries and small arterioles. As the disease progresses, the capillaries are destroyed and the muscles undergo microinfarction. Dystrophic calcification occurs in 10-50% of affected patients.[3],[4],[5]

In our patient, intramural ureteric calcification was the cause of the ureteric obstruction causing hydronephrosis and loin pain. Ureteric calcification has been described before.[6],[7] However, this has always been in the middle and lower thirds. Interestingly in our patient, the upper ureter was the area that was affected and calcification was noticeable on plain X-ray.

Dystrophic calcification in dermatomyositis can affect the ureter. It can and does cause obstruction severe enough to affect the function of the relevant kidney. This can be managed temporarily by JJ stenting. Operative repair in the form of pyeloplasty or excision and uretero-ureteric anastomosis though difficult is successful in preserving renal function.

 
   References Top

1.Symmons DP, Sils JA, Davis SM. The incidence of juvenile dermatomyositis: Results from a nationwide study. Br J Rheumatol 1995;34:732-6.  Back to cited text no. 1    
2.Mendex EP, Lipton R, Ramsey-Goldman R, Roettcher P, Bowyer S, Dyer A, et al . US incidence of juvenile dermatomyositis, 1995-1998: Results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases Registry. Arthritis Rheum 2003;49:300-5.  Back to cited text no. 2    
3.Ramanan AV, Feldman BM. Clinical features and outcomes of juvenile dermatomyositis and other childhood onset myositis syndromes. Rheum Dis Clin North Am 2002;28:833-58.  Back to cited text no. 3    
4.Fisler RE, Liang MG, Fuhlbrigge RC, Yalcindag A, Sundel RP. Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis. J Am Acad Dermatol 2002;47:505-11.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Bowyer SL, Blane CE, Sullivan DB, Cassidy JT. Childhood dermatomyositis: Factors predicting functional outcome and development of dystrophic calcification. J Pediatr 1983;103:882-8.  Back to cited text no. 5  [PUBMED]  
6.Borrelli M, Prado MJ, Cordeiro P, Wroclawski ER, Monteiro J Jr, Kiss MH, et al . Ureteral necrosis in dermatomyositis. J Urol 1988;139:1275-7.  Back to cited text no. 6    
7.Blery M, Lacert P, Touboul A. Lithiasis and bilateral necrosis of the ureters occurring during dermatomyositis. A definite entity or a coincidence? J Radiol Electrol Med Nucl 1978;59:279-82.  Back to cited text no. 7  [PUBMED]  


Figures

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  2005 - Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow 

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