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 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 3  |  Issue : 2  |  Page : 142-146

Calcinosis of the scrotum in children: Report of two cases and review of the literature


1 Murtala Muhammad Specialist Hospital, Department of Surgery, Children Surgical Unit, Kano, Nigeria
2 Aminu Kano Teaching Hospital and Bayero University, Surgical Department, Kano, Nigeria
3 Scientific Centre of Pediatrics and Child Surgery, Surgical Department, Almaty, Kazakhstan
4 National Republican Centre for Mother and Child Health, Surgical Department, Astana, Kazakhstan

Date of Web Publication13-Dec-2013

Correspondence Address:
Musa Ibrahim
Department of Surgery, Children Surgical Unit, Murtala Muhammad Specialist Hospital, Kano
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.122938

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  Abstract 

Scrotal calcinosis consisting of solitary or multiple deposits of calcium within or outside the dermis of the skin of scrotum are generally very rare and are considered to be idiopathic if it is not of metastatic or dystrophic types. Idiopathic calcinosis of the scrotum (ICS) in children under the age of 9 years is not documented in the literature. We are presenting a 6-month-old infant and a 5-year-old male patient with sub-dermal, intra-scrotal, extra-testicular ICS from children surgical unit of Murtala Muhammad Specialist Hospital, Kano in northern Nigeria. Each patient presented with a scrotal mass that was difficult to diagnose clinically. Following investigations including scrotal ultrasound, and serum calcium, scrotal exploration was carried out and the mass excised. Histological examination revealed features of scrotal calcinosis. The objective of this study was to present the clinical presentation, investigation, and treatment of these children. We shall also under-take a review of the literature.

Keywords: Calcinosis, dartos muscle/fascia, idiopathic, intra-scrotal, sub-dermal


How to cite this article:
Ibrahim M, Ibrahim GK, Mohammad MA, Aji SA, Umar AB, Nurlan AN, Rassulbek AR. Calcinosis of the scrotum in children: Report of two cases and review of the literature. Arch Int Surg 2013;3:142-6

How to cite this URL:
Ibrahim M, Ibrahim GK, Mohammad MA, Aji SA, Umar AB, Nurlan AN, Rassulbek AR. Calcinosis of the scrotum in children: Report of two cases and review of the literature. Arch Int Surg [serial online] 2013 [cited 2024 Mar 1];3:142-6. Available from: https://www.archintsurg.org/text.asp?2013/3/2/142/122938


  Introduction Top


Idiopathic calcinosis of the scrotum (ICS) is a rare lesion that is characterized by multiple painless nodules of the scrotum. This lesion was reported as case reports in adults. [1],[2],[3],[4],[5],[6],[7] The only pediatric patient reported in English literature was a 9-year-old child. [8] In the classification of scrotal calcinosis, three forms were identified as metastatic if the patient has abnormal serum levels of calcium, phosphorous or both; the dystrophic if the serum levels of calcium and phosphorous are normal, but local condition such as infection, trauma or any other form of inflammation favors calcium deposit formation. The third form is when neither of the above applies and was called idiopathic. [9],[10] Though, many theories were postulated on the exact nature and pathogenesis of this entity, [11],[12],[13],[14] none is widely accepted. Some argued that, ICS is a late presentation of epidermal inclusion cysts that have undergone dystrophic changes in form of calcification, [15] while others considered the possible role of dartos muscle degeneration in the pathogenesis of ICS. [16] Due to its rare nature and uncertain pathogenesis, we are presenting the management of two pediatric patients with sub-dermal, intra-scrotal, extra-testicular scrotal calcinosis with destruction of the dartos fascia and the review of the literature.


  Case Reports Top


Case 1

A 6-month-old male infant was evaluated for right scrotal swelling. The swelling was noticed 1 month after birth which increased in size within 6 months of age. No birth trauma or neonatal infection history and the baby was delivered normally in a hospital following a normal term pregnancy.

Physical examination revealed a healthy child. Systemic examinations were normal. Local examination revealed a normal left scrotum containing a normal sized testis. The right scrotum was swollen with a shift of the midline raphe. The shaft of the penis was minimally buried within the swollen scrotum [Figure 1]a.
Figure 1:

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On palpation, there was a non-tender, firm, and irreducible mass within the right scrotum. Examination of inguinal regions did not reveal any abnormality. The luminescence test was negative. Regional inguinal lymph nodes were normal.

Clinical investigations including chest radiograph were normal. Blood biochemistry including serum calcium, phosphate, and serum protein was normal. Ultrasound scanning of the scrotum revealed calcified scrotal mass [Figure 1]b. A tentative diagnosis of right scrotal mass was made. Patient was prepared for surgical exploration with biopsy. An incision over the maximum area of bulging of the mass was made longitudinally. A semi-solid non-fatty mass mixed in a cystic component with multiple calcified materials was removed from within the scrotal space [Figure 2]a. Examination of the intrascrotal structures revealed a normal right testis while external spermatic fascia, internal spermatic fascia, parts of cremaster muscle could not be identified as they were part of the lesion. Tunica vaginalis and epididymis were well-defined and normal [Figure 2]b and c. The surgical wound was closed in layers. Histological examination of the excised mass revealed ICS [Figure 3]a and b. 4 years of clinical follow-up was uneventful.
Figure 2

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Figure 3

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Case 2

The records of this patient were retrieved and retrospectively studied. The patient was seen on the 14 th May, 2002 at the age of 8 months with right side scrotal swelling. No history of birth trauma and infections. Laboratory investigations including serum calcium and serum proteins were normal. A diagnosis of right sided resolving congenital hydroceles was made. The patient was operated on the 6 th July, 2007 at the age of 5 years. Under general anesthesia followed by routine surgical scrubbing of the surgical site, an incision measuring about 3 cm was made above the mass transversely. A solid, calcified mass adhered to the dermis of the skin was noted. Gentle mobilization of the mass was carried out. The mass was removed en block. A revision of the anatomical scrotal structures was done with inspection of the tunica vaginalis and an attempt to identify dartos structures could not reveal it. A normal tunica vaginalis with normal size testis was seen. The histological findings are shown in [Figure 3]c.


  Discussion Top


Scrotal Calcinosis is a benign, rare scrotal lesion with obscure etiology and pathogenesis mostly seen in adults and teenagers that causes embarrassment and social isolation. [17]

The first described calcinosis of the scrotum was reported by Lewinski in 1883 [18] followed by sporadic reports. However, Shapiro et al. [10] reviewed histopathologic data which they had reported earlier and they were unable to find any evidence of residual cysts, epithelial lining, lipid or organisms, as such they termed the entity as ICS. Subsequently, various reports emerged supporting or opposing Shapiro's definition.

There are reports suggesting the etiology and pathogenesis of calcified scrotal tissues. [16],[19] However, due to its rare nature, there is no uniform consensus classifying this entity. Shah and Shet in 2007 [20] analyzed 20 cases of scrotal calcinosis and found out that, three-fourth of the scrotal calcinosis cases were associated with calcification in the epithelial cysts as an etiology; thus the term ICS is best avoided.

In 1987, Kumagai et al. [21] suggested that, mast cell accumulation and its degranulation are the cause of ICS. In our two cases, we found degeneration of the dartos structures with fibrosis. These could be the leading factors of absence of such structures microscopically in some adult patients with long standing ailment. However, various theories have been advanced as to whether it is the degeneration of dartos structures that leads to calcinosis or vice versa.

Song et al. [22] described the theory and evolution of the disease according to their findings in 50 nodules. They suggested that, the first stage was the formation of the cyst followed by the calcification of the intracystic material and weakening of the cyst wall due to increase in size; rupture of the wall, mononuclear inflammation triggered by the intracystic content, which was exposed to the outside of the cyst; and finally, resorption of the cyst wall and the keratinous content leaving the calcified material only.

However in 2003, Pabuççuoglu et al.[16] speculated that, dartos muscle degeneration and necrosis were the most important factors in the pathogenesis of this disorder. The dartos muscle theory was also in accordance with King et al. [19] Feinstein et al. [23] thought that minor trauma played an important role as the starting point of dystrophic calcification. However, the present study showed that trauma is not a common occurrence as none of our patients had history of trauma which agrees with other studies. [24],[25] Saad and Zaatari in 2001 [26] hypothesized that, epithelial inclusion cysts constitute the basic abnormality in the formation of the disease and scrotal calcinosis is not idiopathic. This was also suggested previously in 1982 by Swinehart and Golitz. [15]

There is no literature on pediatric ICS, but the lesion begins in childhood or adolescence and tends to increase in size and number. [10] Though, there are reports of neonatal calcified scrotal lesions, [27] the authors argued that, involvement of the tunica vaginalis may be the sole presenting and clinical manifestation of gut perforation with suggestion that calcified scrotal masses may occur as the result of the passage of extraluminal meconium through the patent processus vaginalis with resultant meconium periorchitis leading to calcification.

In our two cases, the tunica vaginalis were examined and did not contain any calcium or material deposits [Figure 2]b and c.

From our intraoperative findings, the following sequence of pathogenesis is hereby postulated:-the dartos muscle is degenerated followed by aseptic necrosis with eventual lysis of the dartos muscle and absorption leading to calcium deposits in fibrous stroma. This agrees with what have been proposed by Pabuççuoglu et al.[16] and King et al. [19]

Though, our argument was based on two cases in pediatric age group, further research could lead to defining the factors that trigger such local tissue reaction with possible involvement of localized autoimmune connective tissue diseases leading to chronic deposition of calcium on, within or below the scrotum for a long period of time in keeping with the work of Gutierrez and Wetter. [28]


  Conclusion Top


Calcinosis of the scrotum in children is rare. Early detection and surgical treatment has satisfactory outcome.

 
  References Top

1.Yahya H, Rafindadi AH. Idiopathic scrotal calcinosis: A report of four cases and review of the literature. Int J Dermatol 2005;44:206-9.  Back to cited text no. 1
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2.Yusuf SM, Uloko AE, Sani MU, Mijinyawa SM, Mohammed AZ. Idiopathic scrotal calcinosis; a rare scrotal tumour: A case report and review of literature. Niger J Med 2010;19:482-4.  Back to cited text no. 2
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3.Vijay DD, Basarkod SI, Kotabagi HB, Unaiza F. Extensive idiopathic calcinosis: A case report. J Clin Diagn Res 2012;6:478-9.  Back to cited text no. 3
    
4.Harish SP, Rohan S, Shalmali A, Balakrishna S, Rajesh B, Jayaprakash SK. Scrotal calcinosis-Rare case reports of two cases. NUJHS (Nitte University Journal of Health Science) 2012;2:57-9.  Back to cited text no. 4
    
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8.Baruchin AM, Baruffaldi Preis FW, Cavallinim M, Ben-Dor D. Idiopathic calcinosis of the scrotum: Report of five cases and review of the literature. Eur J Plast Surg 1998;21:282-5.  Back to cited text no. 8
    
9.Sánchez-Merino JM, Bouso-Montero M, Fernández-Flores A, García-Alonso J. Idiopathic calcinosis cutis of the penis. J Am Acad Dermatol 2004;51:S118-9.  Back to cited text no. 9
    
10.Shapiro L, Platt N, Torres-Rodríguez VM. Idiopathic calcinosis of the scrotum. Arch Dermatol 1970;102:199-204.  Back to cited text no. 10
    
11.Karaca M, Taylan G, Akan M, Eker G, Gideroglu K, Gul AE. Idiopathic scrotal calcinosis: Surgical treatment and histopathologic evaluation of etiology. Urology 2010;76:1493-5.  Back to cited text no. 11
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12.Dubey S, Sharma R, Maheshwari V. Scrotal calcinosis: Idiopathic or dystrophic? Dermatol Online J 2010;16:5.  Back to cited text no. 12
    
13.Esra CK, Metin A, Nigihan C, Hatice B, Ender SD. Scrotal calcinosis: is it idiopathic or dystrophic? APJ (Aegean Pathology Journal) 2005;2:4-7.  Back to cited text no. 13
    
14.Khallouk A, Yazami OE, Mellas S, Tazi MF, El Fassi J, Farih MH. Idiopathic scrotal calcinosis: A non-elucidated pathogenesis and its surgical treatment. Rev Urol 2011;13:95-7.  Back to cited text no. 14
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15.Swinehart JM, Golitz LE. Scrotal calcinosis. Dystrophic calcification of epidermoid cysts. Arch Dermatol 1982;118:985-8.  Back to cited text no. 15
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16.Pabuççuoðlu U, Canda MS, Güray M, Kefï A, Canda E. The possible role of dartoic muscle degeneration in the pathogenesis of idiopathic scrotal calcinosis. Br J Dermatol 2003;148:827-9.  Back to cited text no. 16
    
17.Chiummariello S, Figus A, Menichini G, Bellezza G, Alfano C. Scrotal calcinosis: A very rare multiple clinical presentation. Clin Exp Dermatol 2009;34:e795-7.  Back to cited text no. 17
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18.Lewinski HM. Lymphangiome der Haut mot verkalkem. Inhalt Virch Arch Path 1883;91:371.   Back to cited text no. 18
    
19.King DT, Brosman S, Hirose FM, Gillespie LM. Idiopathic calcinosis of scrotum. Urology 1979;14:92-4.  Back to cited text no. 19
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20.Shah V, Shet T. Scrotal calcinosis results from calcification of cysts derived from hair follicles: A series of 20 cases evaluating the spectrum of changes resulting in scrotal calcinosis. Am J Dermatopathol 2007;29:172-5.  Back to cited text no. 20
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21.Kumagai K, Amemiya H, Muramatsu H, Kariba T, Matsuse K, Toyoshima A, et al. Idiopathic calcinosis of the scrotum: A case report. Hinyokika Kiyo 1987;33:1289-91.  Back to cited text no. 21
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22.Song DH, Lee KH, Kang WH. Idiopathic calcinosis of the scrotum: Histopathologic observations of fifty-one nodules. J Am Acad Dermatol 1988;19:1095-101.  Back to cited text no. 22
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23.Feinstein A, Kahana M, Schewach-Millet M, Levy A. Idiopathic calcinosis and vitiligo of the scrotum. J Am Acad Dermatol 1984;11:519-20.  Back to cited text no. 23
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24.Parlakgumus A, Canpolat ET, Caliskan K, Colakoglu T, Yildirim S, Ezer A, et al. Scrotal calcinosis due to resorption of cyst walls: A case report. J Med Case Rep 2008;2:375.  Back to cited text no. 24
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25.Noël B, Bron C, Künzle N, De Heller M, Panizzon RG. Multiple nodules of the scrotum: Histopathological findings and surgical procedure. A study of five cases. J Eur Acad Dermatol Venereol 2006;20:707-10.  Back to cited text no. 25
    
26.Saad AG, Zaatari GS. Scrotal calcinosis: Is it idiopathic? Urology 2001;57:365.  Back to cited text no. 26
    
27.Salman AB, Karaoðlanoðlu N, Suma S. Abdominal, scrotal, and thoracic calcifications owing to healed meconium peritonitis. J Pediatr Surg 1999;34:1415-6.  Back to cited text no. 27
    
28.Gutierrez A Jr, Wetter DA. Calcinosis cutis in autoimmune connective tissue diseases. Dermatol Ther 2012;25:195-206.  Back to cited text no. 28
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