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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2022  |  Volume : 27  |  Issue : 6  |  Page : 751-752
 

Clitoral hemangioma – Rarest cause of clitoromegaly


Department of Paediatric Surgery, Patna Medical College and Hospital, Patna, Bihar, India

Date of Submission13-Jan-2022
Date of Decision14-Mar-2022
Date of Acceptance30-Apr-2022
Date of Web Publication11-Nov-2022

Correspondence Address:
Ashoka Nand Thakur
Department of Paediatric Surgery, Patna Medical College and Hospital, Patna, Bihar
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_5_22

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   Abstract 


Clitoromegaly in female child is a rare condition. This is often associated with androgen excess in disorders of sexual differentiation usually congenital adrenal hyperplasia. Nonhormonal causes of clitoromegaly are rare and are commonly due to neurofibromatosis, epidermoid cyst, rhabdomyosarcoma, and very rarely hemangioma. Hemangioma is one of the common benign neoplasms of infancy, usually located in head, trunk, and extremities. We are reporting 4-month-old child with clitoromegaly with normal hormonal assay. Clitoromegaly was due to hemangioma. Histopathology of mass confirmed the diagnosis. Only five cases have been reported in literature till date.


Keywords: Clitoris, congenital adrenal hyperplasia, hemangioma


How to cite this article:
Thakur AN. Clitoral hemangioma – Rarest cause of clitoromegaly. J Indian Assoc Pediatr Surg 2022;27:751-2

How to cite this URL:
Thakur AN. Clitoral hemangioma – Rarest cause of clitoromegaly. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2022 Dec 7];27:751-2. Available from: https://www.jiaps.com/text.asp?2022/27/6/751/360961





   Introduction Top


Hemangiomas are most common tumors of infancy and childhood. They are not usually present at birth but appear in the neonatal period, in the first 2 weeks of life.[1] They are usually present in head, neck, trunk, and extremities. Rarely it involves clitoris and leads to clitoromegaly. This is very important to differentiate clitoral hemangioma from enlargement of clitoris secondary to androgen excess as in disorders of sexual differentiations. We are reporting 4-month child with clitoral enlargement as well as hemangiomatous lesion in vagina, perineum, anus, and lower extremities. Surgical excision of mass along with propranolol therapy was given. Histopathology of mass confirmed the hemangioma.


   Case Report Top


Four-month-old female child presented to us with clitoromegaly. On clinical examination, urethra and vaginal orifice were normal. Three small reddish blue lesions of hemangioma were present in lower limb. Similar lesions were present in perineum, anus, and vagina. The size of clitoris was large with bluish appearance. Clitoral mass was firm on palpation. No any other anomalies were found on examination. Hemogram along with 17 hydroxyprogesterone level was done and was within normal limit. Ultrasonography of abdomen and perineum was done. Abdominal ultrasound was normal but perineal ultrasound showed hemangioma of clitoris. Echocardiography was also normal. Parents were very anxious due to abnormal presentation of baby. Surgical planning was done. Under general anesthesia with endotracheal intubation, the baby was positioned in lithotomy position [Figure 1], [Figure 2], [Figure 3]. Under strict aseptic precaution and after catheterization, skin degloving was done up to the root level. Then, after transfixation, whole mass was excised. Redundant skin was also excised up to some extent. Byerization of skin flap was done to cover the stump. Catheter was removed after 48 h. Oral propranolol was started after discharged from hospital for the other lesions.
Figure 1: Clitormegaly

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Figure 2: Hemangioma of perineum

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Figure 3: Histopathology of mass

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   Discussion Top


Clitoromegaly in infancy is rare entity. It is defined as clitoral index (width × length in mm) more than 15 mm2 in the newborn and more than 21 mm2 in an adult women.[2] It is usually associated with androgen excess in disorders of sexual differentiation. Nonhormonal causes of clitoromegaly are rare and are commonly due to neurofibromatosis, epidermoid cyst, rhabdomyosarcoma, and very rarely hemangioma. Hemangiomas are most common tumors of infancy and childhood. They are not usually present at birth but appear in the neonatal period, in the first 2 weeks of life.[1] They are usually present in head, neck, trunk, and extremities. Rarely it involves clitoris and leads to clitoromegaly.[3] So far, only five cases have been reported. This is very important to differentiate between clitoromegaly due to androgen excess from hemangioma. Ultrasonography and magnetic resonance imaging (MRI) are important tool to diagnose the hemangiomatous lesion. MRI was not done in our case. Ultrasound showed hemangioma of clitoris. Hormonal assay like 17 hydroxyprogesterone level should be done to rule out androgen excess due to congenital adrenal hyperplasia. Hemangioma involving other body part is usually treated with medical management such as oral propranolol, timolol maleate 0.5% for local application, intralesional steroids,[4] and oral steroids also. However, clitoral enlargement requires surgical excision of mass.


   Conclusion Top


Hemangioma is one of the rare causes of clitoromegaly. Proper diagnosis and surgical intervention are the key of management of this rare disease.

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 initial s 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.
Grosfeld JL, James A. O'Neill Jr, Eric W. Fonkalsrud MD, Arnold G. Coran MD, Editors. Pediatric Surgery. 6th ed. Mosby, Elsevier, Philadelphia, PA 2006; p. 2098.  Back to cited text no. 1
    
2.
Bruni V, Pontello V, Dei M, Alessandrini M, Li Marzi V, Nicita G. Hemangioma of the clitoris presenting as clitoromegaly: A case report. J Pediatr Adolesc Gynecol 2009;22:e137-8.  Back to cited text no. 2
    
3.
Kaufman-Friedman K. Hemangioma of clitoris, confused with adrenogenital syndrome: Case report. Plast Reconstr Surg 1978;62:452-4.  Back to cited text no. 3
    
4.
Sloan GM, Reinisch JF, Nichter LS, Saber WL, Lew K, Morwood DT. Intralesional corticosteroid therapy for infantile hemangiomas. Plast Reconstr Surg 1989;83:459-67.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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    Abstract
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