
==== Front
Afr J Paediatr Surg
Afr J Paediatr Surg
AJPS
Afr J Paediatr Surg
African Journal of Paediatric Surgery: AJPS
0189-6725
0974-5998
Wolters Kluwer - Medknow India

39162754
AJPS-21-191
10.4103/ajps.ajps_163_22
Case Report
Isolated Vascular Malformation of the Clitoris: An Uncommon Cause of Clitoromegaly
Bakhteyar Asjad Karim
Parveen Zamurrad 1
Rahul Sandip Kumar
Department of Paediatric Surgery, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
1 Department of Obstetrics and Gynaecology, Darbhanga Medical College, Laheriasarai, Darbhanga, Bihar, India
Address for correspondence: Dr. Sandip Kumar Rahul, S/O Shri Kapil Kumar Jha, QR. No.- BN-2B, Near State Health Society, IGIMS Campus, Indira Gandhi Institute of Medical Sciences, Patna - 800 014, Bihar, India. E-mail: sandeep.rahul65@gmail.com
Jul-Sep 2024
10 4 2023
21 3 191193
06 12 2022
19 1 2023
27 1 2023
Copyright: © 2023 African Journal of Paediatric Surgery
2023
https://creativecommons.org/licenses/by-nc-sa/4.0/ This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
Clitoral enlargement (clitoromegaly) due to non-hormonal causes, particularly due to vascular malformation, is rare. Only eight such cases are reported in the literature. We report an additional case of isolated vascular malformation implicating the clitoris of a young girl child and its surgical management.

Clitoral index
clitoromegaly
hyperandrogenism
vascular malformation
==== Body
pmcINTRODUCTION

Clitoral enlargement (clitoromegaly) is defined as clitoral index (width × length in mm of glans clitoridis) more than 15 mm2–21 mm2 in newborn and adult women.[1] It is usually due to hyperandrogenism, i.e., congenital adrenal hyperplasia (CAH).[2] However, occasionally, it is due to preputial or clitoral masses (tumours), clitoral abscess and haematoma. We report a case of an isolated vascular malformation involving the clitoris in a young girl, discuss management and review the relevant literature. Only eight such cases are reported in English literature.

CASE REPORT

A 3.6-year-old healthy female was brought by parents for evaluation of a progressively enlarging mass involving the clitoris since the age of 3 months. She was erroneously investigated for CAH. However, endocrinology was normal, and karyotyping was 46 XX. Ultrasound with colour Doppler revealed a non-homogenous, hypoechoic, vascular lesion at the clitoris; however, other urogenital organs were appropriate for age. The patient was operated under general anaesthesia. Intraoperative examination revealed a dark-coloured, mixed consistency, 15 mm × 25 mm lesion (clitoral index = 375 mm2) around the clitoris. Urethral meatus was not involved [Figure 1a]. The lesion was meticulously separated from the clitoris, and it was preserved [Figure 1b]. The patient had an uneventful post-operative course, and she was discharged on the 3rd post-operative day. Histopathological examination of the lesion revealed features consistent with a benign vascular lesion [Figure 1c]. At 1-year follow-up, she was asymptomatic with near-normal cosmetic appearance.

Figure 1 (a) Pre-operative, (b) Post-operative, (c) Histopathological pictures

DISCUSSION

Clitoromegaly is usually due to raised androgen level [Table 1] as in cases of CAH. Non-hormonal causes are rare, and only a few cases are reported due to neurofibromatosis, epididymal cyst, abscess, haematoma, rhabdomyosarcoma[13] and metastasis from bladder carcinoma.[4] Clitoral vascular malformation and haemangiomas are infrequent; only eight such cases have been reported in the literature. Table 2 presents a summary of these cases.[12356789] Although the differences between vascular malformation and haemangiomas are well established now, they have been presented together because earlier this differentiation was not clear. However, it is now known that vascular malformations are pathologic lesions of vascular endothelium, and contrary to haemangiomas, they are present since birth, do not exhibit typical phases of growth and involution and can attain large sizes.[10] Unlike haemangiomas, they are unresponsive to propranolol. Of all the eight cases reported in Table 2, only two had vascular malformation. Therefore, this is only the third reported case of vascular malformation involving the clitoris and the ninth case overall when considering all the vascular lesions causing clitoromegaly.

Table 1 Causes of clitoromegaly

Congenital (CAH)	Acquired	
	
Hormonal	Non-hormonal	
Classical ambiguous genitalia	CAH	Neurofibromatosis	
In syndromic or isolated conditions	Late-onset adrenal or ovarian (androgen secreting) tumours	Epidermoid cyst (in case of trauma caused by genitalia mutilation or spontaneous)	
	Iatrogenic androgen exposure	Clitoral or preputial haemangioma/vascular malformation	
		Rhabdomyosarcoma	
		Metastatic infiltration	
		Clitoral abscess	
		Idiopathic	
CAH: Congenital adrenal hyperplasia

Table 2 Summary of reported cases of clitoromegaly secondary to vascular lesions

Year	Authors	Clinical features	Outcome/comments	
1978	Kaufman-Friedman et al.	Isolated haemangioma of the clitoris in an 18-year-old female	Underwent initial inguinal exploration as a case of Intersex; diagnosed during 2nd surgery when uncontrolled bleeding was seen locally and total enlarged mass was excised	
1991	Ishizu et al.	Large haemangioma involving only the clitoral prepuce in a 4-year-old female; rest of the genitalia and clitoris normal; had earlier had surgery for a large femoral tumour which was also arteriovenous haemangioma	Excision done; arteriovenous haemangioma confirmed on HPE	
2006	Haritharan et al.	Isolated vascular malformation of the clitoris in a 5-year-old female with enlarged clitoris and history of recurrent bleeding	Delineated on USG and MRI; lesion excised separately from the clitoris and reduction clitoroplasty performed; confirmed on HPE	
2009	Bruni et al.	Haemangioma of the clitoris presenting as massive clitoromegaly in a 20-year-old female	Diagnosed on HPE	
2012	Geramizadeh et al.	Clitoromegaly due to cavernous haemangioma in a 16-year-old female	Excised separate from the clitoris; confirmed on HPE	
2014	Nayyar et al.	Clitoral cavernous haemangioma in a 4-year-old female presenting as clitoromegaly	Excised separate from the clitoris; confirmed on HPE	
2018	Barral et al.	Cliteromegaly due to venous malformation in a 23-year-old female; also had venous malformations over the craniofacial region	Confirmed on imaging; sclerotherapy instituted	
2022	Thakur et al.	Clitoral haemangioma in a 4-month-old female. Similar lesions in the perineum, anus and vagina	Excision of clitoral haemangioma followed by oral propranolol for other lesions; confirmed by HPE	
2023	Current case	Clitoromegaly due to vascular malformation in a 3.6-year-old female	Excision done separate from the clitoris; confirmed on HPE	
HPE: Histopathological examination, USG: Ultrasonography, MRI: Magnetic resonance imaging

Since the hormonal causes of clitoromegaly outnumber other causes, they need to be ruled out first. Radiological assessment in the form of Doppler ultrasound is indispensable for non-hormonal causes as in our case. Once diagnosis is confirmed, both sclerotherapy[8] and surgery have been reported to have successful outcomes. In our case, complete excision of the vascular mass with preservation of the clitoris was possible.

Histopathology confirms the diagnosis and shows the tumour to be composed of large dilated blood-filled vessels lined by flattened endothelium.

CONCLUSION

Vascular malformation rarely affects the clitoris and may mimic cases of CAH in appearance. Systematic clinical, pathological and radiological examination helps in differentiating hormonal and non-hormonal causes of clitoromegaly and guides cause-specific management.

Consent

Informed consent was taken from the parents of the child for the presentation of her case and clinical pictures. Permission was also taken from the institutional ethics committee.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the parents have given their consent for images and other clinical information to be reported in the journal. The parents understand that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.
==== Refs
REFERENCES

1 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 19576811
2 Kaufman-Friedman K Hemangioma of clitoris, confused with adrenogenital syndrome: Case report Plast Reconstr Surg 1978 62 452 4 693675
3 Geramizadeh B Anbardar MH Shakeri S Clitoromegaly caused by cavernous hemangioma: A rare case report and review of the literature Urol Ann 2012 4 175 7 23248526
4 Hanna SJ Kaiser L Muneer A Nottingham JF Kunkler RB Squamous cell carcinoma of the bladder presenting as vulvitis and cliteromegaly Gynecol Oncol 2004 95 722 3 15581990
5 Ishizu K Nakamura K Baba Y Takihara H Sakatoku J Tanaka K Clitoral enlargement caused by prepucial hemangioma: A case report Hinyokika Kiyo 1991 37 1563 5 1767782
6 Haritharan T Islah M Zulfiqar A Thambi Dorai CR Solitary vascular malformation of the clitoris Med J Malaysia 2006 61 258 9 16898327
7 Nayyar S Liaqat N Sultan N Dar SH Cavernous haemangioma mimicking as clitoral hypertrophy Afr J Paediatr Surg 2014 11 65 6 24647298
8 Barral PA Petit P Bartoli JM Agostini A Vidal V Netter A A rare case of a venous malformation of the clitoris Eur J Obstet Gynecol Reprod Biol 2018 224 202 3 29566883
9 Thakur AN Clitoral hemangioma –Rarest cause of clitoromegaly J Indian Assoc Pediatr Surg 2022 27 751 2 36714490
10 McRae MY Adams S Pereira J Parsi K Wargon O Venous malformations: Clinical course and management of vascular birthmark clinic cases Australas J Dermatol 2013 54 22 30 23082725
