Genital Reconstruction in a Marital Dispute Due to Adult-Acquired Clitoromegaly: Righting a Wrong: A Case Report

INTRODUCTION

Clitoromegaly is a rare condition characterized by abnormal enlargement of the clitoris, sometimes resembling a penile structure.^1-3 A clitoral index (product of glans length and width) greater than 35 mm² is considered abnormal. Clitoromegaly may be congenital or acquired. While congenital causes are well described, acquired clitoromegaly is infrequently reported.⁴

Hormonal causes, such as congenital adrenal hyperplasia (CAH), are more commonly implicated; however, non-hormonal and idiopathic causes are rare.⁵ Non-hormonal etiologies include neurocutaneous syndromes (neurofibromatosis, tuberous sclerosis), epidermoid cysts, hemangiomas, nevi, and superficial lipomas.⁶

Genitourinary involvement in neurofibromatosis type 1 (NF1), particularly involving the clitoris, is exceedingly rare, but, when present, clitoromegaly may be the presenting manifestation.⁷

Psychological distress is a major concern in adult females with clitoromegaly, especially those who are sexually active or newly married, due to anxiety related to sexual function, body image, marriage, and fertility.⁸ Literature addressing both surgical management and psychosocial consequences remains limited.

We present a case of adult-acquired clitoromegaly in a newly married woman with NF1, where mistaken gender identity led to marital dispute. Surgical genital reconstruction with reduction clitoroplasty and labiaplasty successfully restored anatomy, sensation, and psychosocial well-being.

CASE REPORT

A 35-year-old, recently married woman presented with progressive enlargement of the clitoris extending into the right labia majora, producing a penile-like appearance [Figure 1]. Her husband, mistaking this for a penis, believed she was a transgender male and accused her of deception, resulting in marital separation and severe psychological distress.

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Figure 1: Pre operative image showing Pseudopenis, multiple nodules on thigh abdomen with café au lait spots

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The patient reported normal female external genitalia at birth and normal pubertal development with appropriate secondary sexual characteristics. She denied any history of gender dysphoria. At 13 years of age, she developed multiple cutaneous nodules and was diagnosed with neurofibromatosis. Several years later, she noticed a gradual enlargement of the clitoris, which progressively extended into the right labia majora.

Psychosocial evaluation revealed that she had concealed this condition due to embarrassment and social stigma, particularly as it involved the genital region. The condition caused persistent emotional distress, culminating in significant sadness during her wedding period. The genital abnormality was discovered by her husband only after marriage, precipitating marital conflict.

There was no history of drug intake, androgen exposure, family history of clitoromegaly, or excessive masturbation. She had a normal distribution of pubic hair, normal body habitus, and no features suggestive of polycystic ovarian syndrome. Gynecological and systemic examinations were normal.

On examination, isolated clitoromegaly was noted, with a phallic structure measuring approximately 9 cm in length. Secondary sexual characteristics were normal. There was no clinical or biochemical evidence of endocrine abnormalities or disorders of sexual development. Abdominal and pelvic ultrasonography revealed no abnormalities. Due to financial constraints, karyotyping was not performed. Routine laboratory investigations were within normal limits.

Surgical technique

Patient underwent genital reconstruction with reduction clitoroplasty with preservation of the neurovascular pedicles along with labiaplasty under spinal anesthesia. A traction suture of 3/0 nylon was placed in the glans of the clitoris Figure 2. An incision was made beginning from clitoral hood down to right lateral phallus over the right labia parallel to the axis of the clitoral shaft, and carried through a semicircular arc to the base of the glans as described by [Figure 3] Riccardi.⁹

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Figure 2: Intraoperative Image showing clitoromegaly extending into right labia.

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Figure 3: Image showing debulked reduced clitoris raised on neurovascular bundle and separated from corporas.

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Two longitudinal incisions were made lateral to the dorsal neurovascular bundle. Two crura were identified until the division, clamped, and the body of the clitoris was resected. The base of the glans was sutured to the divided corpora with 3/0 Vicryl, and the proximal and distal ends of the corpora were closed with 2/0 Vicryl. The skin was closed with 4/0 Vicryl sutures as well. Similarly, excision of one of the thigh nodules was done and sent for histopathology.

Outcome and follow-up

The patient was followed for three months postoperatively. There were no early or late complications. Clitoral sensation was completely preserved, and the patient expressed high satisfaction with both aesthetic and functional outcomes [Figure 4]. Following counseling and education of her husband and family regarding the medical nature of the condition, marital reconciliation was achieved, and the patient’s psychological well-being significantly improved.

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Figure 4: Image of 2 months post operative.

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DISCUSSION

NF1 is an autosomal dominant disorder with an incidence of approximately 1 in 3,000 live births. External genital involvement, particularly clitoral neurofibroma, is extremely rare.⁶ Solitary and plexiform neurofibromas occur in approximately 26.7% of patients with NF1.¹⁰

Female genital involvement is reported more frequently than male involvement, yet fewer than 30 cases of female external genital neurofibromas have been documented in the literature.^11,12 Clitoral neurofibroma was first described by Haddad and Jones.¹³

Surgical methods for the correction of clitoral hypertrophy were first described in 1934 by Young,¹⁴ who performed an operation for clitoral reduction preserving the glans in a child with congenital adrenal hyperplasia, which later on sloughed off. Several reports,^15-18 described simultaneous preservation of dorsal neurovascular bundles and ventral urogenital cutaneous pedicles for clitoroplasties done during infancy. This case was unusual because only dorsal neurovascular bundles were preserved in an adult female using a small semi-circular incision on the dorsum of the clitoris, and the incision was extended into the right labia, reducing the clitoris and elevating the glans clitoris on the dorsal neurovascular bundle, maintaining the sensitivity of the glans.

The Techniques for clitoral resection have changed a great deal over the past years as knowledge of the anatomy has improved, and the goals of clitoroplasty are to achieve an appearance indistinguishable from normal genital structures and to preserve tactile sensation with a satisfactory sexual response, including an appropriately sized clitoris when engorged. These goals are accomplished by shortening the corpora cavernosa while preserving the neurovascular supply to the glans clitoris.¹⁹ There are reports of preservation of the dorsal and ventral neurovascular pedicles described in infants¹⁹. We report the Genital reconstruction of clitoral hypertrophy with clitoroplasty with preservation of dorsal neurovascular pedicles and labiaplasty in an adult woman who landed in marital dispute with mistaken identity of transgender by her husband.

In the past, the entire clitoris was removed, which is not an acceptable option, as the clitoris is considered an important sensory sexual organ.²⁰ Later, as much as an enlarged clitoris was preserved, and the corpora was buried under the skin, but it was very painful when aroused.²¹ Further refinements of the technique involved glans clitoris preservation along with the neurovascular bundle and excision of corporal tissue done to preserve the sensation.²² On the basis of the findings of the patient, she had the right side of the clitoris enlarged, and it extended into the right side of the labia as a shaft, giving it the appearance of a penis. The surgery was customized and individualized as per the requirements and refinements to obtain a better cosmetic result and to preserve clitoral sensitivity.

There have been many variations in surgical techniques. Papageorgiou et al.²³ preserved both the dorsal and ventral neurovascular bundles without touching the glans. Dorsal neurovascular bundle preservation, along with dorsal central wedge glans reduction, was done by Kogan et al.²⁴ In our case, the clitoris was enlarged with more growth towards the right and extending into the labia with close proximity to the urethral opening and the inner wall of the labia. A semicircular incision on the glans was made, and the ventral glans reduction was done; the incision was extended into the labia and was dissected off the urethral opening. Selvaggi et al.²² removed the glans clitoris bilaterally, but in our case, the glans on the ventral side was reduced, where the density of nerves is lowest,¹⁹ along with the neurovascular bundle. By modifying the various procedures used previously, this operation satisfied both the functional and aesthetic desires of the patient, the patient’s husband, and the patient's family. She was followed for 3 months after surgery. After counselling and explaining this condition to the husband and their family, the patient was accepted, and the marital dispute was resolved. Her confidence had increased.

CONCLUSION

Clitoral neurofibroma causing adult-acquired clitoromegaly is a rare manifestation of NF1. This case highlights the successful role of individualized genital reconstruction with reduction clitoroplasty and dorsal neurovascular preservation in restoring anatomy, sensation, and psychosocial well-being. Importantly, appropriate surgery, counseling, and education helped resolve a severe marital dispute arising from mistaken gender identity. This report emphasizes the need for awareness of rare non-hormonal causes of clitoromegaly and the role of gender-affirming, patient-centered surgical care.