Clitoral epidermal inclusion cyst leading to anorgasmia: A case report and literature review

Clitoral epidermal inclusion cyst leading to anorgasmia: A case report and literature review

Journal Pre-proof Clitoral epidermal inclusion cyst leading to anorgasmia: A case report and literature review A.M. DiCarlo-Meacham, MD, K.L. Dengler,...

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Journal Pre-proof Clitoral epidermal inclusion cyst leading to anorgasmia: A case report and literature review A.M. DiCarlo-Meacham, MD, K.L. Dengler, MD, A.N. Snitchler, DO, D.D. Gruber, MS, MD PII:

S1083-3188(20)30151-0

DOI:

https://doi.org/10.1016/j.jpag.2020.01.150

Reference:

PEDADO 2446

To appear in:

Journal of Pediatric and Adolescent Gynecology

Received Date: 4 December 2019 Revised Date:

18 January 2020

Accepted Date: 24 January 2020

Please cite this article as: DiCarlo-Meacham AM, Dengler KL, Snitchler AN, Gruber DD, Clitoral epidermal inclusion cyst leading to anorgasmia: A case report and literature review, Journal of Pediatric and Adolescent Gynecology (2020), doi: https://doi.org/10.1016/j.jpag.2020.01.150. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2020 Published by Elsevier Inc. on behalf of North American Society for Pediatric and Adolescent Gynecology.

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Title: Clitoral epidermal inclusion cyst leading to anorgasmia: A case report and literature review

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A.M. DiCarlo-Meacham, MD, Department of Obstetrics and Gynecology, Walter Reed National

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Military Medical Center, Bethesda, MD

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K.L. Dengler, MD, Department of Obstetrics and Gynecology, Walter Reed National Military

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Medical Center, Bethesda, MD

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A.N. Snitchler, DO, Department of Pathology, Walter Reed National Military Medical Center,

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Bethesda, MD

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D.D. Gruber, MS, MD, Department of Obstetrics and Gynecology, Walter Reed National Military

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Medical Center, Bethesda, MD

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Declaration of interests: None

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Eposter presentation at the 2019 American Urogynecologic Society/International

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Urogynecologic Association Joint Scientific Meeting in Nashville, TN Sept. 24-28, 2019.

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Corresponding author: Angela DiCarlo-Meacham, MD

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Walter Reed National Military Medical Center, Department of Obstetrics and Gynecology

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Address: 8901 Wisconsin Ave, Bethesda, MD 20889

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Phone: 636-288-2955; Email: [email protected]

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Word Counts: Abstract 120, Manuscript 923

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Disclaimer: The views expressed in this article are those of the authors and do not reflect the

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official polices of the Departments of the Army, Air Force or Navy, the Department of Defense or

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the U.S. Government. 1

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Abstract

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Background: Clitoral epidermal inclusions cysts are most frequently seen following trauma,

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especially female genital mutilation. Spontaneous clitoral epidermal inclusion cysts are rare with

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an unclear etiology and their impact on later sexual function has not been described.

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Case: A 15 year-old spontaneously developed a clitoral mass that progressively enlarged over

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seven years, ultimately leading to secondary anorgasmia. Surgical removal resulted in

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restoration of normal anatomy and complete return of clitoral function. Final pathology revealed

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the mass to be an epidermal inclusion cyst.

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Summary and Conclusion: Clitoral epidermal inclusion cysts typically present in childhood or

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early adolescence and can lead to sexual dysfunction if left untreated. Physicians must consider

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the potential sequelae of these cysts when counseling and managing these patients.

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Key Words

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Anorgasmia; Clitoral cyst; epidermal inclusion cyst; sexual dysfunction

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Introduction

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Epidermal inclusion cysts are commonly found on the face, neck and torso, but are less

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commonly reported on the vulva. They are characterized by a cyst wall of stratified squamous

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epithelium containing keratinous debris. Clitoral epidermal inclusion cysts are rare and can be

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spontaneous or a result of implantation of follicular epithelium into the dermis following trauma,

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particularly female genital mutilation.1 Spontaneous cysts can be present at birth or develop

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later in life and can present a diagnostic challenge for clinicians.

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The differential diagnosis of a clitoral cyst is broad and includes congenital, hormonal,

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neoplastic, infectious or structural etiologies which can lead to a costly and timely workup for the

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patient. Diagnosis of an epidermal inclusion cyst is typically made clinically based on the

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appearance of a discrete cyst that is freely mobile and radiologic imaging can help confirm the

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diagnosis.2 On pelvic ultrasound they appear as simple hypoechoic cystic structures and on

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magnetic resonance imaging (MRI) they have high signal intensity on diffusion weighted

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images. Surgical management poses its own challenges as surgeons are tasked with removing

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the cyst while preserving the neurovascular structures vital for normal clitoral function.

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Because spontaneous clitoral epidermal inclusion cysts are rare, no previous descriptions of

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their presentation or growth pattern has been published and their etiology remains unclear.

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Additionally, the impact they can have on sexual function has not been described.2-16 We

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present a case of a large clitoral epidermal inclusion cyst that led to secondary anorgasmia and

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review the growth, development, and sequelae of other reported clitoral epidermal inclusion

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cysts.

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Case

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A 15 year-old developed a spontaneous vulvar mass that progressively grew over seven years.

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She had presented to a gynecologist during that time frame due to bothersome skin splitting and

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irritation from and the cyst rubbing on her clothing. The cyst was drained but later recurred. At

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age 22 she presented to the Urogynecology clinic due to loss of clitoral sensation and the

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development of anorgasmia over the previous year. She had no history of trauma or surgery to

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her vulva and no medical problems. On physical exam, she had hypoestrogenic appearing

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vulvar epithelium and a four centimeter mass located superior to the urethra with inability to

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retract the clitoral hood to visualize the clitoris (Figure 1).

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Pelvic MRI was done to further characterize the mass and showed a 3.9x4.0x1.8cm area of high

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signal intensity on T2 images with minimal peripheral enhancement overlying the clitoris. There

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was no communication with the peritoneal cavity or urethral involvement. She was taken to the

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operating room for surgical excision.

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A transverse incision was made at the inferior aspect of the cyst. This minimized risk of injury to

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the neurovascular structures of the clitoris and maximized epithelium for reconstruction of the

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labia minora and clitoral hood. A clear cyst wall was identified and easily separated from the

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surrounding structures with blunt and sharp dissection. As the base of the cyst was reached, the

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clitoral glans became visible and a clear separation between the cyst and the clitoris was seen.

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The clitoris and its neurovascular structures remained intact. The cyst was found to be an

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epidermal inclusion cyst on pathologic evaluation (Figure 2).

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After removal of the cyst, distended vulvar epithelium was available for reconstruction of the

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labia minora and clitoral hood. The tissue was reapproximated in the midline in a cephalad-

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caudad manner and the labia minora and clitoral hood were reconstructed with a series of

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interrupted delayed absorbable stitches placed bilaterally. A small amount of tissue was

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trimmed to ensure symmetry but excess tissue was intentionally left to allow for tissue

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retraction. Vaginal premarin cream was given post-operatively to assist in wound healing.

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Immediately following surgery, the patient had partial return of sensation to the clitoris. At her six

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week post-operative visit she had an excellent cosmetic result but still had decreased sensation

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to the clitoris from her baseline. By seven months postoperatively she had regained sensation to

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her clitoris and could again achieve an orgasm (Figure 3).

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Summary and Conclusion

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With the addition of our case, sixteen cases of spontaneous clitoral epidermal inclusion cysts

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exist in the literature.2-16 Of these, all required surgical removal aside from one which regressed

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in infancy.3 Nearly all cases were diagnosed prior to adulthood with seven cases (43.8%) being

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diagnosed in early childhood3-5,10,12-13,16 and seven (43.8%) being first noted in early

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adolescence, likely around the time of puberty.2, 7-9, 14-15 An additional case first presented during

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pregnancy, regressed following delivery and then grew again with a subsequent pregnancy,

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ultimately requiring excision.6 These patterns of growth suggest that estrogen may play a key

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role in their development or growth.

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Van der Putte et al demonstrated variation in the embryonic development of the clitoral prepuce.

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In one variant of prepuce development, they found cornified cavities within the lamellae

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separating the prepuce from the glans.17 Spontaneous dilation of these cornified cavities may

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lead to cyst formation and could explain cysts that present in infancy or early childhood.

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Estrogen stimulation of the epithelium within those cavities leading to increased desquamation

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of skin cells could explain why a significant percentage of patients noted cyst formation during

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times of increased estrogen levels. Figure 2 illustrates the desquamation of skin cells found

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within the cyst in our patient. A similar pattern of growth has been seen in patients with clitoral

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epidermal inclusion cysts following female genital mutilation with the theory that implanted 5

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epidermis from the procedure proliferates with estrogen stimulation leading to cyst

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development.18

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Two important sequelae highlighted in this review are psychological distress and sexual

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dysfunction. Of the ten patients that were peri-pubertal or older in the cases reported, 70%

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experienced psychological distress from the anomaly, 50% experienced pain, and 90%

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experienced distress and/or pain.2-16 Furthermore, in the case described above, one patient

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experienced anorgasmia, which has not been previously described as a sequela of a clitoral

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epidermal inclusion cyst. Surgical removal was necessary in all patients with symptomatic cysts.

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In the five women who the authors stated were sexually active at the time of removal, all

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reportedly maintained or regained sexual function. Patients diagnosed and treated early in

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childhood or in early adolescence may have been spared the psychological distress that

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patients who presented at older ages experienced. The potential impact of these cysts on

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current or future female self-image and sexual function is compelling and referral for surgical

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removal should be considered once the diagnosis is made by clinical exam and/or diagnostic

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imaging.

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This case demonstrates the potential significant impact untreated clitoral epidermal inclusion

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cysts can have on sexual function. Because these cysts most often require surgery to resolve,

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the potential impact of clitoral cysts on sexual function should be considered even when

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evaluating patients who are not yet sexually active. Because most spontaneous clitoral

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epidermal inclusion cysts seem to present during the pediatric or adolescent time frames based

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on this literature review, pediatricians and pediatric and adolescent gynecologists should be

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aware of the potential sequelae of these cysts so that patients and parents can be counseled

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appropriately and make the most informed decisions regarding cyst management.

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Disclosures

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All authors have no disclosures or conflicts of interest to declare.

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References 1. Rouzi AA: Epidermal clitoral inclusion cysts: not a rare complication of female genital mutilation. Hum Reprod 2010; 7:1672-4 2. Johnson LT, Lara-Torre E, Murchison A, et al: Large epidermal cyst of the clitoris: a

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novel diagnostic approach to assist in surgical removal. J Pediatr Adolesc Gynecol 2013;

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3. Beltrao LA, Correia EPE, da Rosa EB, et al: Clitoris cyst mimicking ambiguous genitalia and presenting spontaneous regression. Pediatr Int 2019; 1:113-4 4. Schober MS, Hendrickson BW, Alpert SA: Spontaneous clitoral hood epidermal inclusion cyst mimicking clitoromegaly in a pediatric patient. Urology 2014; 1:206-8 5. Saha M: Epidermoid cyst of the clitoris: a rare cause of clitoromegaly. APSP J Case Rep 2013; 3:51 6. Hughes JW, Guess MK, Hittelman A, et al: Clitoral epidermoid cyst presenting as pseudoclitoromegaly of pregnancy. AJP Rep 2013; 1:57-62 7. Al-Ojaimi EH, Abdulla MM: Giant epidermoid inclusion cyst of the clitoris mimicking clitoromegaly. J Low Genit Tract Dis 2013; 1:58-60 8. Beurdeley M, Cellier C, Lemoine F, et al: Imaging of a primitive clitoral epidermoid cyst. Pediatr Radiol 2012; 6:764-7. 9. Lambert B: Epidermoid cyst of the clitoris: a case report. J Low Genit Tract Dis 2011; 2:161-2 10. Aggarwal SK, Manchanda V, Pant N: Epidermoid cyst of clitoris mimicking clitoromegaly. J Indian Assoc Pediatr Surg 2010; 1:23-4

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11. Paulus YM, Wong AE, Chen B, et al: Preputial epidermoid cyst: an atypical case of acquired pseudoclitoromegaly. J Low Genit Tract Dis 2010; 4:382-6 12. Anderson-Mueller BE, Laudenschlager MD, Hansen KA: Epidermoid cyst of the clitoris:

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an unusual cause of clitoromegaly in a patient without history of previous female

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circumcision. J Pediatr Adolesc Gynecol 2009; 50: e130-2

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13. Cetinkursun S, Narci A, Sahin O, et al: Epidermoid cyst causing clitoromegaly in a child. Int J Gynaecol Obstet 2009; 1:64 14. Linck D, Hayes MF: Clitoral cyst as a cause of ambiguous genitalia. Obstet Gynecol 2002; 5 pt 2:963-6 15. Schmidt A, Lang U, Kiess W: Epidermal cyst of the clitoris: a rare cause of clitorimegaly. Eur J Obstet Gynecol Reprod Biol 1999; 2:163-5

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16. Teague JL, Anglo L. Clitoral cyst: an unusual cause of clitorimegaly. J Urol 1996; 6:1057

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17. Van der Putte SC, Sie-Go DM: Development and structure of the glandopreputial sulcus

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of the human clitoris with a special reference to glandopreputial glands. Anat Rec

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(Hoboken) 2011; 1:156-64

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18. Asante A, Omurtag K, Roberts C: Epidermal inclusion cyst of the clitoris 30 years after female genital mutilation. Fertil Steril 2010; 3:1097

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Figure Legends

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Figure 1. Large cyst of the clitoral hood obscuring the clitoris.

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Figure 2. Pathologic (H&E stain at 40x magnification) image of the cyst composed of a thin layer

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of stratified squamous epithelium with keratin debris flaking off into the cyst lumen (arrow),

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consistent with epidermal cyst.

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Figure 3. Post-operative exam at 11 months. The patient had regained complete return of

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clitoral function by that point.

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