Key words
adolescence - benign tumours of the vulva - dysuria
Schlüsselwörter
Adoleszenz - benigne Vulvatumoren - Dysurie
Introduction
Female genital mutilation (FGM) or female genital circumcision (FGC) occurs mainly
in Africa, parts of the Arabian Peninsula and parts of Asia. The central, western
and north-eastern regions of Africa including countries such as Somalia, Sierra Leone,
Mali, Guinea and the Sudan are most affected [1]. The precise origins of FGM are unknown. Earliest historical evidence is found in
a religious text on an Egyptian sarcophagus (approx. 1991–1786 B. C.). The circumcision
of a temple maiden is mentioned on a papyrus from the year 163 B. C. First written
documentation of FGM, by the Greek geographer Strabo, dates back to the year 25 B. C.
[2]. Examinations of Egyptian mummies by an Australian pathologist have shown that infibulation
was not carried out. Further written documents originate from the Greek doctors Galen,
middle 2nd century A. D., and Aetios, middle 5th-6th century A. D. The surgical method
is described in detail in “Book 16” [3]. The work by Aetios is noteworthy for a number of reasons e.g. the exact function
of the clitoris was known, FGM was not performed by midwives, and it was performed
on girls before marriage. Aetiosʼs work confirmed statements by Strabo. In this context
statements made by the Greek doctor Soranus in the 2nd century A. D. “about the finding
of a big clitoris and its surgical treatment” are worthy of mention. The original
document no longer exists however later adaptions of Soranus by Caelius Aurelius,
a doctor from Sicca Veneria in Tunisia in the 5th century A. D., and the Arabic doctor
Al-Zahrawi, 11th century A. D. are noteworthy.
More than 120 surgical techniques are described in “Book 6” of the written records
of Paul of Aegina, a doctor in Alexandria in the 7th century A. D. Interestingly,
the anatomical findings in his patients may be consistent with DSD (developmental
sexual disorder).
From the works of Aetios, Galen and Soranus it is important to note that the indication
for FGM had medical aspects, e.g. size reduction of an enlarged clitoris. There is
only a single text passage from outside of Egypt, by the historian Xanthos from the
5th century B. C. that documents castration equivalent to FGM being performed in a
region of modern-day Western Anatolia. There is no written documentation of FGM as
a practice of the Islamic religion, or of its occurrence in the Kingdom of Saudi Arabia.
Finally, it is important to note that clitoridectomy was performed in 19th century
Europe and the United States of America as treatment for psychiatric illnesses such
as “hysteria” and to prevent masturbation [4].
FGM is performed in infants or between the 8th and 12th years of life [5]. There are an estimated 20 000 to 40 000 women currently living in Germany who have
suffered genital mutilation as infants or children [5]. This mutilation is often associated with acute complications and various late complications
[6]. One of most common late complications is painless progressively enlarging cysts
of the vulva [6], [7]. They result from entrapment of keratinised squamous epithelium in scarred areas
following FGM. Concrements can occasionally form within these cysts [7], [8]. The treatment of choice is complete surgical cyst excision. We present a case of
an 8-year-old girl with a traumatic epithelial inclusion cyst of the vulva.
History
An 8-year-old girl from Eritrea presented to our paediatric surgical emergency unit
with a progressively enlarging vulvar mass. She had first presented to a practice
paediatrician. The patient reported increasing pain on walking/running, progressive
obstructive urinary symptoms and intermittent retention. She had had the swelling
in her vulvar area for approx. 2 years and it had grown markedly in the preceding
months. No other illnesses were known.
Social history: The patient had been living in Germany for 8 months. Her father stated
that as a newborn in her homeland, Eritrea, a “ritual circumcision” had been performed
on her by a female relative in his absence.
Clinical Examination
In the area of the clitoris an approx. 4 × 4 cm tense, fluctuant mass was noted ([Fig. 1]). Genital findings were otherwise consistent with Type II FGM (WHO classification):
clitoridectomy with partial removal of the labia minora. The vaginal orifice had not
been closed.
Fig. 1 Squamous epithelial inclusion cyst of the vulva, preoperative picture.
Operative Management
The patient was admitted for surgical resection of the mass and reconstructive surgery
of the labia. Inspection of the genitalia was performed in general anaesthesia. The
vaginal orifice and external urethral orifice were normal. In the area of the vulva
the 4 × 4 cm tense, fluctuant mass was found to be pedunculated on a scar tissue stalk.
Vaginoscopy was normal. The tumour, which was filled with a yellowish, gelatinous
material, was carefully enucleated after midline diathermic skin incision ([Fig. 2]). Using bipolar forceps it was completely removed on its stalk (peduncle) without
perforation ([Fig. 3]). The labia minora were then reconstructed using the two remaining skin flaps and
a urinary catheter inserted. Wound management comprised Jelonet gauze and compression.
In addition we treated with intravenous cefuroxime and metronidazole.
Fig. 2 The beginning of marsupialisation, intraoperative picture.
Fig. 3 Squamous epithelial cyst with scar tissue stalk (peduncle) after complete marsupialisation,
intraoperative picture.
Histology showed a squamous epithelial cyst of the vulva that was described as “evidently
traumatic in nature” by the pathologist ([Fig. 4]).
Fig. 4 Histological preparation of the squamous epithelial cyst.
The postoperative period was uneventful and the urinary catheter was removed after
a week. Urinary symptoms improved rapidly. The patient, her parents and the surgical
team rated the plastic surgical result as very good. To avoid aggravating the patientʼs
feelings of shame photographic documentation was not performed. Child welfare services
and a local gynaecologist were involved to assist with future psychosocial and medical
care. The responsible police services were notified. In this case there were no legal
ramifications for the family, since the FGM occurred 8 years previously in Eritrea
and there was no risk of FGM to other children in the family in Germany. In Eritrea
FGM has been a criminal offence since 31. 07. 2007 [1], [5].
In Germany various legal aspects are elucidated e.g. the abuse of power over vulnerable
individuals, abuse of parental responsibility/custodianship or grievous bodily harm
are distinguished from threatened FGM in the patientʼs homeland where e.g. deportation
should be prevented [1], [5].
Discussion
According to WHO estimates, approx. 100 to 140 million women are affected by female
genital mutilation worldwide. The WHO classifies FGM into 4 types [1], [5], [9].
Numerous complications are caused by this mutilating practice. They can be classified
as acute or chronic.
Acute complications include local infections that can progress to sepsis. HIV infection
has also been described. Urinary symptoms such as dysuria and acute urinary retention,
particularly following infibulation, are listed by Teufel et al. [10] and other authors. Injury to nearby organs e.g. urethra, bladder and anal sphincter
have been described. Fractures of the femur and clavicle have been reported due to
girls being forcefully immobilized. One of the most common acute complications is
bleeding, often with dramatic consequences [5], [11].
Chronic complications comprise protracted wound healing and ascending infection such
as urethritis and vaginitis. Vaginal stenosis and infertility have been described.
Dysmenorrhoea and menorrhagia occur after menarche.
The complication in our case report is classified with delayed complications that
also include keloid formation or chronic abscess in the area of scar tissue, neurinoma
(amputation neurinoma) and dermoid cyst. Further complications can occur in pregnancy
and labour e.g. increased bleeding, local wound dehiscence, protracted labour, perineal
infections and fistulae (vesico- and rectovaginal) [5], [10].
In their meta-analysis Rigmor et al. showed that women who had undergone FGM were
at increased risk of dyspareunia, reduced libido and reduced sexual satisfaction compared
to controls [12]. Gudu et al. present a case comparable to ours in BMC Womenʼs Health Journal [7]: A 21-year-old woman who had undergone infibulation at the age of 8 years presented
to them with a painful, infected swelling in the vulvar area. Complete surgical excision
of a cyst and simultaneous defibulation were performed. There were no subsequent complications.
Squamous epithelial inclusion cysts and dermoid cysts in the vulvar area are extremely
rare and occur as complications of FGM, episiotomy or other local trauma to the vulva
[7], [8], [13]. The usually painless and progressively enlarging cysts arise through invagination
and entrapment of squamous epithelium, other cell remnants and secretions in scar
tissue beneath the skin surface. Incidence and latency following FGM is variable.
Most cysts are asymptomatic but may enlarge. Patients often consult a doctor only
when local pain or cyst rupture during sexual intercourse occurs, or at vaginal delivery.
Cyst complications include infection, rupture, haematoma or carcinoma. Treatment of
choice is complete removal of the cyst and, if necessary and possible, simultaneous
plastic surgical reconstruction of the genitalia [7].
Different reconstructive options apply to the different forms of FGM ([Table 1]). Here P. Foldès must be mentioned, whose retrospective study presents the results
of genital reconstruction in type II and type III FGM in 2938 patients. His preferred
surgical method is as follows: The remaining clitoris is exposed by resecting the
covering skin. The suspensory ligament of the clitoris is then divided to achieve
better mobilization of the stump. The clitoris is then fixed in its correct position
in the skin [14]. Many of the above mentioned complications can be partially or completely rectified
by defibulation [10].
Table 1 WHO classification of FGM.
|
Type 1
|
Sunna
|
Excision of the clitoral hood with partial or complete removal of the clitoris
|
|
Type 2
|
Excision, clitoridectomy
|
The clitoris is completely removed, the labia minora partially or totally removed,
the vaginal orifice is not closed.
|
|
Type 3
|
Infibulation
|
Complete removal of the clitoris and labia minora, partial removal of the labia majora
by detachment of their inner edges, the remaining skin being stitched together so
that a bridge of scar tissue forms over the vaginal orifice leaving only a small opening
for menstrual blood and urine.
|
|
Type 4
|
Practices not further classified
|
Pricking or piercing of the clitoris, tearing, incising etc.
|
Through increasing migration more girls and female youths with FGM are likely to present
to practices and hospitals in Germany in years to come. According to estimates by
Terre de Femmes approx. 18 000 girls and women are affected and 5000–6000 are at risk
[15]. Clinical experience is generally minimal as demonstrated by Hänselmann et al. [9]. In their study from 2011 only 14 of 223 participating medical practices had treated
women with FGM. There were no exact data on affected girls and female youth.
Conclusion
Vulvar epithelial inclusion cysts and dermoid cysts following FGM are extremely rare.
Complications often require surgical intervention. Through increasing migration, more
girls and female youths with FGM are likely to present to practices and hospitals
in Germany. Thus increased knowledge and awareness of the medical complications of
FGM and their treatment is necessary.
