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Laparoscopic gonadectomy in paediatric and adolescentgirls with intersex disorders

Gloria Esegbonaa, Alfred Cutnera, Peter Cuckowb, Sarah Creightona,*

Gonadectomy is indicated in some children and teenagers with intersex disorders. Traditionally, this has beenperformed by laparotomy. This paper is a retrospective evaluation of laparoscopic gonadectomy in 10 girls(age range 8 to 17 years). Clinical records were reviewed for diagnosis, indication for surgery, complicationsand recovery period. Operative notes were reviewed for details of technique and complications. In all cases,the procedure was uncomplicated and the maximum hospital stay was two days. Laparoscopic gonadectomyis an effective and safe procedure for children and teenagers with these rare conditions.

Introduction

In a number of intersex abnormalities with a Y chro-

mosome, gonadectomy is indicated. This is to reduce the

risk of cancer estimated as approximately 5% for structur-

ally normal testes (androgen insensitivity syndrome) to as

high as 30% with dysgenetic or streak gonads (gonadal

dysgenesis)1. Conventional surgery involves a laparotomy

with the disadvantages of open surgery and a prolonged

recovery period. It may also result in a large and unsightly

scar in a young girl. In a proportion of these cases, the

gonads are within the pelvis and are easily accessible for

laparoscopic surgery. Laparoscopic surgery has been

shown to improve patient recovery in other conditions2.

Methods

This retrospective study was set in a London teaching

hospital that is a tertiary referral centre for paediatric and

adolescent gynaecology. There are established links with

paediatric endocrinology and paediatric urology within the

trust and at a nearby paediatric hospital. It is also a tertiary

referral centre for advanced minimal access surgery.

The notes of all girls referred for gonadectomy by the

paediatric endocrinologists over a 32 month period were

reviewed. For inclusion in the review, the patient needed to

be female with a Y chromosome necessitating gonadec-

tomy, and the presence of mullerian structures. The data

collected from the clinical notes included indication for

surgery and details of any imaging defining gonadal posi-

tion. The type of procedure and the equipment used was

recorded. The notes were also reviewed for length of

hospital stay, complications and gonadal histology.

A carbon dioxide pneumoperitoneum was created using

a direct entry technique under vision through an umbilical

incision in younger patients and a Verres needle in older

patients. A four-port laparoscopy was performed. The

primary 10 mm port was inserted through the umbilicus

for the 10 mm telescope, two secondary 5 mm ports were

inserted, one at the same level lateral to the rectus sheath on

the left side and one on the right side. The fourth port was

placed in the midline in the suprapubic region. Laparo-

scopic inspection of the pelvis was carried out to determine

the presence of the gonads and to inspect the pelvic organs.

The gonads were removed after determining the course of

the ureters and the fallopian tubes were excised where the

gonads appeared continuous with them. Either bipolar

electrosurgery or harmonic scalpel energy was used for

haemostasis. At the end of the procedure, a 5 mm laparo-

scope was inserted through one of the lateral ports so that

the gonads could be extracted through the central umbilical

10 mm port. The umbilical incision was closed in layers

where a direct entry technique had been used. Absorbable

sutures were used for skin closure.

In one patient, a subcostal insertion of the primary port

was used in view of the previous midline incision. There

were marked bowel adhesions and two further ports were

inserted so that a window could be created in the adhesions

to visualise the pelvis and remove the remaining gonad. In

one other patient, one of the gonads lay in the inguinal

canal. This was approached laparoscopically and the gonad

was pulled into the abdomen via the internal inguinal ring

and excised. In three patients, one fallopian tube was

BJOG: an International Journal of Obstetrics and GynaecologyFebruary 2003, Vol. 110, pp. 210–212

D RCOG 2003 BJOG: an International Journal of Obstetrics and Gynaecology

PII: S1 4 7 0 - 0 3 2 8 ( 02 ) 0 2 3 1 4 - 5 www.bjog-elsevier.com

aDepartment of Obstetrics and Gynaecology, University

College London Hospital, UKbDepartment of Paediatric Urology, Great Ormond Street

Hospital for Children, London, UK

* Correspondence: Dr S. Creighton, Department of Obstetrics and

Gynaecology, University College London Hospital, Huntley Street,

London WC1E 6DH, UK.

removed as a result of its close proximity to the gonad and

in one patient both fallopian tubes were removed.

Results

Ten girls fitting the criteria for the study were seen in the

Department of Gynaecology from July 1999 to December

2001. The median age at surgery was 16 years (range 8 to 17

years). Seven of the patients had pure gonadal dysgenesis

(Swyer’s syndrome) and the other three had Turner’s

mosaic conditions with the presence of a Y chromosome

(XO/XY) and a female sex of rearing. In all patients,

ultrasound of the pelvis had confirmed the presence of a

uterus. In seven patients, ultrasound had also detected the

presence of gonads. In the eighth patient, a pelvic MRI had

located the gonads within the pelvis. In the ninth patient, the

MRI had located one gonad within the pelvis and one at the

internal inguinal ring. In the final patient, the gonads could

not be identified by ultrasound or MRI although the

presence of a uterus was confirmed on both. Nine of the

patients had no prior pelvic surgery. One patient had

undergone midline laparotomy at the age of three years

followed by radiotherapy for a malignant dysgerminoma. In

all cases, an intravenous urogram showed normal urinary

tracts.

Eight of the patients were discharged the following day.

Two remained two days after surgery as a result of

discomfort. All were reviewed in the outpatient department

six weeks following surgery and all made a full recovery.

All removed gonads were sent for histological evaluation.

The histology from one patient showed a unilateral gona-

doblastoma. In the other cases, all histology confirmed

streak gonads with no evidence of malignancy.

Discussion

Gonadectomy is indicated in children with intersex

disorders harbouring Y chromosomal material as a result

of the malignant potential of their gonads1. A variety of

tumours have been found to be frequently associated, the

most common being the gonadoblastoma, which was found

to a significant degree in a large case series of women with

a Y chromosome3.

Traditional surgery for those with intersex disorders is a

laparotomy and bilateral gonadectomy. However, with

increasing experience, laparoscopic evaluation of the pelvic

structures and removal of intra-abdominal gonads has

become a part of the management of intersex disorders. It

is more acceptable to the patient than laparotomy. The

widespread use of laparoscopic gonadectomy in adults has

prompted its use in the treatment of children and adolescents.

Laparoscopy is well suited in this age group conferring

advantages regarding post-operative recovery, length of

hospitalisation and improved cosmesis. These advantages

may reduce psychological trauma4—a significant factor in

intersexed children. There are already cases where laparo-

scopic gonadectomy has been performed in children with

no significant complications5 and these correlate well with

our series.

Appropriate timing of gonadectomy hinges on an under-

standing about the different intersex disorders. The chances

of neoplasia and virilisation in the dysgenetic or streak

gonads found in some conditions at puberty are estimated at

30%1. It has been shown that tumours can arise as early as

six months1. In our cohort of 10 patients, one had a

neoplasm and one had already developed a gonadal malig-

nancy at the age of three years. In this particular group of

diagnoses, early gonadectomy is indicated because of the

high malignancy risk. None of the patients in this series had

complete androgen insensitivity syndrome. In complete

androgen insensitivity syndrome, the gonads are essentially

normal gonads with an approximately 5% risk of tumour

disease. With complete androgen insensitivity syndrome,

there is also no risk of virilisation at puberty as a result of

lack of androgen response and so a prophylactic gonadec-

tomy relatively late, at age 16 to 18, to allow the comple-

tion of secondary sexual development may be advocated.

Another consideration is what should be done with

normal mullerian structures. Preservation of the uterus is

important because there have been reports of pregnancy by

ovum donation in this group of women6. However, with

regard to the fallopian tubes, it has been suggested that

complete removal of the gonads can be best accomplished

by adnexectomy rather than gonadectomy. This may be

especially when the gonads are elongated, attenuated and

closely approximated to the fallopian tube. It has also been

suggested that as intrauterine embryo transfer is the pre-

ferred technique during IVF, there is less importance in

preserving the fallopian tubes. There may, however, be a

psychological advantage to leaving the fallopian tubes and

in this series we have conserved them where conservation

did not compromise complete removal of gonadal tissue.

In these rare and complex cases, it is important that there

is thorough investigation and evaluation by paediatric

gynaecologists, endocrinologists, urologists and geneticists

pre-operatively. A combination of ultrasound and magnetic

resonance imaging to determine the exact location of the

gonads allows appropriate surgical planning. Laparoscopic

evaluation can also serve as a useful surgical adjunct

enabling better visualisation and localisation of the gonads

and mullerian ductal remnants as well as the uterus and

vaginal components of the urogenital sinus5.

In conclusion, laparoscopic gonadectomy should be

accepted as the treatment of choice in children and adoles-

cents with these rare conditions. It is effective and safe with

a short recovery period allowing a speedy return to school.

It should be offered within a specialist unit offering the full

range of multidisciplinary medical and psychological sup-

port for children, teenagers and indeed adults with these

complex conditions.

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Accepted 12 November 2002

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