Experience of Laparoscopic Exploration and Gonadectomy in lntersex Children

The use of laparoscopic technique to diagnose and treat intersex children is gradually introduced in clinical urology. From 1985 to 1996, abdominal exploration and gonadectomy were performed in 11 intersex children together with urogenital endoscopy and genitoplastic surgery in our institutes. Their median age was 6.0 (range 0–15) years old and initial gender sex was female in 8 and male in 3. The initial 4 cases (group 1) underwent open abdominal exploration together with gonadectomy, while the latter 7 cases (group 2) underwent laparoscopic exploration simultaneously with 3 laparoscopic gonadectomy and 1 open one via a inguinal incision. Their final diagnoses were male pseudohermaphroditism in 4 cases, mixed gonadal dysgenesis in 3, true hermaphroditism in 2, XX gonadal dysgenesis in 1, and XY gonadal dysgenesis in 1. Consequently, 2 of initial male were reared as a female. Operation time, use of analgesics, postoperative hospital stay and postoperative complications were not significantly different between the two groups, however, postoperative abdominal wound appearance was more acceptable in group 2. The most significant advantage of laparoscopic surgery in intersex children is cosmetic appearance especially when social gender is determined as female irrespective types of intersexuality.


INTRODUCTION
Recently laparoscopic surgery has been widely used in the field of urology. As a minimally invasive therapeutic modality, laparoscopic surgery has been applied for adrenalectomy, nephrectomy, pelvic lymphnode dissection and others. On the other hand, laparoscopy in nonpalpable testis or intersex has been used as a diagnostic tool for defining internal gonadal and ductal structures as well as their vascular supply. Observational laparoscopy can determine the subsequent appropriate 128 K. MORITA et al. management for gonad, which could be performed with open surgical as well as laparoscopic procedures.
Until 1990, children with ambiguous genitalia or incompatibility between gonad and genital expression underwent explorative laparotomy in our institute. After laparoscopic technique was introduced in 1991, we had performed diagnostic and therapeutic laparoscopic procedures on 7 intersex children for 5 years. The laparoscopic procedure and advantage of this technique were reported historically compared with open laparotomy technique.

PATIENTS AND METHODS
From 1985 to 1996, 11 children aged 3 months to 15 years old were referred to our institute for evaluation and management of intersex disorders (Table I). Eight children were raised as female and 3 as male. Five patients presented with ambiguous genitalia, 3 with growth retardation, each of 3 with nonpalpable testes, vaginal atresia associated with unilateral renal agenesis, and congenital adrenal hyperplasia, respectively. One (case No. 6) is a patient of Drash syndrome who had undergone radical nephrectomy for Wilm's tumor at 15 months old and has proteinuria due to glomerulopathy. Case No. 11 is a patient of lipoid adrenal hyperplasia who was born with pigmentation and general weakness. Enzymatic deficiency in the initial step of steroid synthesis pathways was elucidated by thorough endocrinological analysis.
Intraperitoneal pelvic exploration was indicated additional laparoscopic surgery was required, 2 or 3 surgical ports were further inserted as a working port. No preoperative nasogastric tube was inserted and postoperative intraperitoneal drain was placed for 1-2 days when necessary.

Surgical Procedures in 11 Patients
Initial 4 cases in group underwent explorative laparotomy. In 2 patients bilateral intraperitoneal gonadectomy was performed, while bilateral orchiectomy via local or inguinal incision was performed in other 2 patients (Table II). Urogenital endoscopy was simultaneously performed in 3 to confirm the anatomy of urogenital tract and simultaneous genitoplastic surgery was also done in 2.
In the latter 7 cases in group 2 which underwent laparoscopic exploration, 4 gonadectomies (laparoscopic procedure in 3, open inguinal approach in 1), 6 urogenital endoscopies and 2 genitoplastic surgeries were performed as one stage operative session (Table II).
Following evaluation about gonadal and ductal structures by laparotomy or laparoscopy (Table III), preferable gender was determined.
Gender assignment was transformed from male to female in 2 patients (Table I)

Advantages of Laparoscopic Exploration
Mean total operation time in group and 2 was 215 and 216min respectively (Table II). Mean   Long-term wound result of appearance in two each surgical approaches was presented in Figs. 2 and 3. Wound scar of surgical ports which had been used for laparoscopic procedure was neg-ligible (Fig. 3). Regarding cosmetic aspects the results of laparoscopic group were superior.

DISCUSSION
The indications of abdominal exploration in children with intersex disorders have been well presented [1]. Abdominal exploration is required in  these children to establish a diagnosis and adjus-morphological and histological confirmation table gender assignment as well as to ablate dys-about gonads and internal ductal structures. Just genetic gonads which may have a malignant after the laparoscopic evaluation, ablative treatpotential in the future [2,3]. The use of laparoment can be simultaneously performed such as scopic technique in intersex children is not anec-gonadectomy, herniorrhaphy and hysterosalpindotal any more, but now widespread for the gectomy along with the diagnosis and gender evaluation and management of these children identity [6]. Advantage of laparoscopic procedure [4,5]. Laparoscopic surgery for intersex children over explorative laparotomy is especially seen in has two significant aspects: diagnostic and abla-such a case in whom ablative surgery is not tive purposes. Diagnostic procedure includes necessary (Nos. 7, 9, 10 and 11 in our series).  [7]. We properly inserted the first trocar by open-laparotomy technique in all cases to avoid inadvertent injuries. As long as these miniature techniques are considered, laparoscopic exploration and combined genitoplastic surgery could resolve medical and social problems for intersex patients minimal-invasively in early childhood [8]. Laparoscopy has been obviously effective in the diagnosis of a variety of intersex spectrums. The presence and maturity of internal pelvic organs can be documented clearly through a finevision optical lens in magnified view and any gonadal structures can be biopsied or removed if indicated. In intersex patients diagnostic exploration would appear to be better performed laparoscopically than by an open surgical approach [4]. In our study operation time, use of analgesics, postoperative hospital stay period (which would be longer in Japan thanks to the full government coverage) and postoperative complications were not different between the two groups.
However, laparoscopic inspection of internal gonad could be easily performed in shorter time with accuracy compared with open procedure. We could appropriately make the therapeutic surgical strategy including laparoscopic gonadectomy. There is no doubt that laparoscopy is a safe and reliable diagnostic tool even for intersex child.
Our data presented relatively longer hospital stay of patients than that in other countries. It could be explained by Japanese medical pay system. In Japan children with inborn abnormalities are priviledged to pay no expense for the diagnosis and management of main illness. Under these backgrounds, demands for shorter hospital stay are not of parents or ours.
Moreover, we can stress the cosmetic advantage of laparoscopic surgery in children. Most patients with ambiguous genitalia, especially those with nonpalpable gonads, were assigned as a female gender (regarding to the legal sex, 10/11 were female in this series). Indeed in these patients cosmetic outcome of the genitoplasty is one of the most important factors, while better cosmetic abdominal image should be also desirable for their social life in the future.

CONCLUSIONS
Laparoscopic procedure is identical with open exploration for diagnosis and treatment in intersex patients but provides a more acceptable cosmetic result. This technique should become a standard diagnostic and therapeutic method in pediatric urology.