13.2.1 Who Should Perform These Operations, and in What Location?

The make-up of the team will depend on the age and maturity of the patient and the condition with which they present. For example a 16-year-old with an ovarian cyst can be managed in a similar manner to a young adult in terms of anaesthetic and operative techniques. Conversely, a 12-year-old with a complex Müllerian duct anomaly or a difference of sex development may require a paediatric anaesthetist, paediatric urologist, paediatric surgeon, clinical psychologist and a paediatric endocrinologist. In addition the hospital environment for surgical recovery is important and needs to take into account the age of the patient and the potential need for parental support. A room covered in cartoon characters with lights-off at 7:00 PM will put a young child at ease but would not be appropriate for a teenager. However, in most units adolescents are not treated on adult wards as this is not the correct environment.

It would be unusual for an individual to have all the correct knowledge and skills required and hence a team approach is likely to optimise outcome. Some of the laparoscopic surgery will be complex and it is unlikely that an expert PAG consultant who deals with all other aspects of patient management will have a sufficient laparoscopic workload to enable adequate skill acquisition. Likewise an expert laparoscopic surgeon who carries out a heavy surgical workload is unlikely to have sufficient knowledge surrounding all the other aspects of care. Thus a team approach of a PAG specialist (with the correct knowledge) operating with an adult laparoscopic specialist with the skills of dissection of the pouch of Douglas, uterovesical fold and pelvic sidewall and proficiency in laparoscopic suturing will optimise the surgical outcome. This approach is common in units offering complex surgery to this group of patients in the United Kingdom. We would encourage anyone endeavouring to perform complex laparoscopy in the PAG setting to foster this working relationship.

13.2.2 Planning Surgery

The preoperative assessment in making the diagnosis and determining the indications for surgery are dealt with in the relevant chapters in the book. This section will address the surgery.

The suitability for laparoscopic surgery needs to take into account the specifics of the surgery itself and also general considerations. Previous abdominal surgery and the size of the patient may determine the method for obtaining a pneumoperitoneum. Previous abdominal surgery increases the risks of adhesions and hence organ damage during primary port insertion.

The requirement to use a uterine manipulator should be discussed with the patient and her family during the preoperative period, especially with girls who have never been sexually active. This may have significant social and religious implications, as there is a risk that the hymen may tear.

In procedures where there is a Müllerian duct anomaly preoperative knowledge of the renal tract is essential as it is important to know whether there is an absent kidney or a duplex system. This information will be required during surgical dissection of the pelvic sidewall. In XY females, preoperative MRI will in most cases locate the site of the gonads and hence enable preoperative planning of the surgical approach and potential requirements of a paediatric urologist where groin dissection may be required.

13.2.3 Theatre Set-up

Surgery requires an effective team with each member having a specific role. The familiarity of the anaesthetic, scrub and circulating staff with each other, with their equipment and with the procedures being performed will have a direct effect on the smooth running of each case.

The theatre environment has to be fit for purpose. Advanced laparoscopic theatre set-ups may reduce stress in the operating theatre and minimise risks to staff and patients. The layout of equipment in the operating room has become more important as the technology has increased. When open surgery was the norm a single diathermy machine and a suction bottle were the only devices that needed to be near the operating table. With laparoscopic surgery requirements include newer energy machines such as ultracision and advanced bipolar, insufflator stacks, suction/irrigation set-ups and multiple high-definition monitors and control screens. The layout needs to facilitate flow of equipment to the operating table without obscuring the surgeons’ view of the monitors. Ideally cables running along the floor should be minimised as they represent a trip-hazard in the low-light conditions of a laparoscopic theatre. Having an integrated system that allows the surgeon to control gas-flow, light intensity and the recording of images rather than requesting circulating staff to do it saves time and improves efficiency.

State-of-the-art theatres result in a quietly flowing theatre where staff feel less stressed, which allows the team to concentrate on the operation itself. This results in a more efficient and relaxed surgical environment, which enables more complex surgery to be carried out in a safer manner.

13.2.4 Laparoscopic Techniques and Equipment

Most laparoscopic pelvic procedures are performed in the Lloyd-Davies position to enable access to the vagina if required. Prior to insufflation the bladder is emptied either by an in-out catheter for short procedures or an indwelling catheter for longer procedures or ones in which the bladder has to be filled to help identify it. At the end of the procedure this can be removed if the operation was only minor. Consideration should be given to leaving the catheter overnight as trying to catheterise a child who goes into urinary retention postoperatively can be traumatic. If the uterus is present and the operation involves inspection of the pouch of Douglas or uterine manipulation, then the uterus is instrumented.

There are three main methods used to obtain a pneumoperitoneum: insertion of a Veres needle at the umbilicus, open entry (Hasson) and subcostal approach (Palmer’s entry). Many patients in this age group will be more susceptible to vascular injury from a standard umbilical Veres technique due to the short distance from the umbilicus to the major abdominal blood vessels. To minimise the risk of vascular injury a Hasson entry technique should be considered especially in thin patients.

Umbilical entry is not suitable, whether via a closed or open technique, where there is an increased risk of adhesions under the umbilicus. An alternative entry site should be used. A Veres needle or a direct optical entry at Palmers point (left upper quadrant 2 cm below the costal margin) provides a relatively safe entry into the abdomen, allowing the inside of the umbilicus to be inspected and a port placed if free of adhesions. Where a subcostal entry point is utilised there is an increased risk of damaging the stomach if it is distended. An oro-gastric tube should be placed at the start of the operation.

Instrumentation would largely reflect the same used in adult surgery. Minimising the number and size of the ports should be considered to enhance the cosmetic result. However, this should not be at the expense of safe, efficient surgery. For most operations we utilise an umbilical 5 or 10 mm port for the laparoscope and 2 lateral ports in line with the umbilicus and very lateral. This enables good triangulation during surgery. For complex cases we also insert a suprapubic 5 or 10 mm port to enable additional manipulation by the assistant. In cases where further retraction would be useful we insert needles and suspend structures with sutures to carry this out without the need for extra ports.

13.3.1 Appearance and Severity of Endometriosis

Most adolescents who have endometriosis (>60%) will have early-stage disease confined to the pelvis but advanced endometriosis has been described. A major risk factor of disease severity is a Müllerian duct anomaly resulting in an outflow obstruction. The incidence of endometriosis in this group of adolescents with genital tract anomalies varies between 6% and 40%. Surgical treatment of the outflow obstruction will often result in improvement or even spontaneous resolution so many surgeons do not treat the endometriosis at the initial operation. The risk of severe disease also appears to increase with advancing age and early menarche and this goes along with the thinking that endometriosis is a progressive disease that gets worse with increasing number of menstrual cycles.

It should be noted, however, that adolescents with endometriosis often have subtle atypical lesions that are clear, white or red, and not the powder-burn lesions commonly seen in adults. Familiarity with atypical lesions is important at the time of laparoscopy in making the correct diagnosis.

The goal of laparoscopic surgery is to make a diagnosis and to treat the disease conservatively in the hope of reducing pain whilst preserving fertility. Treatment with either resection or ablation of endometriotic lesions and postoperative medical therapy has been shown to result in clinical improvement in symptoms. However, in adults the evidence suggests that excision may be better than ablation when treating endometriosis and that is the approach taken by the authors. An early diagnosis is believed by many authors to be an opportunity to intervene in the progressive nature of the disease. Unfortunately, the recurrence of pain and/or disease is a significant problem and appears to occur regardless of postoperative adjuvant therapy. It is not surprising that the need for a second operation to treat recurrent symptoms has been reported to be as high as 34% 5 years postoperatively in adolescent patients.

13.4.1 General Considerations

The first consideration when deciding on surgical treatment of a presumed benign ovarian mass in children is preservation of ovarian function. An ovarian cystectomy is always preferred over an oophorectomy as many follicles are left behind after cystectomy and can serve as oocytes for reproduction in the future.

Among adolescents, the most common benign ovarian masses are functional cysts and benign neoplasms. There is a bimodal distribution of functional cysts, peaking during the fetal/neonatal and perimenarchal ages. As these cysts are usually benign and resolve spontaneously, every effort should be made to manage these cysts expectantly with serial ultrasound prior to considering surgery.

Paratubal and paraovarian cysts may mimic simple ovarian cysts in both presentation and imaging. Surgical management is usually suggested for any adnexal cyst greater than 5 cm that fails to regress. Surgical intervention will prevent potential torsion as well as provide a histological diagnosis. Fortunately, the majority of ovarian cysts can be managed by laparoscopy.

Neoplastic ovarian masses in the paediatric and adolescent population include tumours of germ cell, epithelial, sex cord stromal and metastatic from other primary sites.  Germ cell tumours are the most common histological subtype in adolescents. Because non-epithelial masses predominate in the adolescent, the following discussion will focus on the most common benign germ cell tumour, the mature cystic teratoma.