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The Developmental Endocrinology Branch National Institute of Child Health and Human Development Bethesda, Maryland 20892 Gordon B. Cutler, Jr. Eli Lilly and Company Lilly Research Laboratories Indianapolis, Indiana 46285
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Introduction |
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The recent availability of smaller instruments has expanded the application of laparoscopy to children and infants. Surgical applications include contralateral inguinal exploration in children with unilateral hernias, appendectomy, orchiopexy, cholecystectomy, splenectomy, Nissen fundoplication, and gastrostomy/jejunostomy. A recent retrospective review of 574 pediatric laparoscopic surgeries showed an overall complication rate of 2% (7), including significant hemorrhage controlled after conversion to laparotomy (n = 4), unintentional esophagotomy during a Nissen fundoplication (n = 1), technical errors (malpositioned Nissen fundoplication and improper gastrostomy tube placement) requiring repeat surgery (n = 2), hernia at the umbilical trocar site used for contralateral inguinal exploration (n = 2), and cellulitis at the trocar site used for gastrostomy tube placement (n = 3). A change in surgical technique and the use of a separate site for gastrostomy tube and trocar placement prevented reoccurrence of the latter 2 complications. All episodes of hemorrhage occurred during the first 5–10 procedures. Similar to the adult experience, intraoperative complications occurred early in the use of laparoscopy and decreased with time.
The two most serious potential intraoperative complications of laparoscopic surgery, including adrenalectomy, are hemorrhage and unintentional viscus injury. Overall, intraoperative blood loss has been less in laparoscopic adrenalectomy than open surgical procedures in adult patients (3, 4, 6), and a similar experience is expected in pediatrics. Unintentional viscus injury in both pediatric laparoscopic procedures and laparoscopic adrenalectomy in adults is rare, but risk is probably greatest in the smaller size child. In general, unintentional viscus injury during laparoscopic procedures in children has occurred primarily in the early surgical experience (7), reinforcing the importance of surgical expertise. Once the appropriate training and experience is achieved, advantages of the laparoscopic approach in children include faster recovery, less postoperative pain, and improved cosmetic appearance. Laparoscopic surgery is gaining acceptance at major pediatric medical institutions, however long-term follow-up is lacking because of the novelty of this approach, and laparoscopic adrenalectomy is rarely performed.
Adrenalectomy is rarely indicated in children. The most common pediatric adrenal tumor requiring adrenalectomy is neuroblastoma, a neural crest cell tumor. Neuroblastoma in children less than 15 yr old occurs in approximately 1 in 100,000 per year, with the majority of cases occurring in the first 5 years of life (8). In general, a laparoscopic approach is contraindicated in the surgical resection of most carcinomas because of the need for extensive exploration and concern of tumor spillage. These concerns may not be applicable to a subset of neuroblastomas detected before 1 yr of age, which have a good prognosis and may not warrant aggressive therapy. In Japan, where a neuroblastoma screening program of 6-month-old infants exists, laparoscopic adrenalectomy was recently performed in 3 infants (8–9 months old) with screening-detected neuroblastomas who had a favorable prognosis (9). To our knowledge, this is the only report of laparoscopic adrenalectomy in infants. The child who has a neuroblastoma with a less favorable prognosis is not a good candidate for a laparoscopic approach.
Endocrine tumors of the adrenal gland are rare in childhood. When present, the risk of cancer is greater in children than adults, and complete surgical resection is the treatment of choice. The annual incidence of childhood adrenocortical carcinoma below age 15 is estimated to be 0.3 per million children (10). Childhood adrenocortical carcinoma typically presents as virilization and/or Cushing’s syndrome and, in children, Cushing’s syndrome due to an adrenocortical tumor is suspicious for carcinoma. Rupture of the capsule and tumor spillage are potential complications of childhood adrenocortical tumor resection (10). Thus, a laparoscopic approach is contraindicated when adrenocortical carcinoma is suspected.
Benign pediatric adrenal tumors that are amenable to a laparoscopic approach include pheochromocytoma and aldosteronoma. Aldosteronomas are extremely rare in children, with 15 reported cases to date (11). Pheochromocytoma is also rare in childhood and may be associated with familial syndromes such as neurofibromatosis and von Hippel Lindau disease. Pheochromocytoma associated with MEN2 syndrome is typically diagnosed later in life, in the 5th and 6th decades.
Primary pigmented nodular adrenocortical disease (PPNAD), a rare cause of Cushing syndrome that often presents in childhood, is characterized by nonadrenocorticotropic hormone-dependent hypersecretion of cortisol by multiple pigmented nodules of adrenocortical cells. PPNAD occurs as part of the Carney complex, an autosomal dominant multisystem syndrome. Other manifestations of the Carney complex include spotty skin pigmentation, mesenchymal tumors (myxomas), endocrine neoplasias (growth hormone-producing pituitary adenoma, testicular Sertoli cell tumor, thyroid tumor), and schwannomas (12). Treatment for PPNAD consists of bilateral adrenalectomy. These patients should be good candidates for a laparoscopic approach.
The use of adrenalectomy as a treatment option in classical congenital adrenal hyperplasia (CAH) has been proposed recently (13). Two treatment approaches have been suggested: adrenalectomy during the second 6 months of life, done concurrently with genitoplasty in girls, and adrenalectomy in select CAH patients that are difficult to manage with conventional medical treatment. To date, 4 cases have been reported in the literature (14, 15, 16, 17), all in difficult to control cases. In addition, at the National Institutes of Health, we performed adrenalectomy on a 17-yr-old with virilization due to CAH that could not be controlled satisfactorily with medication, and other centers have begun performing this procedure on select cases. To our knowledge, adrenalectomy was performed via a laparoscopic approach in only one CAH patient, a 14-yr-old girl with 11ß-hydroxylase deficiency (16).
Adrenalectomy in CAH is an investigational procedure. A laparoscopic approach has the potential to minimize the surgical risks; however, sufficient numbers of patients are needed to acquire the necessary expertise, especially in infants. Modifications to the standard laparoscopic technique used in adults are necessary when the procedure is used on infants and small children because of the small peritoneal cavity (9). Ideally, laparoscopic adrenalectomy in children should be performed by surgeons with pediatric expertise in both adrenalectomy and laparoscopy, however, there is currently little experience in this surgical modality in children. Alternatively, the larger child or adolescent may have laparoscopic adrenalectomy performed by surgeons with experience in this procedure in adults. Currently, in order to maximize operative experience, pediatric laparoscopic adrenalectomy is best done at a large tertiary care center.
Prospective randomized clinical trials comparing new surgical procedures (i.e. laparoscopic adrenalectomy) to well-established surgical approaches (i.e. adrenalectomy by open laparotomy) do not exist, nor is there data on the community hospital experience. Based on available retrospective data of the adult experience at major medical centers, the laparoscopic approach appears to be the preferred surgical modality for adrenalectomy once the appropriate surgical expertise is achieved. We predict that this will also be observed in pediatrics. The incidence of classical CAH is estimated to be 1:15,000 (18), which is much more common than other pediatric indications for adrenalectomy. Thus, adrenalectomy may become a more commonplace pediatric procedure if the long-term follow-up of adrenalectomized CAH patients looks promising. As the adult experience with laparoscopic adrenalectomy expands, this approach will be applied more frequently in the pediatric population and will most likely become the preferred approach except when a potentially invasive malignancy is suspected.
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This article has been cited by other articles:
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  D. P. Merke, M. F. Keil, J. V. Jones, J. Fields, S. Hill, and G. B. Cutler Jr. Flutamide, Testolactone, and Reduced Hydrocortisone Dose Maintain Normal Growth Velocity and Bone Maturation Despite Elevated Androgen Levels in Children with Congenital Adrenal Hyperplasia J. Clin. Endocrinol. Metab., March 1, 2000; 85(3): 1114 - 1120. [Abstract] [Full Text]
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