Adolescent inguinal hernia repair: a review of the literature and recommendations for selective management.

BACKGROUND: The choice of how to repair inguinal hernias in adolescents has historically been a matter of experience and differed between pediatric surgeons who traditionally performed a high ligation of the sac and general surgeons who typically perform a repair using mesh. This up-to-date review thoroughly examines the subject and discusses the suitability of both types of repairs in this unique age group. METHODS: A 20-year Pub Med search was performed for the following terms: adolescent hernia repair including reports of mesh hernia repair in adolescents and postoperative complications including chronic inguinal pain and recurrences. RESULTS: The evidence in the literature suggests that while there appears to be no difference between the two types of repairs with regards to recurrence and complications, changes in the pelvic floor physiology in adolescents suggest that a selective, individualized approach can be recommended depending on the size and nature of the presenting pathology. CONCLUSIONS: A selective approach to the inguinal hernia in adolescent patients based on the size of the defect appears justified.

The current role of laparoscopic surgery for gastroesophageal reflux disease in infants and children.

BACKGROUND: The benefits of surgery for gastroesophageal reflux disease (GERD) in infants and children have been questioned in the recent literature. The goal of this review was to determine the best current practice for the diagnosis and management of this disease. METHODS: The literature was reviewed for all recent English language publications on the management of GERD in 8- to 10-year-old patients. RESULTS: In infants and children, GERD has multiple etiologies, and an understanding of these is important for determining which patients are the best surgical candidates. Proton pump inhibitors (PPIs) have become the mainstay of current treatment for primary GERD. Although laparoscopic surgery appears to be better than open surgery, there remains some morbidity and complications that careful patient selection can minimize. CONCLUSION: Surgery for GERD should be performed only after failure of medical management or for specific problems that mandate it.

Two-year experience with minimally invasive herniorrhaphy in children.

BACKGROUND: Laparoscopic herniorrhaphy in pediatrics is rarely performed. We evaluated our 2-year experience of minimally invasive inguinal herniorrhaphy in children. METHODS: All procedures were performed under general anesthesia using <2-mm instruments and scopes and a surgical awl to accomplish high ligation of the hernia sac under direct vision. RESULTS: A total of 90 consecutive children (76 males and 14 females) older than the age of 6 months underwent a minimally invasive herniorrhaphy (60 unilateral and 30 bilateral; total of 120 hernias repaired). Seventeen children underwent herniorrhaphy in conjunction with another procedure. All children who underwent herniorrhaphy alone were discharged immediately and allowed unrestricted activity. Only four patients requested a narcotic analgesic. There was one recurrence early in the series (0.83%), prompting a change in technique. CONCLUSIONS: Minimally invasive inguinal herniorrhaphy in children is a safe alternative for the experienced pediatric laparoscopist. There is a similar recurrence rate as that of the traditional open approach with a superior cosmetic result.

Lessons learned from a failed multi-institutional randomized controlled study.

BACKGROUND/PURPOSE: In 1996, the Surgical Sections of the Children's Cancer Group (CCG) and the Pediatric Oncology Group (POG) received National Cancer Institute funding to conduct a prospective, randomized, controlled, surgeon-directed study to evaluate the role of minimally invasive surgery (MIS) in children with cancer. Because of lack of patient accrual, the study was closed in 1998. The purpose of this study is to evaluate and describe those factors that impacted on study failure to ensure future successful clinical trials. METHODS: One hundred forty surgeons representing the surgical membership of CCG and POG as well as 111 institutions within CCG and POG were asked to complete a questionnaire about the failed clinical trial. The questionnaire focused on study objectives, organization, and institutional review board (IRB) submission. It also examined the surgeon's ability to perform the minimal access operation, the influence of the pediatric oncologist, and the existence of preconceived biases by surgeons, oncologists, and families. Statistical analysis was performed as appropriate. RESULTS: Eighty-six of 140 (62%) surgeons responded to the questionnaire. Only 23% of the potential protocols were submitted for IRB approval. Of responding surgeons, 39% were not actively performing MIS when the study opened. A surgeon's support of the study was directly related to when the surgeon received the protocols (P <.001) and whether the participating surgeon was actively participating in MIS (P <.016). The oncologist's knowledge and support of the study affected IRB submission and approval (P <.02) and was influenced by whether MIS was practiced at the institution (P <.05). The majority of responding surgeons believed the experimental question was relevant (P <.05). However, responding surgeons believed that a preconceived bias existed within both their local surgical and oncology communities favoring a particular surgical approach (P <.001), but this bias did not extend to the families (P >.05). CONCLUSION: The study failed because of lack of accrual for a variety of reasons: failure to submit to the institution's IRB, lack of surgical expertise with MIS procedures, and preconceived surgeon bias toward either an endoscopic or traditional open approach.

The role of laparoscopy in the management of common bile duct obstruction in children.

BACKGROUND: Laparoscopic common bile duct exploration is commonplace in adults; however, this procedure is not often performed in children. The goal of this study was to evaluate the results of laparoscopic common bile duct exploration in children. METHODS: Of 50 patients undergoing laparoscopic cholecystectomy, six patients (12%) had obstructing lesions of the common bile duct (CBD). Five children underwent laparoscopic common bile duct exploration, and one child had a preoperative endoscopic sphincterotomy and stone removal. RESULTS: The mean age at laparoscopic CBD exploration was 11.6 years (range, 5-16). The obstructing lesion was visualized by intraoperative cholangiography in all five patients. The mean operative time for laparoscopic cholecystectomy along with CBD exploration was 215 min (range, 160-282). The transcystic laparoscopic CBD exploration was performed using a 7-Fr, multichannel rigid, or 10-Fr flexible fiberoptic cystoscope. The stones were either pushed into the duodenum with the scope or extracted through the cystic duct using a 3-Fr Segura basket. In one patient, a candidial ball disintegrated during an attempt to remove it with the basket. A repeat cholangiogram at the end of each procedure showed an anatomically normal CBD with free flow of contrast into the duodenum. All patients enjoyed a quick recovery. They were started on a regular diet on the same day of surgery and discharged on the 1st or 2nd postoperative day. One patient with sickle cell disease developed a pulmonary infarction and required 5 additional days of hospitalization. One patient developed recurrent choledocholithiasis 6 months after laparoscopic exploration and was treated successfully with endoscopic sphincterotomy and stone extraction. CONCLUSIONS: Laparoscopic CBD exploration can be performed safely at the time of the cholecystectomy in children. Endoscopic sphincterotomy before cholecystectomy is not necessary. We recommend laparoscopic CBD exploration for obstructing lesions of the CBD. Endoscopic sphincterotomy should be reserved for recurrent lesions of the CBD after laparoscopic cholecystectomy.

Controversies in the management of paratesticular rhabdomyosarcoma: is staging retroperitoneal lymph node dissection necessary for adolescents with resected paratesticular rhabdomyosarcoma?

PURPOSE: Use of retroperitoneal lymph node dissection (RPLND) in paratesticular rhabdomyosarcoma (PTRMS) is controversial and has changed over the past 2 decades. The Intergroup Rhabdomyosarcoma Study Group (IRSG) required ipsilateral RPLND (IRPLND) for all patients with PTRMS treated on IRS-III (1984-91), but changed to clinical evaluation of RPLNs using computerized tomography (CT) in IRS-IV (1991 through 1997). In IRS-IV, only those patients with identified lymph node involvement on CT required surgical evaluation of the RPLNs. Nodal radiation therapy was administered only to patients with RPLNs recognized as positive; such patients received more intensive chemotherapy as well. Thus, they compared the incidence of recognized RPLN involvement using these 2 different approaches. They then analyzed patient outcome to determine whether this change in management affected outcome. METHODS: Eligible patients with group I or II PTRMS who were treated on IRS III (n = 100) or IRS IV (n = 134) were analyzed. Failure-free survival (FFS) and survival (S) rates were estimated using the Kaplan-Meier method and compared using the log-rank test. RESULTS: There was a significant change in the distribution of patients with group I versus II tumors from IRS-III to IRS-IV (group I, 68% in IRS-III versus 82% in IRS-IV). This was the result of decreased node recognition when CT was used to stage RPLNs in IRS-IV and was most notable for adolescents (>10 years of age). Overall, 3-year FFS was 92% for patients treated on IRS-III and 86% for those treated on IRS-IV (P =.10), whereas survival estimates were 96% and 92%, respectively (P =.30). Adolescents were at higher risk of RPLN relapse than were children (<10 years of age) and their FFS and survival were worse, regardless of IRS protocol. Furthermore, adolescents with recognized group II tumors experienced better 3-year FFS than those with group I tumors on IRS-IV (100% versus 68%, P =.06), most likely as a result of receiving radiotherapy and intensified chemotherapy. CONCLUSIONS: Use of only CT scan evaluation of RPLN in IRS-IV led to a decrease in identification of RPLN involvement in boys who present with localized PTRMS, and a higher rate of regional relapse as compared with IRS-III. Adolescents had much higher likelihood of RPLN disease, and they fared significantly worse than did younger children on both studies. Furthermore, adolescent boys with group I tumors experienced worse FFS than those with Group II tumors on IRS-IV, probably because some patients with group II tumors were not identified by CT imaging and thus received less effective therapy. These data suggest that adolescents should have ipsilateral RPLN dissection as part of their routine staging, and those with positive lymph nodes require intensified chemotherapy as well as nodal irradiation.

Laparoscopic pancreatectomy for persistent hyperinsulinemic hypoglycemia of infancy.

BACKGROUND: A 4-week-old male infant (4.9 kg) with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) underwent a laparoscopic pancreatectomy to evaluate its feasibility. Preoperative medications included diazoxide and glucagon to maintain adequate blood glucose levels. METHODS: Laparoscopic pancreatectomy was performed using a 5-mm cannula at the umbilicus, external fixation, transcutaneous suture-assisted gastric retraction to expose the lesser sac, and three additional 3.5-mm cannula sites. The pancreas was resected from the splenic hilum to the mesenteric vessels. The splenic vein was dissected from the under surface of the pancreas using electrocautery, and the spleen was easily preserved. Surgery time was 75 min, and minimal blood loss occurred. RESULTS: The child required no narcotic medication and tolerated a regular diet immediately after surgery. Serum glucose levels did decrease postoperatively, and the child required diazoxide, dextrose infusion, glucagon, and octreotide. On postoperative day 7, the child underwent an open near-total pancreatectomy, after which he remained asymptomatic. Essentially no scarring was found in the lesser sac, and the remaining pancreatic remnant was resected without difficulty. CONCLUSIONS: Laparoscopic pancreatectomy can be performed safely, even in a newborn patient, without prolonged operative time or unnecessary risk. The technique using external fixation and transcutaneous suture-assisted gastric retraction provides excellent exposure to the pancreas and lesser sac. In patients with PHHI, in whom reoperative additional pancreatectomy is very likely, this technique is the ideal initial surgical approach.

The association of elevated percent bands on admission with failure and complications of interval appendectomy.

BACKGROUND/PURPOSE: The routine use of interval appendectomy for the treatment of perforated appendicitis, with or without abscess, remains controversial. The purpose of this study is to confirm the efficacy of this approach and to identify factors associated with failures and complications. METHODS: All patients (n = 101) with their clinical diagnosis of perforated appendicitis confirmed with imaging were treated prospectively with fluids and intravenous antibiotics (clindamycin, ceftazidime) and were discharged home on oral fluids and analgesics regardless of fever. Intravenous antibiotics were continued at home until the patients were afebrile for 48 hours, and their white blood cell and differential counts were normal. Patients were readmitted at 8 to 12 weeks for an interval appendectomy. Failure to improve by 72 hours of antibiotic therapy mandated an early appendectomy. P values were determined by chi(2) analysis and Student's t test. RESULTS: The 79 patients (78%) successfully treated with interval appendectomy had an overall 6.3% complication rate, and total hospitalization averaged 5.2 days. The treatment in 21 of 22 patients (22%) requiring early appendectomy failed because of a clinical picture suggesting small bowel obstruction. The patients with the failed procedures had a complication rate of 50% and were hospitalized an average of 12.8 days. The overall complication rate for the 101 patients was 15.8%, and the overall total hospitalization was 6.9 days. Patients requiring early appendectomy had a more frequent finding resembling a small bowel obstruction on their initial x-ray (50% v 13%, P = .004) and a higher percent band count on their initial differential blood cell count (22.6% v 7.6%, P<0.0001) than did those successfully treated with interval appendectomy. An initial band count <15% was predictive of an uncomplicated course (84% positive predictive value). CONCLUSIONS: Interval appendectomy without complications is successful in the majority of patients with perforated appendicitis. An elevated initial band count > or =15% is associated with an increased likelihood of failure and complications.

Cholelithiasis and cholecystitis in children.

With advances in medical technology, including intensive care, new medications, alterations in the composition of parenteral nutrition, and the institution of minimally invasive surgery, our understanding of the spectrum of diseases of the gallbladder resulting in stone formation or inflammation, and the management of these disorders has changed over the past few decades. The discussion herein focuses on our thinking about the current diagnosis and treatment for these disorders.

Thoracoscopic thymectomy for myasthenia gravis in children.

The technique for thoracoscopic in children is described. The average operating time is under 2 hours, and the procedure appears to be safe and effective.

Aggressive surgery is unwarranted for biliary tract rhabdomyosarcoma.

BACKGROUND/PURPOSE: Rhabdomyosarcoma (RMS) of the biliary tract is rare, and, in addition to multiagent chemotherapy with or without radiotherapy (RT), some investigators recommend aggressive surgery. To assess the role of surgery, records of all 25 eligible patients with biliary RMS enrolled in IRSG studies I through IV from 1972 to 1998 were reviewed. METHODS: Treatment included surgery with or without vincristine, dactinomycin, cyclophosphamide, doxorubicin, cisplatin, etoposide, ifosfamide, and with or without RT. Data evaluated included clinical presentation, treatment, complications, and outcome. RESULTS: Diagnostic imaging identified the primary tumor but failed to identify regional metastases. Despite aggressive surgery, gross total resection at diagnosis was possible in only 6 cases, 2 of which had negative surgical margins. Although only 6 (29%) patients without distant metastases underwent gross total resection, estimated 5-year survival rate was 78% (95% CI 58%, 97%). Infectious complications were common and frequently associated with external biliary drains. Five (20%) died within the first 2 months, 3 of sepsis. CONCLUSIONS: Surgery is critical for establishing an accurate diagnosis and determining the extent of regional disease. Gross total resection is rarely possible despite aggressive surgery, and outcome is good despite residual disease after surgery. External biliary drains increase the risk of postoperative infectious complications.

Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: a preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991-1997).

BACKGROUND: During the fourth Intergroup Rhabdomyosarcoma (RMS) Study (IRS IV, 1991-97), a preoperative staging system was evaluated prospectively for the first time. The authors evaluated this staging system and the role of surgery in extremity RMS in contemporary multimodal therapy. METHODS: A total of 139 patients (71 girls; median age, 6 years) were entered in IRS IV with extremity-site RMS. Stage was assigned by the IRSG Preoperative Staging System. Postsurgical group was determined by tumor status after initial surgical intervention. Multivariate analysis was performed using all pretreatment factors that were significant by univariate analysis, including clinical Group (i.e., I through IV), tumor invasiveness (T1,T2), nodal status (N0,N1), and tumor size (< or > or =5 cm). Failure-free survival rates (FFS) and survival rates were estimated using the Kaplan and Meier method. RESULTS: Preoperative staging and clinical group distribution were as follows: Stage 2, n = 34; Stage 3, n = 73; Stage 4, n = 32; Group I, n = 31; Group II, n = 21; Group III, n = 54; Group IV, n = 33. Three-year FFS was 55%, and the overall survival rate was 70%. Eighty-seven patients had either unresectable, gross residual disease (Group III) or metastases (Group IV). FFS was significantly worse for these patients with advanced disease, compared with that for patients with complete resection or with only microscopic residual tumor (i.e., Group I or II; Group I, 3-year FFS, 91%; Group II, 72%; Group III, 50%; Group IV, 23%; P<.001). Lymph nodes were evaluated surgically in 76 patients with positive results in 38. Clinically, 13 additional patients had nodal disease. Both stage and group were highly predictive of outcome and were highly correlated. By multivariate analysis, none of the other variables were predictors of FFS. CONCLUSIONS: This review confirms the utility of pretreatment staging for stratification of patients with extremity RMS with widely different risks of relapse, thereby paving the way for development of risk-based therapy. Group (operative staging) remains the most important predictor of FFS, emphasizing the importance of complete gross resection at initial surgical intervention, when feasible without loss of limb function. The high incidence of nodal disease in the patients who had lymph node biopsy confirms the need for surgical evaluation of lymph nodes to ensure accurate staging in children with extremity rhabdomyosarcoma.

Analysis of the costs of surgery for Hirschsprung's disease: one-stage laparoscopic pull-through versus two-stage Duhamel procedure.

The purpose of this report is to evaluate the cost-effectiveness of a single-stage laparoscopic pull-through for Hirschsprung's disease compared to the traditional two-stage Duhamel procedure. In this series of 33 children, the length of hospitalization (2.5 +/- 3.5 vs 10.6 +/- 3.9, p < 0.01), cost (19,088 +/- 13,075 vs 34,110 +/- 19,443, p < 0.05), and complications were all significantly less with the laparoscopic assisted pull-through compared to the more traditional open approach.

Resection of foregut-derived duplications by minimal-access surgery.

Eight children underwent minimal-access surgery (MAS) for duplications of foregut derivatives. The efficacy and safety of this approach are reviewed. The seven patients with mediastinal lesions had video-assisted thoracoscopic resection. One lesion presented as a subdiaphragmatic esophageal diverticulum, which was excised laparoscopically. Between March 1991 and October 1997, eight children were treated. Mean age was 27 months and mean weight was 11. 4 kg. Mean operating time was 106 min, and mean postoperative hospital stay was 4.5 days (median = 2 days). Persistent air leaks occurred in two patients who had centrally-located bronchogenic cysts. One of these, who had undergone subtotal excision with laser photoablation of the remaining cyst mucosa, developed a recurrence that was excised at thoracotomy. We conclude that esophageal and bronchogenic cysts and duplications may be safely excised by MAS in children, with excellent cosmetic and functional outcome. Two technical points are noted: (1) a thoracostomy tube is required for central mediastinal lesions; and (2) complete excision is required to prevent recurrence.

Physiologic consequences of pneumonectomy. Long-term consequences of pneumonectomy done in children.

Lung resections in children are performed for a variety of reasons including congenital malformations, infections, bronchiectasis, and tumors. There are no long-term reports on pneumonectomy alone in children, but those on lung resection as a group state that children tolerate these operations well, with mild sequelae if any, and that the majority of them in adulthood can perform non-physically demanding jobs adequately. The authors' findings concur with the reports that younger patients can endure pulmonary resections with minimal functional limitations.

Progress in the surgical management of vaginal rhabdomyosarcoma: a 25-year review from the Intergroup Rhabdomyosarcoma Study Group.

BACKGROUND/PURPOSE: During its 25 years of experience, the Intergroup Rhabdomyosarcoma Study Group (IRSG) has completed four sequential prospective clinical trials to improve survival and decrease morbidity rates in childhood rhabdomyosarcoma (RMS). Surgical management of vaginal RMS has changed dramatically. METHODS: The records of 72 patients with localized vaginal RMS were reviewed to assess surgical therapy, chemotherapy, and radiotherapy and their relation to outcome. RESULTS: Each successive IRSG trial resulted in decreased need for surgical resection (IRS-I, 100%; IRS-II, 70%; IRS-III, 30%; IRS-IV, 13%) and excellent disease-free survival using increasingly effective multiagent chemotherapy. CONCLUSIONS: Primary chemotherapy after initial biopsy provides excellent tumor control. Local resection may be appropriate, but removal of organs (ie, complete vaginectomy/hysterectomy) has no role except in persistent or recurrent disease. Mature, residual rhabdomyoblasts may be evidence of a chemotherapy response, and further surveillance and biopsy without surgical resection is adequate treatment.

Does debulking improve survival rate in advanced-stage retroperitoneal embryonal rhabdomyosarcoma?

UNLABELLED: BACKGROUND, METHODS, AND PURPOSE: The authors examined demographic and clinical features, therapy, and outcome of patients with advanced (group III or IV) rhabdomyosarcoma (RMS) of the retroperitoneum and nongenitourinary pelvis treated in the Intergroup Rhabdomyosarcoma Study Group (IRSG) III (1984 to 1991, n = 41) or IV pilot (1987 to 1991, n = 53) studies to assess the role of initial debulking surgery. RESULTS: Ninety-four patients with retroperitoneal primary tumors and gross locoregional residual tumor (group III, n = 53) or metastatic disease (group IV tumors, n = 41) were treated with combination chemotherapy (ie, vincristine, dactinomycin, and cyclophosphamide with or without other agents plus radiation therapy, RT) after biopsy only or subtotal resection. These retroperitoneal tumors usually were invasive (T2, 76%). Most patients were younger than 10 years of age (n = 69, 73%), the male to female ratio was 1.4, and tumors usually were embryonal (n = 64, 68%). Overall 4-year failure-free survival (FFS) was 50%; survival was 60%. Survival rate was better for girls (4-year survival rate, 75% v49% for boys; P = .05) and was not significantly different for patients treated in IRS-III (66%) or IRS-IV pilot (52%). However, it was better for patients with embryonal versus alveolar or undifferentiated tumors (4-year survival rate, 70% v 42%; P = .002). In adolescents, RMS is different from that seen in children less than 10 years old; most cases are alveolar or undifferentiated (16 of 29, 55%). Surgery for most (21 of 24) patients with alveolar tumors comprised biopsy only. By contrast, of 64 patients with embryonal tumors, 39 (61%) underwent biopsy only, whereas 25 (39%) had debulking surgery. Patients whose tumors were debulked fared better than those whose tumors underwent biopsy only (4-year FFS rate, 72% v48%; P = 0.03). Patients with group IV embryonal tumors fared unexpectedly better than those with group IV alveolar or undifferentiated tumors (70% versus 42% 4-year survival rate, P < .05), and patients less than 10 years of age with group IV embryonal tumors had 4-year survival rate of 77%, indicating the importance of the biology of these tumors. CONCLUSIONS: Multimodal therapy, including multiagent chemotherapy plus RT, appears to improve survival rate in patients with advanced embryonal RMS arising in the retroperitoneum. These data suggest that debulking tumors of embryonal histology improves outcome further. This approach will be assessed in IRSG V.

Soft tissue sarcomas in children.

In childhood, soft tissue sarcomas comprise a complex group of malignancies of varied histologic subtypes, the prognoses of which depend on the histology, age, site, extent of involvement and a variety of other factors. This paper discusses the varieties of tumors classified as soft tissue sarcomas in childhood and the multimodal approach taken to cure these tumors, with particular attention to the details of difficult surgical problems.