Vaginal tumors in childhood: the experience of St. Jude Children's Research Hospital

Background/Purpose

The aim of this study was to retrospectively analyze the clinical presentation, histology, treatment, and outcomes of children with vaginal tumors who were treated at a single institution.

Methods

A retrospective review of medical records and pathologic materials of all children with vaginal tumors treated at St Jude Children's Research Hospital between 1970 and 2009 was conducted.

Results

Eighteen patients (median age, 3.7 years; range, 0.1-15 years) were identified. Three different histologies were found: rhabdomyosarcoma (RMS; n = 13), germ cell tumor (n = 3), and clear cell adenocarcinoma (n = 2). Bleeding or blood-tinged discharge was the most common clinical presentation (66%), followed by a protruding mass (39%). Vaginal and uterine salvage was 44.4% (8 of 18 patients). Thirteen patients (72.2%) remain disease-free, with a median follow-up of 23.2 years (range, 2-39 years). Four patients (22.2%) died of disease progression (1 RMS, 2 germ cell tumor, and 1 clear cell adenocarcinoma), and 1 patient with RMS died of colon cancer 12 years after the primary diagnosis had been made.

Conclusions

Vaginal tumors are extremely rare in the pediatric population. Early recognition of symptoms like bleeding and a protruding vaginal mass may prevent morbidity and mortality. Our findings confirm the good prognosis of vaginal RMS.     

Pediatric snakebites: lessons learned from 114 cases

Purpose

Evidence-based guidelines for the treatment of pediatric snakebite injuries are lacking because they occur infrequently in most centers.

Methods

We reviewed our experience treating snakebites from January 1995 through December 2005. Demographic (eg, age, sex, geographic location) and clinical information (eg, location of bite, species of snake, vital signs, laboratories, treatment, hospital length of stay) were obtained.

Results

Over the last decade, we have treated 114 children with confirmed snakebites. Mean age was 7.3 ± 4.2 years (range, 1-17 years), and snakebites were more common in males (n = 68, 60%). All bites occurred on the extremities, and lower extremity bites were more common (n = 71, 62%). Copperheads inflicted the most bite injuries (n = 65, 57%), followed by rattlesnakes (n = 9, 8%) and cottonmouths (n = 7, 6%). The snake was not identified in 33 (29%) cases. Seven (6%) children were treated with Crotalidae antivenin. Of the children treated with antivenin, only 4 met criteria for treatment, and 1 had an anaphylactic reaction. If compartment syndrome was suspected based on neurovascular examination, compartment pressures were measured. Only 2 (1.8%) patients required fasciotomies. Over the last 2 years, we have stopped empiric treatment with antibiotics and have not observed any infectious complications. Average hospital length of stay was 30 ± 25 hours.

Conclusions

Most children bitten by pit vipers can be managed conservatively with analgesics and elevation of the affected extremity. Treatment with Crotalidae antivenin, antibiotics, and fasciotomy is rarely indicated.     

Predictors of outcome in children and adolescents with rhabdomyosarcoma of the trunk--the St Jude Children's Research Hospital experience

Purpose

The aim of this study was to determine predictors of outcome in childhood truncal rhabdomyosarcoma.

Methods

Retrospective chart review evaluating the impact of demographic features, disease characteristics, and the extent and timing of surgical intervention on outcome was performed.

Results

Thirty-three patients with a median age of 8 years were identified. Most had advanced Intergroup Rhabdomyosarcoma Study group III (n = 13) or group IV (n = 9) disease. Primary site included 20 (61%) chest wall, 6 (21%) paraspinal, 5 (15%) periscapular, and 1 (3%) abdominal wall. Most tumors were embryonal (n = 21), larger than 5 cm (n = 27), and locally invasive (n = 13); 7 had positive nodes.Tumor size, nodal status, and gross total tumor resection (upfront or delayed) were significant predictors of event-free and overall survival. Tumors 5 cm or smaller were amenable to upfront surgical resection (P = .007). In patients with tumors larger than 5 cm, resection at any time was associated with a 10-year overall survival 57% ± 13% compared with 8% ± 5% in those who had no surgery (P = .003). Tumor recurrence was local in 44% of cases, and survival after local recurrence was rare (1 of 8).

Conclusion

Tumor size, nodal status, and gross total resection at any time are significant predictors of outcome in truncal rhabdomyosarcoma. Gross tumor excision should be the goal of surgical intervention in this disease.     

Short- and long-term outcomes after large pulmonary resection for germ cell tumors after bleomycin-combination chemotherapy

Background

Treatment of nonseminomatous germ cell tumors frequently requires bleomycin-combination chemotherapy followed by resection of residual disease. Bleomycin administration however raises concerns with respect to postoperative respiratory complications, particularly for patients undergoing large pulmonary resections. We undertook an institutional review to determine the outcome of large pulmonary resections after bleomycin-combination chemotherapy.

Methods

Between 1981 and 2001, 530 patients presented to our institution for resection of residual intrathoracic disease for either metastatic testicular or primary mediastinal nonseminomatous germ cell tumors. We subsequently reviewed 32 of these patients who required pneumonectomy (n = 19; right = 9, left = 10) or bilobectomy (n = 13) after bleomycin-combination chemotherapy.

Results

There were four operative deaths (13%). All postoperative deaths occurred in patients undergoing right-sided resections (pneumonectomy, n = 2; bilobectomy, n = 2) as a consequence of pulmonary complications. Operative survivors had a pulmonary morbidity of 18%. Fourteen of 20 long-term survivors were found to have a satisfactory performance status at follow-up.

Conclusions

Otherwise young and healthy male nonseminomatous germ cell tumors patients requiring large pulmonary resections after bleomycin-combination chemotherapy appear to be at higher than anticipated risk for pulmonary-related morbidity and mortality. However long-term survivors report an acceptable functional status.     

Primary hepatic sarcomas in children--a single-center experience over 19 years

Background/ Purpose

Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience.

Methods

A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted.

Results

Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years.

Conclusion

This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.     

The utility of diagnostic laparoscopy in the evaluation of anterior abdominal stab wounds

Background

To assess if diagnostic laparoscopy (DL) is superior to nonoperative modes (serial abdominal examination with/without computed axial tomography [CAT] and diagnostic peritoneal lavage) in determining the need for therapeutic laparotomy (TL) after anterior abdominal stab wound (ASW).

Methods

Retrospective review of ASW patients. Patients were divided into group A (DL/exploratory laparotomy) to identify peritoneal violation (PV) and group B (initial nonoperative modes).

Results

Seventy-three patients met inclusion criteria. In group A (n = 38), 29 patients (76%) had PV by DL and underwent exploratory laparotomy. Only 10 (35%) underwent TL (sensitivity for PV = 100%; specificity and positive predictive value of PV in determining need for TL = 29% and 33%, respectively). In group B (n = 35), 7 patients (20%) underwent TL, yielding an improved specificity (96%) and positive predictive value (88%).

Conclusions

We find no role for DL in the evaluation of ASW patients solely to determine PV.     

Indications and Management of Everolimus After Liver Transplantation

Objective

Our aim was to assess our experience with the use and management of everolimus after orthotopic liver transplantation (OLT).

Materials and Methods

Among the 759 patients who underwent transplantation from 1988 to 2008, 25 (3.2%) received immunosuppression with everolimus. Their mean age was 55.6 years. We analyzed indications for use, time between transplantation and introduction of everolimus, as well as its efficacy, side effects, and patient survival.

Results

The indications for everolimus treatment were: extended hepatocellular carcinoma (HCC) in the explanted liver (n = 6; 24%); HCC recurrence during follow-up (n = 4; 16%); de novo tumor (n = 6; 24%); refractory rejection (n = 3; 12%); side effects of calcineurin inhibitors (CNI; n = 3; 12%); and other causes (n = 3; 12%). Mean time between OLT and everolimus treatment was 40 ± 33 months (range, 10 days-178 months). Mean follow-up after conversion was 10 ± 9 months (range, 1.5-25 months). More than half of the patients resolved the event for which the drug was indicated: 75% of patients with refractory rejection; 60% of those with renal insufficiency; and 100% of those converted for neurotoxicity or hepatotoxicity. Two patients with recurrent HCC and 1 with extended HCC died at a mean time of 10.5 months. The 6 cases of de novo tumors were operated and are healthy. Side effects were dyslipidemia in 8 and infection in 2. Five patients (20%) discontinued the drug.

Conclusions

In the early posttransplantation period, everolimus is indicated for refractory rejection or as prophylaxis for recurrence of extended tumors. In any time but especially in the late period, everolimus is indicated for patients with serious side effects due to a CNI or to a de novo tumor.     

Medical tourism in bariatric surgery

Background

The number of Canadians who self-refer for bariatric surgery outside of Canada or to private clinics within Canada remains undefined. The outcomes from this questionable practice have not been evaluated systematically to date.

Methods

We completed a chart review of known cases referred to our center for complications related to medical tourism and bariatric surgery.

Results

We present a series of patients who have experienced complications because of medical tourism for bariatric surgery and required urgent surgical management at a tertiary care center within Canada. Complications have resulted from 3 commonly used procedures: adjustable gastric banding, gastric sleeve resection, and Roux-en-Y gastric bypass.

Conclusions

Because of this review, we propose that a medical tourism approach to the surgical management of obesity—a chronic disease—is inappropriate and raises clear ethical and moral issues.     

Gastric malignancies in breast cancer survivors: Pathology and outcomes

Background

As the number of breast cancer survivors increases, the appearance of second malignancies and unusual metastatic patterns likely also is increasing. In particular, we and others have observed gastric malignancies in breast cancer survivors.

Methods

We reviewed 3 regional hospital system tumor databases, comprising 19,049 analytic breast cancer cases, to determine the number, types, and outcomes of subsequent gastric malignancies.

Results

Twenty-eight patients developed subsequent gastric malignancies, representing .15% of breast cancer survivors; 82% of patients had gastric symptoms. Overall survival for the cohort was 39%. Twenty-four patients (86%) had gastric primaries and 13 died of their second cancers. Four patients had gastric metastases; all had lobular histology in both their primary tumors and metastatic lesions. Five patients had gastrointestinal stromal tumors; all patients underwent resection and currently are alive.

Conclusion

Gastric symptoms in breast cancer survivors may represent malignant lesions, often second primaries. All gastric metastases in our series were of lobular histology.     

Retrospective study of the surgical management and outcome of nonrhabdomyosarcoma soft tissue sarcomas of the groin and axilla in children

Purpose

The incidence of pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) of the groin and axilla is unknown, and the optimal surgical approach to these patients is unclear.

Methods

We conducted a retrospective study of patients treated at St Jude Children's Research Hospital from January 1962 to March 2007 for NRSTSs of the groin and axilla. Demographic variables, tumor pathology, clinical management, and outcome were reviewed.

Results

Of the 300 patients treated for NRSTSs, only 10 had tumors of the axilla or groin (6 of whom had synovial sarcoma). Surgical interventions included wide resection of the tumor (n = 7), marginal resection (n = 1), subtotal resection (n = 1), and biopsy only (n = 1). Six patients underwent lymph node sampling; all were negative for tumor. Short- and long-term surgical complications were rare. Four patients received adjuvant chemotherapy (n = 3) and/or radiotherapy (n = 2). At a median follow-up of 8.5 years, 7 of the 10 were surviving free of disease. Two of these patients died of tumor progression (1 with metastases at diagnosis and 1 with an unresectable tumor at diagnosis), and one patient who was free of NRSTS died of secondary breast carcinoma.

Conclusions

Pediatric NRSTSs of the axilla and groin are rare, but outcomes are similar to those of other patients with NRSTS. Wide local excision of the tumor with preservation of good limb function should be the surgical goal and may be sufficient therapy in some cases.     

Is a vesicoamniotic shunt intrinsically bad? Shunting a normal fetal bladder

Introduction

We previously demonstrated that in utero vesicoamniotic shunting of obstructive uropathy in fetal lambs produces a shrunken noncompliant bladder. We hypothesized that the normal fetal bladder filling and emptying cycle in fetal life is critical to the development of normal bladder function.

Materials and Methods

We placed vesicoamniotic shunts in 4 normal fetal lambs at 74 days' gestation. The fetuses were delivered at term (145 days), and bladder volume and compliance were measured and compared with those measurements in 3 normal term fetuses. The lambs were then killed and the renal tracts and bladders removed submitted to histologic examination.

Results

All shunted lambs survived to term. Three normal control lambs were delivered at term. The mean bladder volume in shunted lambs was 4 ± 2.8 mL (n = 4) compared with 60 ± 17 mL (n = 3) in control lambs (P < .05). Bladders in the shunted lambs had very poor compliance compared with normal lambs' bladders. Histologic examination of the shunted bladders showed increased fibrosis and distortion of the muscle layers compared with control bladders.

Conclusion

Even in the absence of obstruction, preventing normal bladder filling and emptying in fetal life produces fibrotic bladders with poor compliance.     

Outcome in Children Bridged and Nonbridged to Cardiac Transplantation

Background

Heart transplantation (HTx) in children with end-stage heart disease has become an accepted treatment option.

Objective

To evaluate our results of pediatric cardiac transplantation with vs without bridge methods.

Patients and Methods

The study included 31 patients (34 transplantations) younger than 18 years who underwent orthotopic HTx between March 1995 and December 2008. Ten patients were girls, and 21 were boys. Preoperative diagnoses included cardiomyopathy (n = 20), congenital heart disease (n = 7), hypertrophic cardiomyopathy (n = 2), restrictive cardiomyopathy (n = 1), and ischemic cardiomyopathy (n = 1). Mean (SD) ischemia time was 185 (72) minutes. Thirty-day mortality was 6%, and was due to primary graft failure (n = 2). Overall follow-up was 4.36 (3.93) years. Eleven patients underwent bridge techniques before HTx, and 11 patients required perioperative extracorporeal membrane oxygenation or ventricular assist device support.

Results

In the group that received extracorporeal membrane oxygenation, 8 patients (73%) were successfully weaned and discharged with excellent functional class. There were no differences in operative mortality, functional class, survival, rejection, and infection rates between the bridged and nonbridged groups. Overall actuarial 1- and 5-year survival rates were 93% and 83%, respectively. All survivors had good functional class.

Conclusion

Our findings demonstrate satisfactory medium-term outcome of HTx in selected pediatric patients with end-stage heart disease. Using bridge methods in children at high risk can increase the opportunity to receive a donor heart. These bridge methods achieve similar postoperative outcomes.     

Role of intraoperative enteroscopy in the management of obscure gastointestinal bleeding at the time of video-capsule endoscopy

Background

This study aimed at evaluating the role of intraoperative enteroscopy (IOE) for the management of obscure gastrointestinal (GI) bleeding in patients who had been preoperatively explored by video-capsule endoscopy (VCE).

Methods

Eighteen patients who underwent IOE for obscure GI bleeding were prospectively recorded between November 2000 and January 2007. The bleeding site was preoperatively localized by VCE in the small bowel in 15 patients, but the origin of bleeding remained unknown in 3 patients.

Results

In the 3 patients with negative VCE, IOE was normal, but intraoperative conventional endoscopy identified gastric (n = 1) and colonic (n = 2) lesions. Among the 15 patients with VCE positive for small-bowel lesions, laparotomy and IOE yielded localization and treatment (surgical n = 11 and endoscopic n = 2) guidance for 13 of 15 (87%) lesions. At median 19-month follow-up, 3 bleeding recurrences (3 of 15 [20%]) were recorded, resulting in a 73% therapeutic efficacy of IOE.

Conclusions

IOE remains useful for the management of obscure GI bleeding when preoperative VCE is positive for small-bowel lesions that are not reachable by nonoperative enteroscopy. When VCE is negative, new conventional endoscopy should be proposed instead of IOE.     

Protective effect of polyunsaturated phosphatidylcholine pretreatment on stress ulcer formation in rats

Purpose

The aim of this study was to investigate whether polyunsaturated phosphatidylcholine. (PPC) pretreatment has any protective effect on gastric mucosal damage induced by cold-restraint stress (CRS) in rats.

Methods

Forty swiss albino rats were divided into 3 groups. Group 1 (n = 10) was control, group 2 (n = 15) was stress ulcer, and group 3 (n = 15) was PPC-treated rats with stress ulcer. Stress ulcer was induced by the cold-restraint method for 4 hours at 4°C after a starvation period of 72 hours. In the group 3 rats, PPC treatment was started 10 days before stress at a dose of 100 mg/d by oral route. Rats were terminated, stomachs were excised. Macroscopic ulcer index (UI), gastric tissue malondialdehyde (MDA) and superoxide dismutase (SOD) activities, plasma total nitrite, and erythrocyte catalase (CAT) concentrations were assayed.

Results

Histopathologic examination showed a stress ulcer index of 0.12 ± 0.19 mm in the treatment group and 23.6 ± 8.97 mm in the stress ulcer group (P < .001). Tissue MDA and SOD concentrations were higher in the stress ulcer group than in the treatment group, the differences were statistically significant (P < .001). Plasma NO₃⁻+ NO₂⁻ levels were higher (P< .005) and CAT levels were lower (P < .001) in the nontreatment group. There were no significant differences with respect to Ul, MDA, and SOD levels among the control and treatment groups (P > .05).

Conclusions

These results suggest that pretreating rats with PPC inhibits cold-restraint stress-induced gastric mucosal injury and might be useful in preventing stress-induced stomach ulcers.     

Dynamic tracheal occlusion improves lung morphometrics and function in the fetal lamb model of congenital diaphragmatic hernia

Background

Congenital diaphragmatic hernia (CDH) is associated with significant neonatal morbidity and mortality. Although prenatal complete tracheal occlusion (cTO) causes hypoplastic CDH lungs to enlarge, improved lung function has not been demonstrated. Furthermore, cTO interferes with the dynamic pressure change and fluid flow associated with fetal breathing.

Purpose

The purpose of the study was to assess a novel dynamic tracheal occlusion (dTO) device that preserves pressure changes and fluid flow.

Methods

In this pilot study, CDH was created in fetal lambs at 65 days of gestational age (GA). At 110 days GA, a cTO device (n = 3) or a dTO device (n = 4) was placed in the fetal trachea. At 135 days GA, lambs were delivered and resuscitated. Unoperated lamb co-twins (n = 5), sham thoracotomy lambs (n = 2), and untreated CDH lambs (n = 3) served as controls.

Results

Tracheal opening pressure, lung volume, lung fluid total protein, and phospholipid were significantly higher in the cTO group than in the dTO and unoperated control groups. Maximal oxygenation and lung compliance were significantly lower in the cTO group when compared with the unoperated control and dTO groups.

Conclusion

Preliminary results suggest that in the fetal lamb CDH model, dTO restores normal lung morphometrics and function, whereas cTO leads to enlarged but less functional lungs.     

Fifty-three-year experience with pediatric umbilical hernia repairs

Purpose

The aim of this study was to evaluate the long-term surgical and patient-reported outcomes of pediatric umbilical hernia (UH) repairs.

Methods

A retrospective review of all children (<18 years old) who underwent UH repair at Mayo Clinic-Rochester in the last half century was done. Follow-up was obtained by mailed survey.

Results

From 1956 to 2009, 489 children (boys, 251; girls, 238) underwent a primary UH repair. The mean age was 3.9 years (range, 0.01-17.8 years). Complicated UHs that required emergent repair (n = 34, or 7%) included recurrent incarceration (22), enteric fistula (7), strangulation (4), and evisceration (1). Mean UH size was 1.3 cm (range, 0.2-7.0 cm), varying by operative indication (1.0 cm emergent vs 1.5 cm elective repairs, P = .008) and decade of repair (2.2 cm, 1950s-60s vs 1.3 cm, 1990s-2000s; P = .001). Postoperative morbidity (2%) consisted of superficial wound infection (7), hematoma (3), and seroma (1). With a 66% survey response rate and mean follow-up of 13.0 years (range, 0-53.8 years), 8 (2%) patients experienced a recurrence. Most patients reported satisfaction (90%) with the cosmetic appearance of their umbilicus and are pain free (96%).

Conclusion

Pediatric UH repairs have low morbidity and recurrence rates. Most patients are satisfied and pain free. Importantly, complicated UHs were more likely to be associated with smaller defects; therefore, parental counseling for signs of incarceration is recommended even in small defects.     

Control of presacral venous bleeding during rectal surgery

Background

Presacral venous hemorrhage is an uncommon but potentially life-threatening complication of rectal surgery. It is difficult to control presacral venous hemorrhage with conventional hemostatic measures and several alternative methods for hemostasis have been proposed. We described our experience of using the combination of a hemostatic matrix and an absorbable hemostat as an alternative method of hemostasis.

Methods

From September 2007 to March 2009, 83 patients underwent rectal surgery for cancer, ulcerative colitis, or familial adenomatous polyposis. Three patients (3.6%) had severe presacral hemorrhage, which was controlled by the combined use of a hemostatic matrix (FloSeal; Baxter, USA) and an absorbable hemostat (Surgicel Fibrillar; Ethicon, USA).

Results

Intraoperative blood transfusion was required in 1 patient. Postoperative blood loss was minimal and drain was removed on day 4 in all 3 patients.

Conclusions

The use of synthetic hemostatic agents is an effective and simple way to arrest presacral bleeding where conventional methods fail.     

Malignancy development in lung transplant patients

Introduction

The most common neoplasias among transplant patients are skin cancers and lymphoproliferative disorders.

Objective

To characterize lung transplanted recipients who developed malignancies.

Methods

A retrospective analysis of clinical records of our patients.

Results

Seven patients developed malignancies: skin cancer (n = 5; 71%), and adenocarcinomas of prostatic, gastric, and lung (n = 1 each). One patient developed two hematologic malignancies: T-cell lymphoma and multiple myeloma. Among five patients who died (71%), 3 were due to advanced neoplasia. The mean presentation time was 4.3 years. Skin cancers were resected. The patient with lung adenocarcinoma developed pleural involvement and died. The patient with T-cell lymphoma was treated, but succumbed afterward due to multiple myeloma. The patient with gastric adenocarcinoma died at 3 months after the diagnosis, and the patient with prostate cancer underwent surgery without disease recurrence.

Conclusion

Malignancies are a late complication of transplant recipients that require a prompt diagnosis and treatment to improve outcomes.     

Supplementation of retrobulbar block with clonidine in vitreoretinal surgery: effect on postoperative pain

Study Objective

To evaluate the effect of clonidine when added to local anesthetics on duration of postoperative analgesia during retrobulbar block.

Design

Prospective, randomized controlled trial.

Setting

Operating room and Postanesthesia Care Unit of a university-affiliated hospital.

Subjects

80 ASA physical status 1, 2, and 3 patients undergoing vitreoretinal surgery with or without scleral buckling.

Interventions

Patients in the control group (n = 40) received a retrobulbar block with 4.5 mL of lidocaine-bupivacaine and 0.5 mL of saline. Clonidine group patients (n = 40) received 4.5 mL of lidocaine-bupivacaine and 0.5 μg/kg of clonidine in a 0.5 mL volume.

Measurements

The time to first analgesic request, frequency of postoperative pain, and number of postoperative analgesic requests per patient were assessed.

Main Results

37 patients in the control group (92.5%) versus 24 patients (60%) in the clonidine group reported pain postoperatively (P = 0.001), with a shorter time to first analgesic request noted in the control group (4.9 ± 3 vs 11.9 ± 5.3 hrs; P < 0.001). The median number of postoperative analgesic requests per patient during the first 24 hours was higher in the control group than the clonidine group [2 (0-3) vs. 1 (0-3); P < 0.001].

Conclusions

The addition of clonidine 0.5 μg/kg to the local anesthetics of a retrobulbar block for vitreoretinal surgery decreases the frequency of postoperative pain and prolongs the time of analgesia.