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Background Congenital paraesophageal hernia (CPEH) is a rare diaphragmatic anomaly for which repair has primarily been described by laparotomy, although, more recent case series describe laparoscopic repair. In reports with over five patients, the predominant approach has been with laparotomy. The purpose of our study was to review our recent institutional experience and results with exclusively laparoscopic repair of CPEH in infants and children. Methods An IRB approved retrospective review of all patients with CPEH who underwent laparoscopic treatment at a tertiary children's hospital from 2010 to 2017 was performed. We included only those patients from our own institution with primary CPEH, or CPEH with prior repair (s) at other centers, with recurrence presenting for operation. Data including demographics, diagnostic studies, operative details, complications, outcomes, and follow up were analyzed. Age at diagnosis was 1 day to 25 years of age (mean 2.5 years). Results A total 28 patients underwent 30 operations to treat CPEH. All operations were completed laparoscopically with no conversions to open. There were 6 Type II, 16 Type III, and 6 Type IV CPEH patients. Seventeen patients were less than one year of age (61%). Weight at time of repair was 10.3 kg (1.2–44 kg). Twelve patients were less than 5 kg (43%), eight patients (28.5%) were less than 10 kg, and 8 were more than 10 kg (28.5%). Operative time averaged 125 min (range 61–247 min). Three patients underwent initial CPEH repair (s) (open: 2 and laparoscopic: 1) at other institutions before laparoscopic revision was performed at our hospital (11%). Crural repair was performed in all patients, fundoplication in 26 (93%) and concomitant gastrostomy was performed in 14 patients (50%). Complications included two patients with recurrent hiatal hernias, which were redone laparoscopically (2/28 or 7% recurrence) and 1 capnothorax requiring pigtail drainage postoperatively. There were no deaths, no requirement for esophageal dilations, or esophageal lengthening. One patient required laparoscopic gastrostomy six weeks post initial repair for failure to thrive. Follow-up ranged from 4 months to 8 years (average 36 months). Conclusion Congenital paraesophageal hernia in infants and children is uncommon. Based on our experience, the laparoscopic approach to repair is feasible, even for neonates, with excellent results, acceptably low recurrence rate, and may even be considered for revisional operations. Study type Clinical research paper. Level of evidence Type IV.

Purpose Primary lymphedema presenting in adolescence is known as lymphedema praecox. Older children presenting with leg swelling are often subjected to a myriad of diagnostic tests. The purpose of this study is to review a large-cohort of patients with lymphedema praecox to determine the fiscal impact of diagnostic testing on these patients. Methods A 13-year review was performed of patients with lymphedema praecox. Information was obtained on demographic parameters, diagnostic studies performed, and clinical outcomes. Results Forty-nine patients were identified. The median age was 14 (range: 7–21) years. Participants were predominantly female (n = 40, 81.6%). 19 patients had bilateral disease and 30 had unilateral disease. The diagnosis was made on clinical exam only in 14 patients. 35 patients had imaging which consisted of plain X rays, Doppler ultrasound (DUS), lymphoscintigraphy (LSG) or MRI as the sole imaging study (n = 28) or in combination with others (n = 7). The charges for plain X-rays, DUS, LSG, and MRI with contrast were $335, $1715, $1269, and $6006 respectively. Conclusion We believe that in the adolescent female with physical findings consistent with lymphedema praecox, diagnostic imaging should be limited to a Doppler ultrasound to rule out a secondary cause of the swelling. Level of Evidence IV Type of Evidence Case series with no comparison group.

The surgical management of retroperitoneal tumors extending into the inferior vena cava (IVC) can be challenging. Although Wilms' tumor is the most common retroperitoneal tumor extending into the IVC, one must approach these tumors systematically as other diagnoses are possible. We present 4 consecutive cases of retroperitoneal tumors with IVC extension as a basis for a management strategy in approaching these patients. Despite similar presentations, these cases illustrate the nuances in surgical management and need for multidisciplinary care with the pediatric oncologists, pediatric surgeons, and pediatric cardiac surgeons.

Background/Purpose: The aim of this study was to determine if the presence of "blush" (an indication of active bleeding) on abdominal CT in children with blunt liver injury adversely affected their clinical outcome as has been reported in adults. Methods: The authors reviewed the records of 105 children ages 1 to 16 years with blunt liver injury seen on admission IV contrast CT seen over a 6-year period. Demographic characteristics measured were age, mechanism of injury, and injury severity score (ISS). Clinical outcomes included ICU stay, hospital length of stay (LOS), transfusion requirement (milliliters per kilogram), operations performed, and mortality rate. CT scans were evaluated retrospectively by a radiologist blinded to prior reports, for a "blush" and grade of liver injury. No patient underwent arterial embolization. The authors eliminated children with grade I-II injuries (30 patients), because only one had a blush, and analyzed the 75 patients with severe liver injuries (grades III-V). Those patients without a blush (n = 53) seen on CT were the control group, whereas patients with a blush (n = 22) were the study group. Data were analyzed using the Fisher's Exact and Mann-Whitney U test. The level of significance was set at.05. Results: Patients with a blush had a significantly larger transfusion requirement (17.3 ± 30.5 mL/kg v 5.0 ± 10.9 mL/kg; P =.02) and mortality rate (23% v 4%; P =.02), but the ISS also was significantly greater (25.8 ± 14.5 v 17.5 ± 12.2; P =.019). All other data were similar between the 2 groups. Conclusions: Children with a blush seen on abdominal CT after blunt liver injury have higher transfusion requirements and greater risk of mortality than those without blush. Mortality is primarily related to the severity of their other injuries. J Pediatr Surg 38:363-366. Copyright 2003, Elsevier Science (USA). All rights reserved.

Blunt trauma to the abdomen resulting in aortic injury is rare in children with only a few case reports in the past 40 years. We describe the diagnosis and management of a 2-year-old boy who survived an aortic bifurcation rupture after blunt trauma.

A patient affected by blue rubber-bleb nevus syndrome had chronic gastrointestinal bleeding requiring weekly blood transfusions. Despite multiple surgical and endoscopic procedures to treat the venous malformations, the patient continued to bleed primarily from lesions in the small bowel. Therefore, this patient was treated with octreotide, a somatostatin analog known to decrease splanchnic blood flow and that is used for acute and chronic gastrointestinal bleeding.Octreotide therapy, 5.7 microg/kg subcutaneously twice daily, was initiated, and the patient was followed up clinically. Complete blood counts, blood glucose concentration, pancreatic enzyme concentration, liver function tests, and growth hormone concentration were monitored during treatments.During the 4 weeks after initiation of octreotide therapy, hemoglobin concentration was maintained without the need for transfusions. Octreotide decreased the patient's monthly need for blood transfusion from 52 +/- 7 mL. kg-1. mo-1 of packed red blood cells to 23 +/- 7 mL. kg-1. mo-1. She had no detectable side effects or growth inhibition. Other medical interventions including -epsilonaminocaproic acid, nadolol, and total parenteral nutrition with bowel rest were not as effective as octreotide alone.Octreotide decreased the patient's need for blood transfusions. Possible mechanisms include altering blood flow to the gastrointestinal tract and direct effects on the venous malformations.

Background/Purpose: Fecal incontinence impedes social and psychological development in children. Conventional bowel management with rectal enemas, medicaton, and biofeedback frequently will provide intervals free of fecal soiling sufficient for children to be socially continent. Methods: This report details the improvement achieved by antegrade irrigation of the colon when conventional bowel management programs have failed to provide satisfactory intervals free of fecal soiling. Twenty-three children had conduits performed to administer antegrade continent enemas in the colon from December 1993 to May 1997. Results: Twenty of the children were available for evaluation. One child was lost to follow-up, and two were noncompliant with the irrigation program. Fourteen of the 20 children are out of diapers. Four of the six wearing diapers, do so for urinary, not fecal incontinence. Two patients (10%) still require diapers for fecal incontinence. Parents were pleased with the efficacy of antegrade irrigation and the decrease in family stress with the elimination of fecal soiling. Conclusions: Ninety percent of the children available for follow-up benefited from antegrade irrigations of the colon. The antegrade irrigations were an improvement over conventional bowel management programs because a predictable interval free of soiling could be established. This allowed families to plan activities and improved the self-esteem of their children.

To compare laparoscopic and traditional open splenectomy in children with nonmalignant hematologic disorders.Retrospective review of 36 consecutive nonrandomized splenectomies (16 laparoscopic and 20 open) performed for hematologic disorders at a single pediatric institution during the past 3 years. The two-sided Mann-Whitney U test for non-parametric variables was used for statistical analysis.An open procedure was performed on 20 patients (mean age, 9.7 years), five of whom had a concomitant cholecystectomy. A laparoscopic splenectomy was performed on 16 children (mean age, 10.3 years), seven of whom had a concomitant cholecystectomy. The mean anesthesia and operative times were longer in the laparoscopic than in the open group (p0.001). However, the mean number of hours of postoperative analgesia was less in the laparoscopic group (p0.005). Patients who had laparoscopic splenectomy were also discharged home earlier (p0.01) and resumed a regular diet sooner. Mean operating room charges were higher in the laparoscopic group (p0.001), but total hospitalization costs were not significantly different. Postoperative complication rates were similar. The hematologic response was comparable.laparoscopic splenectomy is feasible and safe in children with hematologic disorders. Although it currently requires more operative time than the open approach, it is superior with regard to duration of postoperative analgesia, duration of hospital stay, and recovery of bowel function.

Prolonged preoperative fasting can be associated with adverse outcomes, particularly in children. Our aims were to assess the time pediatric patients fasted prior to surgical or radiologic procedures and evaluate whether fasting (NPO) orders complied with national guidelines. We measured NPO start time, time of last intake, and time test or surgery was scheduled, took place, or was cancelled in 219 pediatric patients. Findings demonstrate that pediatric patients experienced prolonged fasting before procedures and that the majority of NPO orders were non-compliant with national guidelines. We have developed strategies to reduce fasting times and ensure compliance with recommended national fasting standards.

Our laboratory has reported previously spontaneous acceptance of class II-matched, single haplotype (but not 2 haplotype), class I-mismatched renal allografts in miniature swine. All class II-mismatched animals rejected acutely regardless of class I matching. We have also demonstrated recently that a short course of high dose (10 mg/kg/day for 12 days) CsA uniformly induces donor-specific tolerance to 2-haplotype, class I-mismatched renal allografts. The survival of 2-haplotype, fully MHC mismatched renal allografts was prolonged by the same treatment, but tolerance was not induced, as all animals rejected eventually. We have now tested this short course of immunosuppressive therapy for its effect on renal allografts mismatched selectively for 2 haplotypes at class II. We have observed long-term graft survival in 5 of 7 animals under these conditions. Each of the 5 acceptor animals was demonstrated to be specifically tolerant by its response either to donor-matched skin grafts or to a second donor-matched kidney transplant without further immunosuppression. These data suggest the existence of a common pathway for induction of specific transplantation tolerance to MHC antigens when these antigens are recognized on vascular endothelium under conditions of altered cytokine production. They also suggest that tolerance induction under these conditions requires matching for either class I or class II antigens, which may have implications for the mechanism by which peripheral tolerance is induced, as well as practical implications for the extension of these results to potential clinical practice.

In miniature swine, one-haplotype class I disparate renal allografts are accepted without exogenous immunosuppression by approximately 35% of recipients. Alternatively, transplants bearing a two-haplotype class I mismatch are always rejected acutely. However, long-term acceptance in the latter animals can be achieved uniformly with a 12-day course of cyclosporine. In vitro studies of recipient cell-mediated lymphocytotoxicity responses have shown donor-specific cytotoxic T lymphocyte clones in tolerant animals, suggesting that tolerance may be a local phenomenon or a central phenomenon activated in the milieu of the graft. Six animals were retransplanted with kidneys MHC-matched to their original allograft to determine whether (1) tolerance is a central phenomenon; (2) host tolerance can be broken with a fresh challenge of donor antigen and antigen-presenting cells; and (3) graft adaptation is required for maintenance of tolerance. Four of the retransplanted animals had been spontaneous acceptors of one-haplotype class I-disparate grafts and two had been rendered tolerant to two-haplotype class I-mismatched kidneys with CsA induction. All six explanted allografts showed no histological evidence of rejection and all six retransplants were accepted without exogenous immunosuppression. These findings suggest that in miniature swine tolerance of class I-disparate kidneys is a stable, centrally mediated phenomenon that cannot be broken with a challenge of fresh donor antigen and donor-type APCs. Furthermore, successful retransplantation without immunosuppression in animals receiving CsA induction therapy for their first transplant suggests that graft adaptation is not necessary for the maintenance of tolerance.

Studies of the myeloablative regimens capable of permitting successful BMT across MHC barriers in miniature swine have been performed. To minimize graft-versus-host disease (GVHD), engraftment was studied in the F1-->P combination (i.e., MHC homozygous ["parental"] swine receiving bone marrow from one-haplotype matched MHC heterozygous ["F1"] donors). Animals given total body irradiation (TBI) up to 1100 cGy, 10 cGy/min, in a single dose failed to engraft. Increasing the dose rate led to unacceptable extramedullary toxicity without improving engraftment. Eleven different fractionated TBI regimens were tested in this F1-->parent model. At all of the dose rates tested, a total dose of less than 1000 cGy was insufficient for engraftment, and a total dose of 1400 cGy led to unacceptable toxicity. Between these extremes, a window was defined in which engraftment could be obtained without unacceptable extramedullary toxicity utilizing 2 equally divided fractions of TBI delivered 24 hr apart. The addition of 50 mg/kg cyclophosphamide i.v. to fractionated TBI (1150 cGy total dose [500 + 650]) also permitted engraftment, with decreased incidence of interstitial pneumonitis as compared to fractionated TBI (1300 cGy total dose [650 x 2]). Both of these regimens were also confirmed to permit engraftment between heterozygous donors and recipients sharing a single common haplotype ("F1-->F1"). The regimen of 1300 cGy (650 x 2) also permitted engraftment in completely MHC mismatched BMT, but with subsequent death from GVHD. These studies of the myeloablative regimens permitting engraftment across defined MHC barriers in miniature swine provide a basis for further studies of allogenic BMT and GVHD in this large animal preclinical model.

Questions persist about the management of postoperative chylothorax in infants and children. Our experience with postoperative chylothorax over the most recent decade (1980 to 1990) has been reviewed. The type and amount of drainage, data from cardiac catheterization and echocardiography, operative decisions and details, and eventual outcomes have been cataloged. All patients were initially treated with total gut rest, with operation reserved for unabated drainage. Chylothorax developed postoperatively in 15 infants and 11 children (18 with a cardiac procedure and 8 with a noncardiac procedure). The average age was 3.1 years. Spontaneous cessation and cure occurred in 19 (73.1%) of these 26 patients, with an average drainage duration of 11.9 days (range, 4 to 30 days). Those for whom operation was chosen drained preoperatively for an average of 29.2 days (range, 25 to 40 days). There were no deaths in either group. Complications were lymphopenia (2 patients) and fungal sepsis (1 patient). The amount of drainage per day was not significantly different between patients treated operatively and those treated nonoperatively. Failure of nonoperative management was associated with venous hypertension from increased right-sided cardiac pressures or central venous thrombosis (p < 0.05, Fisher's exact test). Presumably this increased pressure is transmitted to the lymphatic system. These patients should be identified early and considered for thoracic duct suture or pleuroperitoneal shunting.

Previous studies in miniature swine have suggested that the mechanism underlying the spontaneous development of tolerance in one third of one-haplotype class I disparate renal allografts (i.e., ag----ad) involves a relative T cell help deficit at the time of first exposure to antigen. If this hypothesis were correct, then one might expect the administration of an immunosuppressive agent capable of inhibiting lymphokine production during this period to lead to the induction of tolerance to class I MHC antigens in two-haplotype class I mismatched renal allografts (i.e., gg----dd), which are otherwise uniformly and acutely rejected. This hypothesis was tested in eight two-haplotype class I disparate, class II matched donor-recipient pairs, in which recipients were treated with cyclosporine 10 mg/kg, i.v. q.d. for 12 days. This protocol led to the induction of long-term (greater than 100 days) specific tolerance in 100% of recipients, as compared with control animals that rejected grafts in 13.7 +/- 0.9 days (P less than 0.0001). The specificity of tolerance was assessed both in vivo with subsequent skin grafts and in vitro by mixed lymphocyte response (MLR) and cell-mediated lymphocytotoxicity (CML). Survival of donor-specific skin grafts was prolonged compared with skin grafts bearing third-party class I antigens (19.5 +/- 2.0 versus 11.5 +/- 2.0 days, n = 4, P less than 0.05). Tolerant recipients had markedly diminished or absent anti-donor MLR and CML responses, but maintained normal reactivity to third party. Four of eight CsA-treated recipients showed detectable levels of anti-donor IgM, while none demonstrated the presence of anti-donor IgG, which was found in all rejecting controls.

Recent data in mice have shown that early administration of recombinant human interleukin-2 (rIL-2) provides significant protection from lethal graft-versus-host disease. Because of the potential clinical importance of these findings, it will be important to assess the effectiveness of this therapy in a large animal preclinical bone marrow transplantation model. We report here our initial studies of the in vitro and in vivo effects of rIL-2 in miniature swine. In vitro 4-day cultures of pig peripheral blood lymphocytes (PBL) in complete medium containing rIL-2 at 1,000 U/ml resulted in optimal proliferation and generation of lymphokine-activated killer (LAK) cells. A pig-mouse hybridoma cell line was found to be highly sensitive as a LAK cell target. Two naive pigs received 20,000 U/kg and 2 pigs received 100,000 U/kg of rIL-2 intravenously twice a day for 4 days. No clinical symptoms were seen during or after administration at the lower dose while both high dose-treated animals showed generalized erythema from days 2 to 4, and one showed mild diarrhea during this period. The disappearance of IL-2 activity from the serum showed two components: (1) an initial fast component with a half-time of approximately 10 min and (2) a slow component with a half-time of approximately 60 min. LAK cell precursors disappeared from the peripheral circulation by 6 min after rIL-2 administration and began to recover by 6 h in the low dose recipients and only after 12 h in the high dose recipients.(ABSTRACT TRUNCATED AT 250 WORDS)

Major histocompatibility complex class II matching is of overwhelming importance for achieving tolerance to kidney transplants (KTX) in miniature swine. When class II antigens are matched, long-term specific tolerance across complete MHC class I antigen barriers can uniformly be induced by a 12-day perioperative course of cyclosporine. This same regimen is ineffective in fully MHC-mismatched combinations. We hypothesized that initial induction of tolerance to kidney donor class II antigens by bone marrow transplantation might allow tolerance to be induced to a subsequent fully allogeneic KTX in combination with CsA therapy. We report here the results of such fully allogeneic KTX performed in 4 recipients of prior single-haplotype class II-mismatched BMT. All animals received KTX from donors class II matched to the BMT donor and received a 12-day course of intravenous CsA (10 mg/kg/day). All four animals have maintained normal serum creatinine values (less than 2.0 mg/dl) for greater than 200 days posttransplant. Specific hyporesponsiveness to both BMT and KTX donor-type MHC antigens was found in mixed lymphocyte culture and cell-mediated lympholysis assays. Compared with third-party grafts, significantly prolonged survival of BMT donor-specific (P = 0.031) and KTX donor-specific (P = 0.031) skin grafts was observed. These results demonstrate that induction of tolerance to class II antigens by BMT allows a short course of CsA to induce specific tolerance to fully allogeneic renal allografts.

Previous studies from our laboratory have shown that permanent lymphohematopoietic chimerism can be induced in MHC-disparate miniature swine by bone marrow transplantation after lethal total-body irradiation. The purpose of the present study was to determine in this large animal model whether such chimerism would lead to permanent tolerance to a vascularized allograft without a requirement for exogenous immunosuppression. Eight miniature swine that had received MHC-mismatched BMT more than five months earlier underwent kidney transplantation (KTx) from a donor MHC matched (n = 5) or MHC mismatched (n = 3) with the BMT donor. All animals had regained in vitro responsiveness to third-party MHC antigens, as measured by mixed lymphocyte reaction (MLR), before KTx but remained nonresponsive to MHC antigens of the BMT donor and self. All three animals that received KTx mismatched for BMT donor MHC rejected promptly (mean survival time 7.0 days). Of the five animals that received KTx matched for BMT donor MHC, four showed no evidence of rejection and have functioning KTx greater than 200 days after KTx. The fifth animal had excellent renal function for 60 days but then developed a slowly rising BUN and serum creatinine, and died 75 days after KTx. The course of this animal's rejection is consistent with that previously described for rejection due to minor antigen disparities. The difference in survival of KTx matched or mismatched for the MHC of the BMT donor was statistically significant (P = 0.0062). The survival of KTx matched for the MHC of the BMT donor was significantly different from that of control animals without BMT receiving KTx mismatched for MHC (P = 0.0018). We therefore conclude that BMT is an effective means for induction of tolerance to an MHC mismatched KTx in this large animal model.

Parathyroid carcinoma is a rare cause of hypercalcemia in children but should be considered in a child presenting with an extremely elevated serum calcium level. The authors report the fifth case of parathyroid carcinoma in a child less than 16 years of age.

Solid pseudopapillary tumors of the pancreas (SPTP) are very rare, and an SPTP arising in a pancreatic rest has been reported only 4 times previously and never in association with the jejunum. We report this unusual case of a 16 year old girl who presented with 4 days of intermittent, crampy abdominal pain and was found to have an SPTP arising in a pancreatic rest of the jejunum.

Previous studies utilizing a recombinant MHC haplo-type in our partially inbred miniature swine herd have demonstrated that some recipients matched only for SLA class II show long-term acceptance of renal allografts without exogenous immunosuppression. Such animals have been shown to develop systemic tolerance as evidenced by prolonged rejection times of subsequent donor-specific, but not third-party, skin grafts. In the present studies in vitro cellular responses of long-term tolerant animals and of 7 animals studied sequentially are presented. Long-term tolerant animals demonstrated responses consistent with the absence of the class I reactive helper populations normally present in naive controls. Animals studied sequentially segregated into two groups based on cellular reactivity and survival. All animals showed complete loss of antidonor class I cell-mediated lymphocytolytic (CML) reactivity by postoperative day 10. However, animals surviving less than 20 days maintained CML reactivity to donor class I plus third-party class II in the posttransplant period, while animals surviving greater than 40 days lost such reactivity. Addition of exogenous interleukin 2 could not reverse this loss. These studies suggest that tolerance induction to a renal allograft across a class I only difference involves effects on both helper and killer class I reactive cell populations.

Trauma is the leading cause of death for children over 1 year of age. This study was undertaken to identify the patterns of injury among children admitted to a regional pediatric trauma center. During a 34-month period, 3,472 injured children were consecutively admitted to a regional pediatric trauma center. Data were collected on medical, etiological, and financial aspects of injury. Eight subgroups were defined by mechanism of injury: motor-vehicle crash occupants, pedestrian and cycle injuries, falls, child abuse, gunshot and stab wounds, burns, poisonings, and foreign body ingestions or aspirations. Analysis of variance, Duncan's multiple range test, and contingency table analysis were used to determine differences among subgroups of children. Blunt and penetrating trauma accounted for 64.3% of all admissions. The mean age of injured children was 5.5 years; 64% of the children were boys. Sixty-seven percent of the children were admitted directly from the scene of injury. One-way analysis of variance yielded significant differences in mean age, mean hospital length of stay (LOS), mean intensive care LOS, mean trauma score, mean injury severity, and mean hospital charges by mechanism of injury (P less than .01). The overall mortality rate was 2.4%. Child abuse, gunshot/stab wounds, and drowning had the highest mortality rates, but injuries to motor-vehicle crash occupants and pedestrians accounted for the greatest number of deaths.

We present a case of lap-belt motor vehicle injury in a 7-year-old male who was admitted with abdominal ecchymoses and pain with associated aortic intimal flap, bowel injury, hemoperitoneum, and retroperitoneal hematoma at initial imaging with CT. Most of these findings were confirmed at subsequent laparotomy, and the patient underwent operative repair of bowel injuries. His aortic intimal flap was followed with US and color Doppler imaging during which time he was treated conservatively until there was sonographic evidence of intimal healing. This patient illustrates a multimodality approach to imaging and conservative management.

Importance While propranolol is touted as superior to prednisolone for treating infantile hemangiomas (IH), a randomized clinical trial (RCT) comparing the outcome and tolerability of these medications for symptomatic, proliferating IH has not been reported. Objectives To determine if oral propranolol is more efficacious and better tolerated than prednisolone in treating symptomatic, proliferating IH and to determine the feasibility of conducting a multi-institutional, RCT comparing efficacy and tolerability of both medications. Design, Setting, and Participants Phase 2, investigator-blinded, multi-institutional RCT conducted in 3 academic vascular anomalies clinics on 19 of 44 eligible infants aged between 2 weeks and 6 months. All participating patients had symptomatic proliferating IH treated between September 1, 2010, and August 1, 2012. Interventions Treatment with oral propranolol vs prednisolone (2.0 mg/kg/d) until halted owing to toxic effects or clinical response. Main Outcomes and Measures Primary outcome was change in IH size after 4 months of therapy. Secondary outcomes were response rate and frequency and severity of adverse events (AEs). Results The primary outcome showed no difference in lesion size or affected skin area after 4 months of therapy: 41% and 1.32 mm 2 for prednisolone vs 64% and 0.55 mm 2 for propranolol ( P = .12 for lesion size, and P = .56 for affected skin area). Longitudinal analyses showed a faster response in total lesion outer dimension with prednisolone ( P = .03), but this advantage over time was not noted when central clearing and outer dimension were included in the analysis ( P = .91). The overall frequency of AEs was similar (44 for prednisolone vs 32 for propranolol) ( P = .84), but prednisolone-treated participants had more grade 3 severe AEs (11 vs 1) ( P = .01), particularly growth retardation resulting in size and weight below the fifth percentile. Early study withdrawal owing to AEs occurred in 6 (75%) of 8 patients in the prednisolone group but 0 of 11 propranolol-treated participants. The mean duration of therapy was shorter for prednisolone (141 vs 265 days), reflecting the higher rate of early withdrawals. Conclusions and Relevance Both medications show similar efficacy for reducing the area of symptomatic, proliferating IH. Although prednisolone showed a faster response rate, propranolol was better tolerated with significantly fewer severe AEs. Propranolol should be the first line of therapy for symptomatic IH unless contraindicated or unless future studies demonstrate severe AEs from propranolol. Recruiting participants for a phase 3 RCT would be difficult owing to safety profiles measured here and emerging trends favoring propranolol. Trial Registration clinicaltrials.gov Identifier:NCT00967226

Background/Purpose: The preferred treatment for children with major pancreatic ductal injury remains controversial. This report compares our results using early operation with previously reported series using both operative and nonoperative management. Methods: This is a retrospective analysis of all children with pancreatic transection identified at Children's Medical Center of Dallas, Texas, from 1995 through 1999. Results: There were 11 children with pancreatic transection. There was a delay of 2.3 days before presentation to a hospital in 6. Transection was diagnosed within 12 hours of presentation in 5 children. In the other 6 there was a mean delay of 36 hours. Nine children underwent operation within 72 hours of injury. Two late presenters initially were treated nonoperatively, and both developed a pseudocyst. The length of hospital stay in patients undergoing early operation averaged 11 days. Early postoperative morbidity occurred in 4 children and late morbidity in 1. Conclusions: Major pancreatic injuries are uncommon in children, and the diagnosis often is delayed. A high index of suspicion and repeated computed tomography scans should lead to earlier diagnosis. When compared with nonoperative management, early pancreatic resection more expeditiously returns the child to good health and lessens the inconvenience and emotional stress associated with prolonged hospitalization. J Pediatr Surg 36:341-344. Copyright © 2001 by W.B. Saunders Company.

Video-assisted thoracoscopic surgery (VATS) has a recognized role in treatment of empyema thoracis. The purpose of this report is to show the value of initial VATS as the primary treatment of parapneumonic collections.A retrospective review was done of 139 children who required surgical consultation for parapneumonic collections between January 1992 and July 1998. Management options were (M1) thoracentesis, chest tube drainage, or fibrinolytic therapy and delayed thoracotomy for unresolved collections; (M2) thoracentesis, chest tube drainage, fibrinolytic therapy with delayed VATS if the child remained ill; or (M3) primary VATS. Comparative data included age, duration of prehospital illness, oxygen requirements, white blood cell count, bacterial culture results, number of procedures performed per patient, duration of chest tube drainage, complications, and length of stay. Kruskal-Wallis 1-way analysis was used, with significance at P less than .05.A total of 60 children were treated by M1, 38 by M2, and 41 by M3. Age, duration of prehospital illness, oxygen requirements, white blood cell count, bacterial culture results, and complication rates were comparable. The median length of stay was 12 days for M1, 11 days for M2, and 7 days for M3, with M3 significantly shorter at P.001. The number of procedures was a median of 2 in M1, 2 in M2, and 1 in M3, with M3 significantly fewer at P.001. Duration of chest tube drainage was a median 5 days for M1 and 3 days for M2 and M3, with M1 significantly longer at P.001. There were 9 thoracotomies in the M1 group, 3 in the M2 group, and none in the M3 group. One child in M3 required a second VATS.Primary VATS has significantly decreased the number of procedures, duration of chest tube drainage and length of stay for children with parapneumonic effusions. Primary VATS appears to be of value in management of bacterial pneumonia with effusion.

Since 1981, eight children have been treated at this hospital for hypertension due to bilateral renal artery stenosis (RAS). Useful diagnostic studies were DTPA renal scan following pretreatment with captopril, and selective renal angiography. All patients underwent attempted surgical revascularization of the RAS and three had aortoaortic bypass of an abdominal aortic narrowing. Of the 14 kidneys that had repair of RAS, a successful outcome was obtained in 11 (80%). Three patients required unilateral nephrectomy. Five of eight patients are normotensive and off all medications, and three are normotensive on reduced medication doses.

PURPOSE: Posterior sagittal anorectoplasty, regarded as a standard surgical primary repair for anorectal malformations in infancy, was evaluated for effectiveness when performed as a secondary operation for establishing continence in the adult. The purpose of this review was to evaluate our results of performing posterior sagittal anorectoplasty in adult patients and to emphasize the extensive evaluation required to perform proper patient selection. METHOD: From January 1, 1992, to December 31, 1996, eight patients with Grade 3 incontinence underwent posterior sagittal anorectoplasty. The ages ranged from 13 to 40 (mean, 26) years. RESULTS: All patients had diverting stomas at the time of repair and all but one had restoration of intestinal continuity. Of eight patients who underwent posterior sagittal anorectoplasty, one failed secondary to rectal ischemia and retained a diverting stoma. Six patients had restoration of continuity. Five patients were continent and one had incontinence only to gas. DISCUSSION: We have established that posterior sagittal anorectoplasty can effectively be used to establish continence as a secondary procedure for a select group of adult patients.

It has been previously reported that the administration of dexamethasone (DEX) to adult rats increases the activity of plasma platelet-activating factor acetylhydrolase (PAF-AH) and prevents the development of intestinal necrosis caused by platelet activating factor (PAF) injection. In this report, we examined the effect of DEX administration on plasma PAF-AH activity during the perinatal period. Timed-pregnant rats received DEX (0.2–1.0 mg/kg/d) or normal saline (controls) on days 16–18 (early group) or days 18–20 (late group) of gestation. Maternal plasma PAF-AH activity was lower in late gestation than in postpartum period (P < 0.001). Fetal and neonatal plasma PAF-AH activity was higher than maternal values (P < 0.05). No changes of PAF-AH activity were seen in maternal, fetal or neonatal plasma after prenatal DEX administration at the aforementioned doses. A higher dose of DEX (1.3 mg/kg/d × 4d) or cortisone (200 mg/kg/d) produced an elevation of maternal plasma PAF-AH activity (DEX 79.2 ± 3.0, cortisone 70.5 ± 1.9 vs. controls 49.4 ± 2.3 nmol/min/ml, P < 0.01), but resulted in a high fetal mortality. Treatment of newborn rats with DEX (0.5 mg/kg/d) on days 1–3 after birth, increased plasma PAF-AH activity on day 4 (DEX 292 ± 5 versus controls 140 ± 9 nmol/min/ml, P < 0.001) and day 6 (DEX 302 ± 12 versus controls 136 ± 6 nmol/min/ml, P < 0.001). Postnatal administration of DEX increases the plasma PAF-AH activity in the rat. Only high doses of prenatal corticosteroids that cause fetal death can elevate maternal plasma PAF-AH activity.

We report a case of primary retractile mesenteritis presenting as acute abdominal pain requiring surgery in a 3-year-old boy. At laparotomy, a hemoperitoneum was discovered and the diagnosis of primary mesenteritis was made with the aid of frozen section biopsy of the small bowel mesentery. No resection was necessary, and he made an uneventful recovery and remains well on follow-up.

This study compared the postoperative pain relief provided by simple instillation of bupivacaine into a hernia wound with that provided by ilioinguinal/iliohypogastric (IG/IH) nerve block. Sixty children undergoing inguinal hernia repair under general anesthesia were randomized to receive 0.25 ml/kg of 0.25% bupivacaine for either IG/IH nerve block or up to 0.5 ml/kg of the same solution for instillation nerve blocks. In the postanesthesia care unit (PACU), a trained blinded observer evaluated the patient's level of postoperative pain using a standardized 10-point objective pain scale. Fentanyl 1-2 micrograms/kg was administered intravenously to any child scoring 6 or more points on the pain scale. The difference in pain scores among the two groups were compared. The two groups were not significantly different in age, duration of surgery, or anesthesia. There was no significant difference between patients who received the two treatment modalities in their pain scores, analgesic requirements in the PACU, recovery times, and discharge times. These results demonstrate that the simple instillation of local anesthetics into a wound provides postoperative pain relief following hernia repair, which is as effective as that provided by intraoperative IG/IH nerve block.

Utilizing a pulse oximeter, oxygen saturation was determined in the periphery (tongue) and compared to the intestinal wall in a canine model. Segmental intestinal ischemia was then produced and the last obtainable pulse oximetry reading was marked with a suture on either end of the ischemic segment. Reexploration was performed within 48 hr and full thickness biopsies were taken in normal intestine and at various distances into the ischemic segment. Blinded evaluation of the biopsies showed that all biopsies taken within 1 cm of a normal pulse oximetry reading were normal, whereas at 3 cm into the ischemic segment 7 of 15 biopsies showed partial necrosis and 5 of 15 showed full thickness necrosis. We conclude that pulse oximetry of the intestinal wall is a valuable method of assessing intestinal viability in this model.

PLATELET ACTIVATING FACTOR (PAF) AND PAF-ACETYLHYDROLASE (PAF-AH) ACTIVITY IN NECROTIZING ENTEROCOLITIS (NEC). † 1200

Patients with end-stage renal disease who develop hypersplenism, patients with mild neutropenia, and those patients whose WBC fails to increase in response to cortisol administration will develop significant neutropenia following transplantation with routine doses of azathioprine. This "intolerance" of azathioprine mandates a reduction in the dose of azathioprine often resulting in allograft rejection. Splenectomy will prevent azathioprine-induced neutropenia, but the hazards of splenectomy in these immunosuppressed patients have led to attempts to salvage at least part of the spleen. Partial splenic ablation by embolization has been utilized in adults prior to transplantation to prevent azathioprine-induced neutropenia while preserving the spleen's protective mechanisms against infection. Eight children in our series of transplant candidates required a reduction of splenic function to prevent azathioprine induced neutropenia. One child had a functioning renal allograft but had recurrent neutropenia limiting the azathioprine dose. Partial splenic embolization was attempted in four children and was initially successful in two. Both patients later developed recurrent neutropenia and needed partial splenectomy. The two patients in whom partial splenic embolization was unsuccessful and five further patients in whom embolization was not attempted also underwent partial splenectomy. Approximately 75% to 80% of the spleen was resected. Six children have since undergone renal transplantation and one child had a transplant with chronic rejection at the time of partial splenectomy. Routine doses of azathioprine have been used in these children with no episodes of neutropenia or sepsis observed. We recommend partial splenectomy in those children requiring renal transplantation who are at risk for development of azathioprine induced neutropenia.

Burns constitute a major cause of childhood death and disability. Prevention of these injuries remains the most effective method of dealing with them and is the responsibility of all persons caring for children. Despite all therapeutic advances, 3% of children admitted to our burn unit die of their injury; many more suffer significant physical and psychological morbidity. Care for these patients is best provided by a multidisciplinary unit with many professionals working together as a team from the day of injury until the child reaches adulthood.

Tumors of the liver are uncommon in children. It is imperative that surgeons caring for these patients have a knowledge of the likely diagnosis, proper radiographic evaluation, and appropriate selection and extent of surgical therapy. Of the benign lesions, infantile hemangioendothelioma often is best treated nonoperatively, whereas mesenchymal hamartoma and teratoma should be resected. All malignant lesions must be completely excised to cure the patient, but some may be converted from unresectable tumors to resectable ones by preoperative chemotherapy. Major hepatic resection is one of the most formidable procedures in the surgery of children. Careful preparation, attention to technical detail, and precise postoperative management of the critically ill child are prerequisites for success. For a recent review of the extrahepatic biliary tract problems of infants and children, the reader is referred to the article by Stolar and Altman in Surgical Clinics of North America, Volume 65, October 1985.

Burns constitute a major cause of childhood death and disability. Prevention of these injuries remains the most effective method of dealing with them and is the responsibility of all persons caring for children. Despite all therapeutic advances, 3% of children admitted to our burn unit die of their injury; many more suffer significant physical and psychological morbidity. Care for these patients is best provided by a multidisciplinary unit with many professionals working together as a team from the day of injury until the child reaches adulthood.

The use of percutaneous transluminal angioplasty as the primary treatment of renovascular stenosis in adults has recently been described. Previously, only three children have been reported to have undergone transluminal angioplasty for stenosis of the renal artery and hypertension. At our hospital, transluminal angioplasty was attempted in four children with renal artery stenosis; one attempt was successful and three were unsuccessful. The three patients who required surgical repair of the renal artery after unsuccessful transluminal angioplasty have been described in detail. The histopathology of the stenotic vessels is also discussed. Based on the analysis of the three children, certain criteria have been derived to select pediatric patients with renovascular hypertension either for attempted transluminal angioplasty or for primary surgical revascularization.

Polytetrafluoroethylene (PTFE) grafts have been a useful addition to the pediatric hemodialysis vascular access armamentarium. In this study, 17 pediatric patients underwent 331 total months of hemodialysis via PTFE grafts. There was a statistically significant (P less than .025) decrease in the incidence of graft infections in 12 patients (235 patient-months) while receiving prophylactic parenteral vancomycin compared with 9 patients (96 patient-months) while receiving no vancomycin (0% v 44%). Vancomycin side effects were uncommon and mild. Vancomycin is a safe and effective agent for the prevention of PTFE graft infections in pediatric patients receiving chronic hemodialysis.

Colon interposition is an accepted method of esophageal replacement in children. Severe symptomatic reflux has occurred in only two of our patients with colonic esophageal substitution. Formation of an antireflux submucosal gastric tunnel eliminated gastrocolic reflux in both patients without impeding normal passage of food into the stomach.

Four children, aged 5 to 13 years, were treated for malignant mesenchymoma of the liver. Two survived longer than 5 years with complete resection and adjuvant chemotherapy. Two children who had incomplete resection with microscopic residual mesenchymoma both had tumor recurrence and died of disease progression despite radiation therapy and chemotherapy. Although previous reports emphasize a poor prognosis for children with this tumor, our experience would indicate that long-term survival can be achieved using complete surgical extirpation and adjuvant chemotherapy.

Cholelithiasis in infancy is believed to be a rare finding. It is also held that all cholelithiasis in infancy has a predisposing factor such as hemolysis, prolonged fasting, congenital abnormality of the biliary tree, or the administration of TPN and is associated with severe morbidity and mortality. In the last three years, four infants at our hospital have been found on real-time ultrasonography to have gallstones. Their ages ranged from 3 weeks to 6 months. One patient had a hemolytic disease but the finding of cholelithiasis in the other three patients was incidental. A repeat sonogram in the infant with hemolysis three months after initial recognition and one day before scheduled cholecystectomy revealed spontaneous resolution of the gallstones. This led us to a conservative approach in the other three patients. Two more infants had spontaneous resolution of their gallstones and one child, now aged 16 months, is thriving with a persistent asymptomatic gallstone visible on sonogram.