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    Barrie Rich

    ABSTRACT We report a case of mixed type IIIb and type IV intestinal atresia. The baby underwent exploratory laparotomy where he was found to have the entire distal bowel supplied by one blood vessel that appeared to have undergone an... more
    ABSTRACT We report a case of mixed type IIIb and type IV intestinal atresia. The baby underwent exploratory laparotomy where he was found to have the entire distal bowel supplied by one blood vessel that appeared to have undergone an antenatal volvulus. The volvulus was untwisted and upon further exploration the patient was noted to have twelve distal atresias. We performed a total of six primary small bowel anastomoses. The baby did not require any further procedures and did well post-operatively. We conclude that patients with this congenital abnormality can be treated with primary anastomoses, and when appropriate, without the need for enterostomy or stent.
    We evaluated prognostic factors among young patients with early stage melanoma, with particular attention to survival, recurrence, and development of a second primary melanoma. We retrospectively reviewed patients (age <22years) with... more
    We evaluated prognostic factors among young patients with early stage melanoma, with particular attention to survival, recurrence, and development of a second primary melanoma. We retrospectively reviewed patients (age <22years) with pathologically confirmed in-situ and stage 1 non-Spitzoid melanoma treated at our institution from 1980-2010, assessing demographics, clinical presentation, treatment, disease-specific survival, recurrence-free survival, and probability of developing a second primary melanoma. One hundred patients with in-situ melanoma (n=16) or stage 1A (n=48) or 1B (n=36) melanoma were identified. Median age was 19.4years (range, 11.2-21.9), and median follow-up was 7.6years (range, 0.1-31.7). Median tumor thickness was 0.76mm (range, 0.23-2.0). No lesions were ulcerated. All patients underwent wide local excision with negative margins, and 21 had a concomitant negative sentinel lymph node biopsy (SLNB). Sixteen patients developed recurrences, and 8 subsequently di...
    Image-defined vessel encasement is a significant risk factor for surgical complications and incomplete resection for intermediate-risk tumors. We sought to examine the impact of vessel encasement on complications or resectability in... more
    Image-defined vessel encasement is a significant risk factor for surgical complications and incomplete resection for intermediate-risk tumors. We sought to examine the impact of vessel encasement on complications or resectability in intermediate-risk or high-risk patients after neoadjuvant chemotherapy. We retrospectively reviewed 207 consecutive patients with circumferential encasement of the renal vessels, celiac axis, and/or superior mesenteric artery (SMA) who underwent resection between 1991 and 2009. Specifically, we evaluated resection rates, complications, and outcome. Median age at diagnosis was 3.0 years, and 79% of patients had stage 4 disease. Of known MYCN status, 23.4% had MYCN amplification. Vessel encasement included renal vessels, celiac axis, or SMA alone in 107, 7, and 4 patients, respectively. Both the renal vessels and celiac axis were encased in 5 patients, renal vessels and SMA in 7 patients, and celiac axis and SMA in 14 patients. Sixty-three patients had all...
    Introduction. Pancreas transplantation (PTx) is the only definitive intervention for type 1 diabetes. Medical advancements in diabetes care have led to an aging PTx candidate pool. We report our experience with patients ≥50 years of age... more
    Introduction. Pancreas transplantation (PTx) is the only definitive intervention for type 1 diabetes. Medical advancements in diabetes care have led to an aging PTx candidate pool. We report our experience with patients ≥50 years of age undergoing PTx. Methods. We reviewed 136 consecutive PTx patients at our institution from 1996-2010; 17 were ≥50 years of age. We evaluated demographics, surgical complications, acute rejection (AR) rates, nonsurgical infections, and survival outcomes. Results. Demographic data was similar (P > .05) between groups, excluding age. The two groups had comparable major and minor surgical complication rates (P = .10 and P = .25, resp.). The older group had a lower 1-year and overall AR rate (P = .04 and P = .03, resp.). The incidence of non-surgical infections and overall patient and graft survival was similar between groups (P > .05). Conclusion. Older patients with type 1 diabetes are feasible candidates for PTx, as surgical morbidity, incidence of infections, and AR rates are low.
    The study of cancer immunology has provided diagnostic and therapeutic instruments through serum autoantibody biomarkers and exogenous monoclonal antibodies. While some endogenous antibodies are found within or surrounding transformed... more
    The study of cancer immunology has provided diagnostic and therapeutic instruments through serum autoantibody biomarkers and exogenous monoclonal antibodies. While some endogenous antibodies are found within or surrounding transformed tissue, the extent to which this exists has not been entirely characterized. We find that in transgenic and xenograft mouse models of cancer, endogenous gamma immunoglobulin (IgG) is present at higher concentration in malignantly transformed organs compared to non-transformed organs in the same mouse or organs of cognate wild-type mice. The enrichment of endogenous antibodies within the malignant tissue provides a potential means of identifying and tracking malignant cells in vivo as they mutate and diversify. Exploiting these antibodies for diagnostic and therapeutic purposes is possible through the use of agents that bind endogenous antibodies.
    Cefazolin is used commonly to prevent surgical site infection (SSI) after operations on patients with morbid obesity (MO), but specific dosing guidelines are lacking. We hypothesized that cefazolin 2 g given by intravenous (IV) push over... more
    Cefazolin is used commonly to prevent surgical site infection (SSI) after operations on patients with morbid obesity (MO), but specific dosing guidelines are lacking. We hypothesized that cefazolin 2 g given by intravenous (IV) push over 5 min (IVP) or infusion over 30 min (INF) would suffice for SSI prophylaxis in MO (body mass index [BMI] 40-50 kg/m(2)), and cefazolin 3 g would be sufficient in patients with super-morbid obesity (SMO) (BMI >50 kg/m(2)). Twenty-five patients undergoing elective surgical procedures were given a single dose of cefazolin: Ten with MO received 2 g via IVP (MO2-IVP), five with MO received 2 g via 30-min infusion (MO2-INF), five with SMO received 2 g via infusion (SMO2-INF), and five with SMO received 3 g via infusion (SMO3-INF). Serum cefazolin concentrations were measured 5, 30, 120, and 360 min after initiation of the dose. The half-life of the drug was calculated for each patient, as was the time the concentration was above the minimum inhibitory free concentration (fT>MIC) using 8 mcg/mL as the breakpoint. The protective duration of each cefazolin dose was assessed using the pharmacodynamic target for fT>MIC of 70%. The mean cefazolin concentrations after 30 min were similar in all groups; the mean concentrations at 120 and 360 min were 67.1-84.8 mcg/mL and 22.9-40.8 mcg/mL, respectively. The half-life ranged from 2.3 to 3.6 h and was unaffected by administration method. The protective duration was 5.1 h for MO2-IVP, 4.8 h for MO2-INF, 5.8 h for SMO2-INF, and 6.8 h for SMO3-INF. A single 2-g dose of cefazolin appears to provide antibiotic exposures sufficient for most common general surgical procedures of <5-h duration, regardless of BMI.
    Proper dosing of specific antibiotics in morbidly obese patients has been studied inadequately. However, these data are beneficial as this patient population is at an increased risk to develop postoperative infections. Cefepime is an... more
    Proper dosing of specific antibiotics in morbidly obese patients has been studied inadequately. However, these data are beneficial as this patient population is at an increased risk to develop postoperative infections. Cefepime is an antibiotic used for the treatment of both gram-positive and especially gram-negative infections; administration of the appropriate dose in the morbidly obese population is crucial. We therefore examined the pharmacokinetics of cefepime in patients with body mass index >40 kg/m(2). Ten morbidly obese patients, with a mean [±SD] estimated glomerular filtration rate of 108.4 ± 34.6 mL/min, undergoing elective weight loss surgical procedures were administered cefepime in addition to standard prophylactic cefazolin and studied. Serial serum cefepime concentrations were analyzed after dosing using a validated high performance liquid chromatography method. Pharmacokinetics and duration above the minimum inhibitory concentration (MIC) were determined using a protein binding value of 15% and a MIC threshold of 8 μg/mL. Mean free cefepime concentrations for t = 30, 120, and 360 min were 69.6, 31.6, and 9.2 μg/mL, respectively. The dosing interval was calculated to maintain the free concentration above the MIC (fT > MIC) for 60% of the interval. This was determined to be 10.12 h, including time for infusion. There was no toxicity. Based on this analysis, an increased dose of 2 g every 8 h is necessary to maintain an adequate fT > MIC throughout the dosing interval. Further studies are necessary to determine the efficacy of this regimen in the settings of active infections and critical illness.
    This report describes a rare benign cystic lesion of the pancreas known as acinar cell cystadenoma. There are 12 previously reported cases, the youngest patient from which was age 16. We report the youngest case to date, occurring in a... more
    This report describes a rare benign cystic lesion of the pancreas known as acinar cell cystadenoma. There are 12 previously reported cases, the youngest patient from which was age 16. We report the youngest case to date, occurring in a 9-year-old boy. The pancreatic lesion was found incidentally and confirmed by laparoscopic biopsy. A pseudocyst formed, which was drained endoscopically. Given that the lesion was benign and the patient was asymptomatic, no further intervention was recommended. Complete resection would require a total pancreatectomy, with attendant morbidity, and review of the literature suggests a benign course. In asymptomatic cases, we recommend biopsy for histologic diagnosis, with close follow-up and without further surgical intervention.
    Patients with locally recurrent or persistent high-risk neuroblastoma are difficult to treat. We describe our experience using intraoperative radiation therapy (IORT) after re-resection in this high-risk population. We retrospectively... more
    Patients with locally recurrent or persistent high-risk neuroblastoma are difficult to treat. We describe our experience using intraoperative radiation therapy (IORT) after re-resection in this high-risk population. We retrospectively reviewed 44 consecutive patients who received IORT at our institution between April 2000 and September 2009 after gross total resection of recurrent/persistent tumor. Specifically, we evaluated local recurrence rates, complications, and overall survival. The median age at diagnosis was 41.5 months. Median follow-up after IORT was 10.5 months. Each patient received prior chemotherapy and surgery, while 94.5% had previous external beam radiation therapy. MYCN was amplified in 34% of patients. There were no operative or postoperative deaths, and 18 patients (40.9%) had postoperative complications. There was a 50.4% probability of local control. MYCN amplification did not affect local control (local recurrence rate of 53.9% vs 52.4%, P = .89). Median overall survival was 18.7 months (95% confidence interval, 11.7-25.6 months). Mean survival for MYCN-amplified patients was 13.0 vs 39.2 months for those without MYCN amplification (P = .035). Intraoperative radiation therapy after re-resection of locally recurrent/persistent neuroblastoma results in a reasonable rate of local control with acceptable morbidity and survival. This approach should be considered in this high-risk population.
    This case report documents the eighth reported case of renal cell carcinoma (RCC) occurring after treatment of Wilms tumor. Although secondary malignancies after treatment of Wilms tumors are not infrequent, RCC as the second malignancy... more
    This case report documents the eighth reported case of renal cell carcinoma (RCC) occurring after treatment of Wilms tumor. Although secondary malignancies after treatment of Wilms tumors are not infrequent, RCC as the second malignancy is rare. We discuss a 17-year-old girl whose RCC was diagnosed 12.5 years after diagnosis of a Wilms tumor. In addition, we review the literature on the subject. Renal cell carcinoma has been proposed as a consequent of chemoradiation; however, a genetic susceptibility must be considered. Because it is routine to assess the functional status of the remaining solitary kidney by annual ultrasonography, we recommend assessing for the presence of secondary renal malignancies and perhaps continuing routine ultrasounds long-term.
    As application and awareness of single-incision pediatric endosurgery (SIPES) are increasing, various techniques and indications have been independently described by select centers around the world. In order to facilitate a cooperative... more
    As application and awareness of single-incision pediatric endosurgery (SIPES) are increasing, various techniques and indications have been independently described by select centers around the world. In order to facilitate a cooperative approach toward advancing and investigating the practice of SIPES, we conducted a survey among members of the International Pediatric Endosurgery Group (IPEG), asking them about their experience and attitudes toward single-incision endosurgery. After institutional review board approval, an invitation to participate in an online survey was sent to all IPEG members. Questions focused on demographic information, practice patterns, indications, and equipment used regarding SIPES. Of the 560 contacted active IPEG members, 115 completed the survey (recall 21%). The respondents represented pediatric surgeons from 32 countries on six continents. Of respondents, 97% had heard of, while 71% had performed, SIPES. Reasons for not having performed SIPES included disbelief in benefit (59%), lack of proficiency (34%), and inadequate resources (28%). The most commonly performed SIPES procedures were appendectomy (85%), cholecystectomy (66%), splenectomy (42%), pyloromyotomy (35%), and intestinal surgery (13%), as well as Nissen fundoplication and gynecologic adnexal pathology (7%). The equipment and techniques utilized showed large variation and included some self-devised, innovative, low-resource approaches. Complications with SIPES reported by the survey participants included technical difficulties, wound infection, and prolonged operating time. SIPES is being performed worldwide for a large spectrum of common indications in pediatric surgery. The equipment and techniques used vary with geographic location and resources. Some encountered complications are common to those seen with conventional minimally invasive surgery, whereas others may be SIPES-specific. Different respondents reported diverging views on pain, operating time, and cost.
    Paragangliomas are a type of neuroendocrine tumor that has been reported to be present in patients with cyanotic congenital heart disease. This report documents the first case of a patient with successful resection of a sympathetic... more
    Paragangliomas are a type of neuroendocrine tumor that has been reported to be present in patients with cyanotic congenital heart disease. This report documents the first case of a patient with successful resection of a sympathetic paraganglioma in the setting of unrepaired tetralogy of Fallot, the most common cause of cyanotic heart disease, with pulmonary atresia. We present a 33-yr-old woman with hypertensive crises from a paraganglioma who presented for surgical resection. The patient's preoperative workup was consistent with a functioning sympathetic paraganglioma. Preoperative transesophageal echocardiogram displayed normal ventricular function, moderate-severe right ventricular hypertrophy, severe right ventricular hypertension, an overriding aorta, bidirectional shunting, pulmonary atresia, and aortopulmonary collaterals. The patient underwent a successful laparoscopic resection of a functioning 7-cm paraganglioma after careful preoperative preparation and intraoperative monitoring. Pathology returned as a well-defined, partially hemorrhagic mass measuring 7.0 × 4.5 × 4.5 cm adjacent to and compressing the adrenal gland. Surgical resection of paraganglioma tumors in rare patients such as this one is appropriate; however, surgery requires meticulous perioperative management with a multidisciplinary approach. Future studies are needed to determine whether there is a link between neuroendocrine tumors and cyanotic congenital heart disease.
    Tracheal occlusion (TO) induces lung growth in congenital diaphragmatic hernia (CDH) but is also associated with drawbacks. We devised a temporary gel plug that induced lung growth when placed in the fetal trachea. This study evaluates... more
    Tracheal occlusion (TO) induces lung growth in congenital diaphragmatic hernia (CDH) but is also associated with drawbacks. We devised a temporary gel plug that induced lung growth when placed in the fetal trachea. This study evaluates the effects of temporary versus permanent TO on histologic radial alveolar count (RAC) and vascular morphometrics. Experimental CDH was created surgically in 64 New Zealand White rabbit fetuses on gestational day (GD) 24. On GD 27, these fetuses were randomized to intratracheal instillation of a fibrin gel plug (GP), tracheal suture ligation (SL), intratracheal instillation of normal saline (NS), or sham amniotomy (SH). Non-manipulated fetuses served as controls (NM). Histologic lung sections were assessed blindly for RAC and relative arterial adventitial thickness (%AT) as a variable for vascular remodelling. Results were statistically compared. RAC was significantly lower in the ipsilateral lung of SH fetuses than in the contralateral lung (p = 0.011). Mean RAC was higher after SL (p < 0.001) and GP (p = 0.03) compared to SH. Furthermore, %AT was higher in GP (50 ± 28, p < 0.001) and SL (45 ±2 6, p = 0.003) fetuses than in controls (36 ± 19). Temporary and permanent TO leads to increased RAC; this effect was more pronounced with permanent TO. Both interventions were associated with an increased %AT. These findings may explain the adverse clinical effects of TO, despite causing accelerated lung growth.
    In kidney, liver, heart, and lung transplantation, extremes of body mass index (BMI) have been reported to influence post-operative outcomes and even survival. Given the limited data in pancreas transplantation, we sought to elucidate the... more
    In kidney, liver, heart, and lung transplantation, extremes of body mass index (BMI) have been reported to influence post-operative outcomes and even survival. Given the limited data in pancreas transplantation, we sought to elucidate the influence of BMI on outcomes. We reviewed 139 consecutive pancreas transplants performed at our institution and divided them into four categories based on BMI: underweight (≤18.5 kg/m(2)), normal (18.6-24.9 kg/m(2)), overweight (25-29.9 kg/m(2)), and obese (≥30 kg/m(2)). Parameters analyzed included post-operative complications, early graft loss, one-yr acute rejection rate (AR), non-surgical infections, and survival. Demographic data were similar between the groups. Compared with normal, only obese patients trended toward more post-operative complications (p = 0.06). Underweight and obese patients had significantly more post-operative infectious complications than normal (p = 0.0005 and p = 0.03, respectively). Obese patients had more complications requiring percutaneous drainage compared with normal (p = 0.03). Overweight and obese patients had significantly more complications requiring re-laparotomy (p = 0.03 and p = 0.048, respectively). Early graft loss, AR, non-surgical infections, and patient and graft survival rates were not different between normal and underweight, overweight, or obese patients (p > 0.05). Extremes of BMI were associated with increased morbidity. Donors and recipients should be carefully selected to maximize potential for successful outcomes.