Frederick C Ryckman

Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States

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Publications (115)382.7 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: The surgical consent serves as a key link in preventing breakdowns in communication that could lead to wrong-patient, wrong-site, or wrong-procedure events. We conducted a quality improvement initiative at a large, urban pediatric academic medical center to reliably increase the percentage of informed consents for surgical and medical procedures with accurate safety data information at the first point of perioperative contact. Improvement activities focused on awareness, education, standardization, real-time feedback and failure identification, and transparency. A total of 54,082 consent forms from 13 surgical divisions were reviewed between May 18, 2011, and November 30, 2012. Between May 2011 and June 2012, the percentage of consents without safety errors increased from a median of 95.4% to 99.7%. Since July 2012, the median has decreased slightly but has remained stable at 99.4%. Our results suggest that effective safety checks allow discovery and prevention of errors.
    AORN journal 07/2014; 100(1):42-53. DOI:10.1016/j.aorn.2013.07.023
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    ABSTRACT: Bed capacity management is a critical issue facing hospital administrators, and inefficient discharges impact patient flow throughout the hospital. National recommendations include a focus on providing care that is timely and efficient, but a lack of standardised discharge criteria at our institution contributed to unpredictable discharge timing and lengthy delays. Our objective was to increase the percentage of Hospital Medicine patients discharged within 2 h of meeting criteria from 42% to 80%. A multidisciplinary team collaborated to develop medically appropriate discharge criteria for 11 common inpatient diagnoses. Discharge criteria were embedded into electronic medical record (EMR) order sets at admission and could be modified throughout a patient's stay. Nurses placed an EMR time-stamp to signal when patients met all discharge goals. Strategies to improve discharge timeliness emphasised completion of discharge tasks prior to meeting criteria. Interventions focused on buy-in from key team members, pharmacy process redesign, subspecialty consult timeliness and feedback to frontline staff. A P statistical process control chart assessed the impact of interventions over time. Length of stay (LOS) and readmission rates before and after implementation of process measures were compared using the Wilcoxon rank-sum test. The percentage of patients discharged within 2 h significantly improved from 42% to 80% within 18 months. Patients studied had a decrease in median overall LOS (from 1.56 to 1.44 days; p=0.01), without an increase in readmission rates (4.60% to 4.21%; p=0.24). The 12-month rolling average census for the study units increased from 36.4 to 42.9, representing an 18% increase in occupancy. Through standardising discharge goals and implementation of high-reliability interventions, we reduced LOS without increasing readmission rates.
    BMJ quality & safety 01/2014; DOI:10.1136/bmjqs-2013-002556 · 3.28 Impact Factor
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    ABSTRACT: Portal vein thrombosis (PVT) occurs in ≤12% of pediatric recipients of liver transplantation (LT). Known complications of PVT include portal hypertension, allograft loss, and mortality. The management of PVT is varied. A single-center, case-control study of pediatric LT recipients with portal vein (PV) changes after LT was performed. Cases were categorized as early PVT (if PVT was detected within 30 days of transplantation) or late PVT (if PVT was detected more than 30 days after transplantation or if early PVT persisted beyond 30 days). Two non-PVT control patients were matched on the basis of the recipient weight, transplant indication, and allograft type to each patient with PVT. Thirty-two of the 415 LT recipients (7.7%) received 37 allografts and developed PVT. In comparison with control patients, a higher proportion of patients with PVT had PVT present before LT (13.3% versus 0%, P = 0.01). Patients with early PVT usually returned to the operating room, and 9 of 15 patients (60%) had PV flow restored. Patients with late PVT had lower white blood cell (4.9 [1000/μL] versus 6.8 [1000/μL], P < 0.01) and platelet counts (140 [1000/μL] versus 259 [1000/μL], P < 0.01), an elevated international normalized ratio (1.2 versus 1.0, P < 0.001), and more gastrointestinal bleeding (25% versus 8.3%, P = 0.03) compared to controls. Patients with PVT were also less frequently at the expected grade level (52% versus 88%, P < 0.001). The patient survival rates were 84%, 78%, and 78% and 91%, 84%, and 79% for cases and controls at 1, 5, and 10 years, respectively. The allograft survival rates were 90%, 80%, and 80% for cases and 94%, 89%, and 87% for controls at 1, 5, and 10 years, respectively. In conclusion, patients with early and late PVT had preserved allograft function, and there was no impact on mortality. Patients diagnosed with early PVT often underwent operative interventions with successful restoration of flow. Patients diagnosed with late PVT experienced variceal bleeding, and some required portosystemic shunting procedures. Academic delays were also more common. In late PVT, the clinical presentation dictates care because the optimal management algorithm has not yet been determined. Multi-institutional studies are needed to confirm these findings and improve patient outcomes. Liver Transpl 19:315-321, 2013. © 2013 AASLD.
    Liver Transplantation 03/2013; 19(3):315-321. DOI:10.1002/lt.23583 · 3.79 Impact Factor
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    ABSTRACT: To determine whether portoenterostomy (PE) revision in patients afflicted with biliary atresia (BA) is a viable treatment option and, if so, identify which patients may benefit. BA, the most common cause of neonatal liver disease, results in biliary tract obstruction and hepatic fibrosis. Kasai PE is the initial surgical intervention performed and, if successful, restores drainage and preserves the native liver. Portoenterostomy failure warrants liver transplantation, but because of complications related to transplantation, treatment strategies to salvage the native liver may be beneficial. Using uniformly applied criteria, we have revised PEs to delay or avoid transplantation. A retrospective review of medical records of patients diagnosed with BA since 1983 was performed. Patient demographics, symptoms, indications for revision, laboratory values, and outcomes were recorded. A cohort of patients who underwent revision after initial PE was identified. Survival rates were assessed using the Kaplan-Meier method. For patients who required transplantation, operative data from the revised PE cohort were compared with those from the unrevised PE cohort. A Cox proportional hazards model was used to determine covariates predictive of a favorable outcome. Of 181 children who underwent PE, 24 underwent revision. Adequate biliary drainage, as evidenced by normalized conjugated bilirubin levels, was achieved in 75% of revised patients. Overall survival in patients who underwent revision, regardless of transplantation, was 87%. Among patients who underwent PE revision, 46% have survived with their native liver. Experience at our center suggests that with appropriate patient selection, PE revision may delay the need for liver transplanation yielding encouraging patient outcomes.
    Annals of surgery 03/2012; 255(3):570-6. DOI:10.1097/SLA.0b013e318243a46e · 7.19 Impact Factor
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    ABSTRACT: Multiple visits for the evaluation, treatment, and follow-up of straightforward surgical problems are inconvenient, can result in lost work for the parents, and missed school for the child. We hypothesized that with proper previsit screening, patients with select diagnoses can be evaluated in an outpatient clinic setting and undergo operation the same day. Criteria were developed to identify straightforward referrals to our surgical practice for umbilical, epigastric, or inguinal hernias. Scripting was created to offer families the option of consultation and, if indicated, surgical treatment on the same day. Data collected included number of patients, cases performed, insurance status, and consultation reimbursement and surgical fees. Families were surveyed postoperatively. Sixty-one patient candidates participated. The diagnosis and indication for surgery were confirmed in 56 (92%), of which 50 underwent repair the day of their consultation. Seventy-two percent of patients had commercial insurance, whereas 28% had Medicaid. The preoperative consultation fee was reimbursed in 39 (78%) of 50 encounters (57% Medicaid, 86% commercial). All surgical cases were reimbursed. Patient and family satisfaction was high. We conclude that it is feasible to provide same-day evaluation and service for straightforward pediatric hernias with acceptable financial reimbursement and high parent satisfaction.
    Journal of Pediatric Surgery 01/2012; 47(1):213-6. DOI:10.1016/j.jpedsurg.2011.10.047 · 1.31 Impact Factor
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    ABSTRACT: A 15-year-old boy presented with a growing mass on the anterior chest wall. History, clinical examination, and preoperative imaging studies were consistent with soft tissue sarcoma. He underwent open biopsy, and the intraoperative pathology diagnosis of nodular fasciitis resulted in performance of a lesional excision, rather than a potentially morbid wide resection. Nodular fasciitis is a rare but important soft tissue lesion, which can be easily confused with sarcoma. The possibility of benign etiologies for soft tissue masses should be considered when planning surgical options, even when preoperative imaging studies suggest more aggressive lesions.
    Journal of Pediatric Hematology/Oncology 10/2010; 33(4):316-9. DOI:10.1097/MPH.0b013e3181e88649 · 0.96 Impact Factor
  • Janice A Taylor · Frederick C Ryckman
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    ABSTRACT: Patients' inability to take oral nutrition calls for alternative feeding. In selected pediatric patients, traditional feeding tubes are not tolerated and jejunal feeding tubes can be obstructive. One option is a Roux-en-Y feeding limb. Our institution noted complications secondary to small bowel volvulus around this limb. Goals of this study were to review patients who experienced volvulus after Roux-en-Y creation, and to identify factors contributing to this complication. Institutional review board approval was obtained for a retrospective chart review. 25 patients were identified as having a Roux-en-Y jejunal feeding limb. Five developed volvulus. Factors documented included age, time to complication, revision, and outcome. Average age at limb creation was not statistically significant between those with or without volvulus. Mean time to obstruction was 228 +/- 117 days post-limb creation. Average limb length was 18.7 +/- 7 cm in patients with volvulus, 14 +/- 2.3 cm in patients without. 3 of 5 patients presenting with volvulus were discharged home after revision; two patients died. There is no definitive way to prevent small bowel volvulus around Roux-en-Y feeding limbs. No predictors of volvulus were identified. Once revised, no recurrences were observed. While this complication is uncommon, it has potentially catastrophic outcomes requiring early intervention.
    Pediatric Surgery International 02/2010; 26(4):439-42. DOI:10.1007/s00383-010-2553-1 · 1.06 Impact Factor
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    ABSTRACT: Poor flow of patients into and out of the ICU can result in gridlock and bottlenecks that disrupt care and have a detrimental effect on patient safety and satisfaction, hospital efficiency, staff stress and morale, and revenue. Beginning in 2006, Cincinnati Children's Hospital Medical Center implemented a series of interventions to "smooth" patient flow through the system. Key activities included patient flow models based on surgical providers' predicted need for intensive care and predicted length of stay; scheduling the case and an ICU bed at the same time; capping and simulation models to identify the appropriate number of elective surgical cases to maximize occupancy without cancelling elective cases; and a morning huddle by the chief of staff, manager of patient services, and representatives from the operating room, pediatric ICUS, and anesthesia to confirm that day's plan and anticipate the next day's needs. New elective surgical admissions to the pediatric ICU were restricted to a maximum of five cases per day. Diversion of patients to the cardiac ICU, keeping patients in the postanesthesia care unit longer than expected, and delaying or canceling cases are now rare events. Since implementation of the operations management interventions, there have been no cases when beds in the pediatric ICU were not available when needed for urgent medical or surgical use. A system for smoothing flow, based on an advanced predictive model for need, occupancy, and length of stay, coupled with an active daily strategy for demand/capacity matching of resources and needs, allowed much better early planning, predictions, and capacity management, thereby ensuring that all patients are in suitable ICU environments.
    Joint Commission journal on quality and patient safety / Joint Commission Resources 11/2009; 35(11):535-43.
  • Amit S Rattan · Tal Laor · Frederick C Ryckman · Alan S Brody
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    ABSTRACT: Pectus excavatum (pectus) is a common congenital deformity of the chest wall resulting in a diminished anterior-posterior dimension. Chest CT has become a common study for preoperative assessment. CT evaluation was initially described using a single CT image; it is now common to perform a CT of the entire chest to evaluate pectus. To evaluate the efficacy of chest radiographs compared to chest CT in identifying additional clinically significant abnormalities in the preoperative evaluation of children with pectus. We reviewed the chest CT scans of 209 children and young adults who had been evaluated for possible surgical repair of pectus. Additional abnormalities were categorized as (1) incidental, (2) potentially significant, and (3) findings that affected the decision to perform surgery. Chest radiographs were reviewed for category 3 findings. Seventy-six scans showed additional abnormalities, five in group 2 and two in group 3. Both group 3 findings, a vascular ring and an acute pneumonia, were identified on chest radiographs. Conventional radiographs identified clinically important findings in children and young adults evaluated for pectus surgery. Radiation risks and medical costs might be substantially decreased by obtaining a chest radiograph and using a limited CT technique when a CT scan is ordered for the purpose of obtaining a Haller index.
    Pediatric Radiology 10/2009; 40(2):168-72. DOI:10.1007/s00247-009-1417-0 · 1.65 Impact Factor
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    ABSTRACT: Tracheobronchial separation (TBS) due to blunt chest trauma in children is extremely rare. We report the case of a 14-year-old boy who fell 20 feet and developed respiratory distress, bilateral pneumothoraces, pneumomediastinum, and subcutaneous emphysema. Computed tomography imaging at the initial institution failed to detect tracheobronchial disruption, and the patient was managed conservatively. The patient's worsening condition prompted bronchoscopic examination which revealed complete separation of the right main bronchus from the trachea. Single-lung ventilation was instituted using a fabricated extra-long nasotracheal tube due to the patient's large size and mandibular fracture, and the airway was primarily anastamosed with an open thoracotomy approach. The clinical features of tracheobronchial separation as well as anesthetic, clinical and surgical management of this rare but life-threatening injury are described.
    International journal of pediatric otorhinolaryngology 06/2009; 73(8):1163-7. DOI:10.1016/j.ijporl.2009.04.012 · 1.32 Impact Factor
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    ABSTRACT: Surgical site infections (SSIs) remain a substantial cause of morbidity, mortality, increased length of stay, and increased hospital costs. Cincinnati Children's Hospital Medical Center (CCHMC) used reliability science to dramatically reduce the rate of surgical site infections. Key activities included the development and implementation of strategies to enhance the proportion of patients who receive timely antibiotic administration, a pediatric surgical site infection-prevention bundle, and procedure-specific pediatric surgical site infection-prevention bundles. Measures are presented in monthly reports and annotated control charts that are shared with the improvement team and organizational leadership and that are also posted on the hospital's patient safety intranet site. The Class I and II SSI rate decreased from 1.5 per 100 procedure days at baseline to 0.54 per 100 procedure days, a 64% reduction. The process has remained stable (within control limits) since August 2007. There were 33 fewer surgical site infections in fiscal year (FY) 2006 than in FY 2005, and 21 fewer in FY 2007 than in FY 2006. By December 2007, 91% of eligible same-day surgery patients received antibiotics within 60 minutes before a procedure, and 94% of patients undergoing inpatient surgery received antibiotics within 60 minutes prior to incision. Pediatric surgical patients can now expect a safer, more efficient experience with CCHMC's care system and reduced variation in care across CCHMC's surgeons and procedures. Sharing data on individual and collective provider performance was important in recruiting provider support. Examining data on any failures each day allowed assessment and correction, facilitating rapid-cycle improvement. Making the right thing to do the easy thing to do facilitated the behavior changes required.
    Joint Commission journal on quality and patient safety / Joint Commission Resources 05/2009; 35(4):192-8.
  • Gastroenterology 05/2009; 136(5). DOI:10.1016/S0016-5085(09)63679-9 · 13.93 Impact Factor
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    ABSTRACT: Surveillance ileoscopies are performed regularly immediately post-transplantation to prevent allograft rejection. We investigated whether variability in apoptosis exists between proximal and distal intestinal limbs of double-barreled ileostomies, and if detection varies according to number of biopsies taken and sections prepared for evaluation. We retrospectively analyzed endoscopy/pathology reports of patients who underwent simultaneous proximal and distal ileoscopies during surveillance. We re-reviewed three sections of selected biopsies for the presence of apoptotic bodies and viral inclusions. Seven patients underwent 26 endoscopies in which both distal and proximal limbs were investigated simultaneously. Apoptosis was identified in each limb simultaneously in 21/26 cases (81%). Of the discrepant results, 3/5 (60%) revealed apoptosis in the proximal limb with normal distal limb and 2/5 (40%) had apoptotic bodies identified in the distal limb and normal proximal biopsies. Re-reviewing discrepant biopsies, two patients had at least one piece of mucosa without apoptosis and apoptotic bodies were seen in only 47% of sections. Histologic variability exists between proximal and distal limbs of double-barreled ileostomies and detection of apoptosis increases with number of pieces obtained and sections examined. Investigating both limbs with adequate sample size and rigorous processing may have clinical implications.
    Pediatric Transplantation 02/2009; 14(1):72-6. DOI:10.1111/j.1399-3046.2008.01125.x · 1.63 Impact Factor
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    ABSTRACT: Infants with multiple cutaneous hemangiomas often present with hepatic hemangiomas. They can follow a benign clinical course or require complex management. We reviewed our experience in the management of hepatic hemangiomas. We performed a retrospective review of patients (1996-2007) with hepatic hemangiomas treated in our institution. Twenty-six patients were diagnosed with hepatic hemangiomas as follows: 8 focal, 12 multiple, and 6 diffuse lesions. Nineteen (73%) patients had associated cutaneous hemangiomas. Sixteen patients had multiple and 3 patients had single cutaneous hemangiomas. All patients with multiple or diffuse liver lesions were screened for heart failure and hypothyroidism. Congestive heart failure developed in 4 patients, 3/4 of these patients had diffuse lesions. Two patients required thyroid replacement because of elevated thyroid-stimulating hormone. Because of progression of disease, 9 patients required steroid treatment. Two patients were treated with vincristine and 3 patients received alpha-interferon because of poor response to steroid treatment. Two patients went on to surgical resection for failed response to medical management and worsening heart failure (left lobectomy, liver transplant). Both patients had uncomplicated postoperative courses. Five patients had a previously undescribed constellation of rapidly involuting cutaneous hemangiomas (gone by 3 months, glut-1-negative) with associated liver lesions also resolving at a faster pace (mean resolution of cutaneous hemangiomas, 1.9 vs 7.9 months; P = .001; liver, 5.8 vs 25.3 months; P = .004). All patients in our series survived. Patients with multiple cutaneous hemangiomas should be screened for hepatic lesions. Patients with diffuse or multifocal liver hemangiomas should be screened for congestive heart failure and hypothyroidism. A subgroup of rapidly involuting cutaneous hemangiomas have a significantly shorter time for involution of hepatic lesions. The status of cutaneous lesions can be used as indicators for the liver hemangiomas.
    Journal of Pediatric Surgery 02/2009; 44(1):125-33. DOI:10.1016/j.jpedsurg.2008.10.021 · 1.31 Impact Factor
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    ABSTRACT: As the field of Liver Transplantation has matured, survival alone is no longer in acceptable single metric of success. This chapter explores the impact of the PELD system for donor organ allocation, surgical modification of donor organs, living donation, and long-term transplant-related complications oil overall quality of life and outcome. Strategies to improve survival, overall outcome, and health-related quality of life in long-term recipients are outlined. (C) 2009 Published by Elsevier Inc.
    Seminars in Pediatric Surgery 06/2008; 17(2):123-30. DOI:10.1053/j.sempedsurg.2008.02.008 · 1.94 Impact Factor
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    ABSTRACT: The need for evidence-based practice guidelines requires high quality, carefully controlled clinical research trials. This multidisciplinary conference attempted to: identify urgent clinical and research issues, identify obstacles to performing clinical trials, develop concepts for organ-specific and all-organ research and generate a report that would serve as a blueprint for future research initiatives. A few themes became evident. First, young children present a unique immunologic environment which may lead to tolerance, therefore, including young children in immunosuppression withdrawal and tolerance trials may increase the potential benefits of these studies. Second, adolescence poses significant barriers to successful transplantation. Non-adherence may be insufficient to explain poorer outcomes. More studies focused on identification and prevention of non-adherence, and the potential effects of puberty are required. Third, the relatively naive immune system of the child presents a unique opportunity to study primary infections and alloimmune responses. Finally, relatively small numbers of transplants performed in pediatric centers mandate multicenter collaboration. Investment in registries, tissue and DNA repositories will enhance productivity. The past decade has proven that outcomes after pediatric transplantation can be comparable to adults. The pediatric community now has the opportunity to design and complete studies that enhance outcomes for all transplant recipients.
    Pediatric Transplantation 04/2008; 12(2):153-66. DOI:10.1111/j.1399-3046.2007.00811.x · 1.63 Impact Factor
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    ABSTRACT: Recurrent tracheoesophageal fistulas (RTEFs) remain a therapeutic challenge because open surgical approaches have been associated with substantial rates of morbidity, mortality, and repeat recurrences. Recently, endoscopic techniques for the repair of RTEF have provided an alternative approach with the potential for improved surgical outcomes. However, previous reports have been limited by small patient numbers and variations in technique. By examining a single institution's experience and performing a systematic review of previously published results, we aimed to identify an optimal approach to managing RTEF endoscopically. Retrospective chart review of patients undergoing endoscopic management of RTEF at a single tertiary care institution was performed. Medline search and summated analysis of previously published cases of endoscopically treated RTEF from 1975 to 2007 was conducted. Four patients with RTEF were identified and selected for endoscopic repair at our institution from 2003 to 2007 (mean age, 11.5 months). Under endoscopic guidance, fistula tracts were de-epithelialized with a Bugbee fulgurating diathermy electrode (5-15 W) and then sealed with fibrin glue (Tisseel with added aprotinin). Closure of RTEF was successful in 3 patients after a single attempt. One revision was required after inadvertent recannulation of the tract with an emergent tracheostomy tube change. No patient has evidence of recurrence (mean follow-up, 16 months). In 15 articles of endoscopically repaired RTEF, 37 cases have been reported from 1975 until present. In general, 3 approaches to endoscopic repair have been explored. Analysis of all reported cases in the literature and results from our patient series suggests that endoscopic techniques designed to both de-epithelialize the fistula tract and seal with fibrin glue have the best chance for cure after a single attempt. Patients with long, thin, and small diameter fistula who have enough distal trachea to accommodate a postoperative cuffed ventilating tube beyond the fistula are ideal candidates for endoscopic repair. In select patients, endoscopic management of RTEF using Bugbee cautery and tissue adhesives can reduce morbidity and recurrence associated with open approaches and alternative endoscopic techniques.
    Journal of Pediatric Surgery 02/2008; 43(1):238-45. DOI:10.1016/j.jpedsurg.2007.08.062 · 1.31 Impact Factor
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    ABSTRACT: Achalasia is a rare disorder with less than 5% of patients diagnosed in childhood. Although Heller esophagocardiomyotomy is a proven intervention, incomplete myotomy can lead to clinical failure. Intraoperative esophageal manometry has been used to ensure adequacy of myotomies in adults. The purpose of the present study was to review our experience in the management of children with achalasia. A retrospective review was conducted on the medical records of patients with achalasia diagnosed between November 1999 and March 2007. Patient demographics and interventions were recorded. Outcomes after surgical intervention and esophageal dilation were assessed. Mean follow-up was 3.5 +/- 0.6 years. Intraoperative manometry was used over the past 3 years. Nineteen patients were treated for achalasia. The average age at diagnosis was 13.8 +/- 0.8 years. Most patients underwent esophageal dilation (14/19), receiving on average 2.1 +/- 0.3 dilations. One patient experienced a contained perforation that was treated conservatively. Eleven patients underwent myotomy, as primary therapy (n = 5) or after recurrence of symptoms after dilation (n = 6). Six patients underwent intraoperative manometry. More patients who underwent Heller myotomy without intraoperative manometry had recurrence of symptoms (80% vs 0%, P < .05). Inadequate myotomy is a potential cause for recurrent symptoms after esophagocardiomyotomy in childhood achalasia. Intraoperative esophageal manometry is a safe technique that may improve the success rate of surgery by confirming the adequacy of myotomy thereby decreasing recurrence of symptoms.
    Journal of Pediatric Surgery 02/2008; 43(1):66-70; discussion 70. DOI:10.1016/j.jpedsurg.2007.09.019 · 1.31 Impact Factor
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    ABSTRACT: Despite advances in infection-control practices, surgical site infections (SSIs) remain a substantial cause of morbidity, mortality, and increased costs among hospitalized patients. We used a matched cohort design to compare costs and length of stay for 16 pediatric patients with an SSI with those of 16 matched control patients who had a similar operative procedure during the same time period but in whom an SSI did not develop. On average, length of stay was increased by 10.6 days (P = .02) and costs were increased by $27,288 (P = .01) for each patient with a potentially preventable SSI. On the day of the surgical procedure, costs between study patients and matched controls differed by only 1.4%. By day 3, however, costs were 36% higher for patients with an SSI. While matching study patients and control patients requires significant time from financial and clinical staff, this approach and the resulting data analysis strengthened and focused our efforts to prevent future SSIs and aligned initiatives to reduce SSIs with the business case for quality.
    Quality management in health care 07/2007; 16(3):219-25. DOI:10.1097/01.QMH.0000281058.99929.ea
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    ABSTRACT: Total parenteral nutrition (TPN) has prolonged survival in children with intestinal failure; however, end-stage liver disease owing to TPN-induced cholestasis (ESLD-TPN) may preclude its use. ESLD-TPN is an indication for isolated liver transplantation (ILT) or multivisceral transplantation (MVT). Isolated liver transplantation for ESLD-TPN should only be considered in patients who have the potential for enteral autonomy. We retrospectively reviewed the records of patients with ESLD-TPN who underwent ILT (n = 7) or MVT (n = 5) between 1994 and 2005. The median age at the time of transplantation was 10.0 months. Intestinal failure followed necrotizing enterocolitis (n = 3), gastroschisis (n = 3), gastroschisis with volvulus (n = 3), gastroschisis with atresia (n = 1), malrotation (n = 1), and megacystis microcolon intestinal hypoperistalsis syndrome (n = 1). Isolated liver transplant patients had a median length of small bowel of 70 cm and tolerated a median of 50% of enteral calories. The median length of small bowel in patients who underwent MVT was 29 cm, and none tolerated more than 30% of goal enteral feeds. Reduced-size (n = 5) and whole-liver (n = 2) allografts were used for patients undergoing ILT. Patients undergoing MVT received liver-small bowel-pancreas (n = 4) or liver-small bowel-pancreas-colon (n = 1). Overall patient survival was 57% in ILT (median follow-up = 25.1 months); 3 survivors are TPN independent, and the fourth patient requires TPN 3 days/wk. Patient survival was 40% after MVT (median follow-up = 13.0 months); 1 MVT patient died of abuse 16.9 months after transplant and was TPN independent at the time of death. Both survivors are TPN independent. Bilirubin levels are within normal range in all survivors. Isolated liver transplantation for ESLD-TPN in the setting of intestinal failure is a viable option in patients who have the potential for enteral autonomy. Multivisceral transplantation is the only alternative in patients without the potential for intestinal recovery. Survival can be achieved in patients with ESLD-TPN, but mortality remains high for both procedures.
    Journal of Pediatric Surgery 02/2007; 42(1):143-7. DOI:10.1016/j.jpedsurg.2006.09.049 · 1.31 Impact Factor

Publication Stats

2k Citations
382.70 Total Impact Points


  • 1986–2013
    • Cincinnati Children's Hospital Medical Center
      • • Division of Pediatric General and Thoracic Surgery
      • • Division of Pediatric Neurosurgery
      • • Pediatric Liver Care Center
      • • Division of Pediatric Gastroenterology, Hepatology and Nutrition
      Cincinnati, Ohio, United States
  • 2008
    • University of Utah
      • Department of Pediatrics
      Salt Lake City, Utah, United States
  • 1993–2006
    • University of Cincinnati
      • Department of Surgery
      Cincinnati, Ohio, United States
  • 1992
    • Georgetown University
      • Department of Pediatrics
      Washington, Washington, D.C., United States
  • 1989
    • Yale-New Haven Hospital
      New Haven, Connecticut, United States