Asa G Yancey: The first to describe a modification of the Swenson Technique for Hirschsprung disease.

Hirschsprung disease is a common disease treated by surgeons using different approaches. One approach is eponymously ascribed to Franco Soave and Scott Boley involving a pull-through of ganglionated colon through a distal portion of aganglionated rectum. However, more recent evidence has shown that this same technique was described twelve years before by Asa Yancey, a black surgeon. A review of the evidence follows with a focus on why Yancey may not have rightly received the credit that was because of him.

Standardization of operative equipment reduces cost.

BACKGROUND: We hypothesize that standardizing operative equipment, and reducing variability can safely achieve cost reduction. METHODS: We retrospectively measured supply costs, operative time, intra-operative complications, and length of stay in a cohort of 145 patients at a children's hospital who underwent a laparoscopic appendectomy. A standardized preference card for laparoscopic appendectomy was developed and implemented. Data were prospectively collected on 101 consecutive patients and compared to the retrospective cohort using multiple linear regression. A survey assessing the perception of surgeons, nurses and scrub technologists of the impact of standardization on patient safety, patient care, OR efficiency, and cost was conducted. Wilcoxon rank sum test was used to evaluate associations between clinical role and years of experience with the total level of agreement on the survey. RESULTS: A 20% average reduction was achieved in supply cost per case, with no significant change in operative time (p=0.14), total time in OR (p=0.15), or length of stay (p=0.60). No intra-operative complications were identified in either group. Survey participants agreed that standardization improves cost and safety. Nurses tended to have greater agreement that standardization improved efficiency and patient care compared to other roles (p=0.06). CONCLUSIONS: Standardization of operative equipment can result in a significant cost reduction without impacting quality or delivery of care. Based on average case number per year, a total annual cost savings of >$41,000 could be realized. Survey participants agree that standardization improves cost and patient safety, yet perceptions regarding the impact on efficiency and patient care varied by occupation.

Upper thoracic shape in children with pectus excavatum: impact on lung function.

OBJECTIVE: Pectus excavatum (PE) can present with respiratory complaints in childhood. However severity of the PE, measured by the Pectus Severity Index (PSI), correlates only modestly with reduced vital capacity (VC). We hypothesized that another upper thoracic feature, a pectus gracilis (PG) or slender chest, co-exists with PE, and impacts lung function. PATIENTS AND METHODS: We developed the Pectus Gracilis Index (PGI) based on the chest width to depth ratio at the gladiolar-manubrial sternal junction on computerized tomographic (CT) scans, and measured PGI among 316 control children 10-20 years old. PG was defined by PGI values >2 z-scores above the mean normal value. We determined the prevalence of PG in 97 children with PE and correlated PGI and PSI with VC among the 86 that performed spirometry. RESULTS: The mean and upper limit of normal for PGI averaged 2.73 and 3.55, respectively for control children. The prevalences of a PG among controls and children with PEs were 3.2% and 59%, respectively (OR = 45, P < 0.00001). Among the children with PEs, the PGI, and PSI correlated with one another (r = 0.77, P < 0.001). Both PSI and PGI significantly correlated inversely with VC. (r = -0.34, P < 0.001 and r = -0.38, P < 0.001, respectively). Importantly, PGI correlated with VC after adjusting for PSI among children with PE. (r = 0.20, P < 0.03). CONCLUSION: The upper thoracic feature of a PG is common among children with PE and contributes to reductions in VC. Assessment of the thorax, using the PGI, may improve the structure-function correlations previously described for children with PE.

Hepatic artery thrombosis and liver malignancy in pediatric liver transplantation.

BACKGROUND: Hepatic artery thrombosis (HAT) remains a significant cause of graft failure and mortality after pediatric liver transplantation. Conditions not associated with hepatic failure, such as liver tumors, may be more prone to thrombotic problems after transplant. We hypothesized that liver transplant for hepatic malignancies may be associated with increased rates of HAT in the posttransplant period. METHODS: We conducted a retrospective review of pediatric patients (age, 0-21 years) who underwent primary liver transplantation at a free-standing children's hospital from 1990 to 2009. We reviewed cause of underlying liver disease, age, sex, weight, occurrence of HAT, use of antiplatelets and anticoagulants perioperatively, as well as reintervention, retransplant, and death. RESULTS: A total of 129 children underwent 146 liver transplants, and 15 (12%) patients developed HAT. Nine liver transplants were performed for hepatic malignancy, and 4 (44%) of these patients developed HAT (relative risk, 4.85; 95% confidence interval, 1.9-12.2; P = .0015). All 4 children with hepatic malignancy and HAT required reintervention, including 3 retransplants (75%). One of these patients died. CONCLUSIONS: Hepatic artery thrombosis occurs approximately 5 times more often and appears to be more morbid in children with hepatic malignancy after transplantation. Prospective evaluation of prophylactic anticoagulation regimens in the setting of hepatic malignancy requiring transplantation is warranted.

Teratomas and ovarian lesions in children.

Ovarian pathology in children is common and the pathology can be quite diverse. The most common benign ovarian tumor in childhood is a teratoma. In this article, we discuss the origin of these germ cell tumors followed by a complete discussion of ovarian pathology.

Correlating Haller Index and cardiopulmonary disease in pectus excavatum.

BACKGROUND: The Haller Index (HI) has become standard for determining the severity of pectus excavatum. We compared patterns of cardiopulmonary dysfunction and their relationship with HI in patients with pectus excavatum. METHODS: We performed cardiopulmonary exercise testing and chest computed tomography scans on 90 patients with pectus excavatum deformities at a regional pediatric hospital. RESULTS: The median HI was 4.9 in patients with combined dysfunction, 4.4 in patients with isolated pulmonary dysfunction, 3.6 in patients with isolated cardiac dysfunction, and 3.4 in patients with normal function. HI varied significantly by disease group (P < .009). HI was significantly lower in patients with normal forced vital capacity than with abnormal forced vital capacity (P = .001). However, HI was similar in patients with normal and abnormal oxygen pulse (P = .24) or peak oxygen consumption (P = .37). CONCLUSIONS: Fifty-nine percent of patients had cardiac and/or pulmonary limitation. A HI greater than 3.6 is associated with pulmonary dysfunction, but not cardiac dysfunction.

Outcomes associated with type of intervention and timing in complex pediatric empyema.

BACKGROUND: The presence of effusion/empyema in pediatric pneumonia can increase treatment complexity by possibly requiring pleural drainage. Currently, no data support the superiority of any drainage modalities in children. METHODS: We performed a retrospective cohort study using the Pediatric Health Information System database from 2003 to 2008. RESULTS: A total of 14,936 children were hospitalized with effusion/empyema. Fifty-two percent of children were treated with antibiotics alone. Compared with patients receiving a chest tube, patients receiving antibiotics alone, thoracotomy, and video-assisted thoracoscopic surgery had a shorter length of stay, lower mortality rates, and fewer re-interventions. Delaying drainage by 1 to 3 days was associated with a lower mortality rate, and a delay of more than 7 days was associated with a higher mortality rate. CONCLUSIONS: Half of all children with effusion/empyema are treated with antibiotics alone with low morbidity and mortality. Initial video-assisted thoracoscopic surgery or thoracotomy had improved outcomes compared with other interventions. Intervention should not be delayed beyond 7 days.

Parapagus conjoined twins with unilateral mesenteric venous outflow.

This case presents a complication of attempted separation of parapagus conjoined twins, related to loss of an intact mesenteric-portal venous axis. Despite known lack of a superior mesenteric artery in the right twin, initial evaluation in the operating room suggested that separation was possible. After hepatic division, however, it became apparent that the mesenteric drainage was not separable; and the operation was aborted. Subsequently, significant growth failure and hypoglycemia were noted in the right twin. The situation was corrected by creating a shunt to reinstitute mesenteric flow to the right twin's liver and separating the twin's mesenteric drainage. One year postoperatively, both twins are independently nourishing themselves and have been free from hospitalization with stable glucoses.

Robotic lateral pancreaticojejunostomy (Puestow).

A lateral pancreaticojejunostomy (LPJ), also known as the Puestow procedure, is a complex procedure performed for chronic pancreatitis when the pancreatic duct is dilated and unable to drain properly. Traditionally, these procedures are performed with open surgery. A minimally invasive approach to the LPJ using rigid handheld nonarticulating instruments is tedious and rarely performed. In fact, there are no prior laparoscopic case reports for LPJ in children and only a small handful of cases in the adult literature. This lack of laparoscopic information may be an indication of the difficulty in performing this complex operation with nonarticulating laparoscopic instruments. The advantages of robotic surgery may help overcome these difficulties. We present the first robotic LPJ ever reported in a 14-year-old child with idiopathic chronic pancreatitis. This case demonstrates the utility of this advanced surgical technology and may lead to a new minimally invasive option for both adults and children with chronic pancreatitis requiring surgical intervention.

Application of lean methods improves surgical clinic experience.

BACKGROUND: A quality visit in high volume surgery clinics is challenging. There is variability in numbers of patients seen and care provider behavior. Documentation, regulatory and compliance issues and computerization of patient care systems may decrease clinic efficiency and throughput. We tried to reduce variability and improve patient experience. METHODS: Baseline data included: patients seen, time in exam rooms, and spent with providers, and patient satisfaction surveys. Two Rapid Process Improvement Workshops (RPIWs) were conducted to apply lean methods. 5S techniques helped standardize exam rooms. Similar data were collected at 30 days, 60 days, and 1 year. Satisfaction surveys were followed at 6 months and 1 year. RESULTS: Median pre-RPIW room time was 49 minutes. Post-RPIW times were 33 minutes at 30 days, 41 minutes at 60 days, and 42 minutes at 1 year. Face to face provider-patient time increased 30% to 61% at 30 days, 58% at 60 days, and 59% at 1 year. The median number of patients in a 4-hour clinic increased from 10 to 12. Satisfaction survey Problem Scores improved and were sustained. CONCLUSIONS: Lean methodology may be used to improve clinic efficiency as well as patient and staff's experience.

Thoracoscopic repair of congenital diaphragmatic hernia in infancy.

BACKGROUND: Minimally invasive surgical techniques, specifically the thoracoscopic approach, have been applied to congenital diaphragmatic hernia (CDH) with varying outcomes from selected centers. The aim of our study was to examine the rate of successful completion and compare outcomes between open and thoracoscopic approaches in CDH repair. METHODS: We performed a retrospective analysis of infants with CDH repair (From February 2004 to January 2008). Patients were divided into thoracoscopic and open groups, based on operative approach. We analyzed demographic, clinical, and hospitalization characteristics to compare the completion rate and outcomes in these two groups. RESULTS: Analysis of 31 infants with CDH (14 thorascocopic and 17 open) demonstrated no differences in sex (P = 0.132), age (P = 0.807), birthweight (P = 0.256), weight at operation (P = 0.647), pulmonary hypertension (P = 0.067), preoperative intensive care unit (ICU) days (P = 0.673), ventilator days (P = 0.944), or use of a patch (P = 0.999) between the groups. Seventy-nine percent of thoracoscopic operative approaches were completed successfully. There was a significant difference between the open and thoracoscopic groups with respect to estimated gestational age (39 versus 36.5 weeks; P = 0.006) and operating room time (70 versus 145 minutes; P = 0.004). The total (P = 0.662), ICU (P = 0.889), and postoperative (P = 0.619) length of stay and days on ventilator (P = 0.705), as well as days until initial enteral feeds (P = 0.092), were not significantly different between groups. There were no deaths and no evidence of recurrence, with a mean follow-up of 346 days. CONCLUSIONS: In our early experience, the thoracoscopic approach for congenital diaphragmatic hernia repair was completed in 80% of our patient population with minimal exclusion criteria. Further study, with larger sample sizes, is needed to ascertain differences in outcomes, such as length of stay and initiation of enteral feeding.

Ureteral extension in Wilms' tumor: a report from the National Wilms' Tumor Study Group (NWTSG).

BACKGROUND: Extension of Wilms' tumor into the ureter is a rare event. We reviewed the National Wilms' Tumor Study Group (NWTSG) database to define the clinical presentation, associated pathologic features, and clinical outcome of these patients. METHODS: Records of children identified to have ureteral extension of Wilms' tumor enrolled in NWTS-3, 4, and 5 were reviewed. Presenting symptoms, diagnostic studies, histopathologic findings, operation performed, and outcome were recorded. The NWTS-5 surgical data were prospectively collected as part of the quality assurance program. RESULTS: Forty-five children were identified with ureteral extension. For NWTS-5, the incidence of ureteral extension was 2%. Clinical presentations were gross hematuria in 22 patients, 2 had passage of tissue per urethra, and 1 child had a urethral mass. The remainder had nonspecific presentations. Ureteral extension was seen on preoperative imaging in 14 patients, intraoperatively in 22, and on pathologic examination in 9 patients. Hydronephrosis was noted in 12 patients, and there was nonfunction of the kidney in another 8. Laterality of the tumor was right side in 26 and left in 19. Cystoscopy was performed in 12 children. Findings included tumor seen at the ureteral orifice in 6 patients and bleeding from the orifice in one child. All patients had radical nephrectomy including partial ureterectomy. The ureteral margin was positive in 3 patients, including 2 of the 7 with separate removal of the ureteral extension. The number of patients in each clinical stage was as follows: stage I, 10; stage II, 18; stage III, 14; and stage IV, 3. The tumor extended into the proximal ureter in 23 patients, distal ureter in 13, 7 had extension into the bladder, and 1 had urethral involvement. The level of ureteral extension was not clearly noted in the 45th child. The median follow-up was 96 months. Overall, 41 of 45 patients were alive at last contact. There were 3 deaths because of tumor in patients with unfavorable histologic tumors, and 1 because of treatment toxicity in a child with favorable histologic findings. CONCLUSIONS: Ureteral extension occurs in approximately 2% of patients with Wilms' tumor. The diagnosis should be suspected in patients with gross hematuria, hydronephrosis, or nonfunctioning kidney. Cystoscopy with retrograde ureterogram may aid in preoperative diagnosis in these patients. Preoperative diagnosis is important because complete resection of the involved portion of ureter at the time of nephrectomy can avoid residual disease and the need for second surgery or radiation therapy.

Adrenocorticotropin-secreting pancreatoblastoma.

We present a 3-year-old child with Cushing's syndrome due to an ACTH-secreting metastatic pancreatoblastoma. This malignancy is a rare cause of Cushing's syndrome, particularly at pediatric age. We describe her course including the use of ketoconazole to alleviate hypercortisolemia.

Aminoglycoside-based triple-antibiotic therapy versus monotherapy for children with ruptured appendicitis.

OBJECTIVE: We conducted a retrospective cohort study to compare the use of triple therapy versus monotherapy for children and adolescents with perforated appendicitis and to determine whether there has been a transition to monotherapy within the freestanding children's hospitals that contribute to the Pediatric Health Information System database. METHODS: We used the Pediatric Health Information System database, which includes billing and discharge data for 32 children's hospitals in the United States, to examine the trend in antibiotic usage and whether the postappendectomy antibiotic regimen was associated with differences in complication-related readmissions, length of stay, or charges in a population of children and adolescents with ruptured appendicitis and discharge dates between March 1, 1999, and September 30, 2004. Pairwise regression analyses were performed to compare the most common monotherapy regimens with the triple therapy. RESULTS: A total of 8545 patients met the inclusion criteria, of whom 58%, over the entire study period, received the aminoglycoside-based triple antibiotic therapy on postoperative day 1. There was, however, a notable transition over this 6-year period, from 69% to 52% of surgeons using aminoglycoside-based combination therapy. There were no significant differences in the odds of readmission at 30 days except for the group receiving ceftriaxone, which was associated with significantly decreased odds. The subgroup receiving piperacillin/tazobactam monotherapy demonstrated significantly decreased length of stay (-0.90 days) and total hospital charges, and the group receiving cefoxitin demonstrated significantly decreased length of stay (-1.89 days), as well as decreased pharmacy and total hospital charges. CONCLUSIONS: Single-agent antibiotic therapy in the treatment of perforated appendicitis is being used with increasing frequency, is at least equal in efficacy to the traditional aminoglycoside-based combination therapy, and may offer improvements in terms of length of stay, pharmacy charges, and hospital charges.

Do antireflux operations decrease the rate of reflux-related hospitalizations in children?

OBJECTIVE: Gastroesophageal reflux disease is extremely common in the pediatric population, and antireflux procedures are performed with increasing frequency. The objective of this study was to determine whether pediatric antireflux procedures are associated with a decreased rate of reflux-related hospitalizations. METHODS: A study was conducted of pediatric patients who were undergoing antireflux procedures using data that were derived from the Washington State Comprehensive Hospital Abstract Reporting System and Vital Records. Patients were identified by a search of all records (1987-2001) for procedure codes that pertained to antireflux procedures in patients who were younger than 19 years. The number of hospitalizations for and rates of reflux-related events per patient-year before and after an antireflux procedure was calculated, and factors that were associated with higher antireflux procedure rates were examined. RESULTS: A total of 1142 patients underwent antireflux procedures. The rate of reflux-related events declined sharply with age both before and after an antireflux procedure. The cohort was divided into 3 groups on the basis of age at first antireflux procedure (<1 year, 1-3 years, or 4-19 years), and the calculations of incidence rate ratios before to after an antireflux procedure were done within the same age strata. Results suggest an overall benefit of antireflux procedures in young children. For antireflux procedures that were performed in children who were older than 4 years, the benefit is less clear. Developmental delay was significantly associated with higher rates of reflux-related events among patients who underwent an antireflux procedure after age 4. CONCLUSIONS: The rate of reflux-related events was lower after an antireflux operation for children who underwent an antireflux procedure before age 4. Older children, however, were hospitalized at equal rates before and after an antireflux procedure, and older children with developmental delay were hospitalized at greater rates after an antireflux procedure. These findings highlight the need to clarify the subjective and objective indications for antireflux procedures in infants and children.

Initial nonoperative management and delayed closure for treatment of giant omphaloceles.

PURPOSE: Traditional treatment of giant omphaloceles with silo closure has been associated with respiratory insufficiency, hemodynamic compromise, dehiscence, and inability to close the abdomen with subsequent death. To minimize such complications, initial nonoperative management with delayed closure of the defect has been used. METHODS: Between January 1981 and December 2002, 111 patients with omphaloceles were treated. Twenty-two patients with giant omphaloceles (19 containing liver) underwent initial nonoperative management consisting of silver sulfadiazine dressing changes. After pulmonary and other comorbidities stabilized, the contents were gradually reduced with a loose elastic bandage, and delayed closure was planned at 6 to 12 months. The medical records of these 22 patients were retrospectively reviewed to determine the efficacy and safety of this technique in the setting of severe associated anomalies. Those 15 patients (n = 15) from the latter 10 years were further reviewed to determine additional end points (length of hospital stay, length of intensive care unit stay, duration of mechanical ventilation, time to feed, time to closure, and type of closure). RESULTS: Of the 15 patients treated during the latter 10 years, mean gestational age and birth weight were 38 +/- 1.4 weeks and 3.1 +/- 0.57 kg, respectively. Median length of stay after birth was 20 days (range, 5-239 days). Median time to full diet was 8 days (range, 4-80 days). Four patients were discharged on oral feedings only, 7 with combination oral/gavage, and 4 with tube feedings. Pulmonary hypoplasia or pulmonary hypertension was present in 11 (50%) of 22 patients. There were 11 patients with major cardiac anomalies, 14 with a patent ductus arteriosus, and 8 with a patent foramen ovale. Three early complications (2 ruptured sacs and 1 bleeding sac) and 1 late complication (gastric necrosis) occurred in the initial nonoperative period. In addition, 4 patients were treated for line sepsis, 1 patient for acute renal insufficiency, and 1 for aspiration pneumonia. Three patients required tracheostomy and were discharged with home ventilators. There were no complications associated with the use of silver sulfadiazine. Of the 22 patients, 16 have undergone delayed repair, 2 did not require repair, 1 is awaiting repair, 2 died before closure, and 1 was lost to follow-up. Delayed closure was achieved at a median age of 14 months (range, 2-28 months) and mean weight of 8.8 +/- 3.3 kg. Four patients required implantation of mesh for definitive closure. Median postoperative length of stay was 4 days (range, 2-21 days). Postoperative complications included prolonged ileus, recurrent ventral hernia, and prolonged intubation. Overall mortality rate was 9.1%. One death occurred after diaphragmatic hernia repair, and 1 death was from overwhelming sepsis in the patient with a late gastric perforation. CONCLUSION: The use of silver sulfadiazine dressing changes for initial nonoperative management of giant omphaloceles is a safe and effective bridge to delayed closure. We recommend this method as initial nonoperative management given the high incidence of associated cardiopulmonary malformations because it may facilitate enteral feeding, minimize respiratory compromise, and reduce morbidity and mortality.