Newborn circumcision outcomes: are parents satisfied with the results?

BACKGROUND/PURPOSE: Although physician-reported complications following circumcision are very low, parental satisfaction is not well documented. This study examined parental opinions and compared these with those of the medical professional. METHODS: Physicians independently assessed complications and cosmetic outcome following the circumcision. Six weeks post-circumcision, parental report of complications, cosmetic outcome, and overall satisfaction were assessed. RESULTS: Newborn infants (n = 710) were prospectively recruited and underwent either a Gomco [n = 552 (78 %)] or Plastibell(®) [n = 158 (22 %)] circumcision. Physician assessed complication rates were equivalent (Gomco 4.3 % versus Plastibell 5.1 %; p = 0.67), however, parental assessment found a much lower complication rate for Gomco 5.6 % versus Plastibell 12.0 % (p < 0.001). There was no difference between who performed the procedure nor between the techniques in regards to parental rating of overall satisfaction (excellent/good: Gomco 96.9 % versus Plastibell 95.6 %, p = 0.45). However, perceived post-operative pain as scored by parents was significantly higher in patients undergoing Plastibell procedure (6.4 % too much pain) versus Gomco (2.7 %; p = 0.05). Gomco accounted for 72.7 % of parental cosmetically unsatisfactory cases. CONCLUSIONS: Clinicians and parents differed considerably in terms of opinion of cosmetic outcome and occurrence of post-operative complications. This study emphasizes the need for clinicians to better understand and address parental concerns before and after circumcision.

Another dimension to survival: predicting outcomes with fetal MRI versus prenatal ultrasound in patients with congenital diaphragmatic hernia.

PURPOSE: A major determinant of survival in patients with congenital diaphragmatic hernia (CDH) is severity of pulmonary hypoplasia. This study addresses the comparative effectiveness of prenatal methods of lung assessment in predicting mortality, extracorporeal membrane oxygenation (ECMO), and ventilator dependency. METHODS: We retrospectively reviewed all patients born with isolated CDH between 2004 and 2008. Lung-to-head ratio (LHR) and observed-to-expected LHR (OELHR) were obtained from prenatal ultrasounds. Percent-predicted lung volume (PPLV) was obtained from fetal MRI (fMRI). Postnatal data included in-hospital mortality, need for ECMO, and ventilator dependency at day-of-life 30. RESULTS: Thirty-seven patients underwent 81 prenatal ultrasounds, while 26 of this sub-cohort underwent fMRI. Gestational age during imaging study was associated with LHR (p=0.02), but not OELHR (p=0.12) or PPLV (p=0.72). PPLV, min-LHR, and min-OELHR were each associated with mortality (p=0.03, p=0.02, p=0.01), ECMO (p<0.01, p<0.01, p=0.03), and ventilator dependency (p<0.01, p<0.01, p=0.02). For each outcome, PPLV was a more discriminative measure, based on Akaike's information criterion. Using longitudinal analysis techniques for patients with multiple ultrasounds, OELHR remained associated with mortality (p=0.04), ECMO (p=0.03), and ventilator dependency (p=0.02), while LHR was associated with ECMO (p=0.01) and ventilator dependency (p=0.02) but not mortality (p=0.06). CONCLUSION: When assessing fetuses with CDH, OELHR and PPLV may be most helpful for counseling regarding postnatal outcomes.

Contemporary outcomes in infants with congenital heart disease and bochdalek diaphragmatic hernia.

BACKGROUND: Fifteen percent of infants with congenital diaphragmatic hernia (CDH) are born with a coexisting cardiac anomaly. The purpose of this study was to evaluate contemporary outcomes in this patient population and to identify potential risk factors for in-hospital mortality. METHODS: Data from all CDH neonates with congenital heart disease managed at a single pediatric tertiary care referral center between 1997 and 2011 were retrospectively analyzed. RESULTS: Forty (18%) of 216 CDH patients had a cardiac anomaly. This group was associated with a significant decrease in overall survival when compared with patients without cardiac anomaly (55% versus 81%; p = 0.001). There was no association between type of cardiac anomaly and mortality based on risk stratification according to the Risk Adjustment for Congenital Heart Surgery and The Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery scoring systems (p = 0.86 and p = 0.87, respectively). Birth weight was similarly no different between survivors and nonsurvivors (2.8 ± 0.6 kg versus 2.8 ± 0.9 kg, respectively; p = 0.98). There was a trend toward increased extracorporeal membrane oxygenation use among nonsurvivors (p = 0.13). Infants with hemodynamic stability enabling subsequent cardiac repair were associated with lower mortality (p = 0.04). Survivors had a wide spectrum of long-term morbidity, but most had some evidence of neurodevelopmental impairment. CONCLUSIONS: This large single-institution series suggests that the overall prognosis of infants with concomitant CDH and congenital heart disease can be quite variable, regardless of the type of heart anomaly. Hemodynamic instability and need for extracorporeal membrane oxygenation correlate with higher mortality. Although some long-term survivors have excellent outcomes, most suffer from chronic, long-term morbidities.

Fetal lung lesions: can we start to breathe easier?

OBJECTIVE: The purpose of this study was to develop a simple and accurate approach for risk stratification of fetal lung lesions that are associated with respiratory compromise at birth. STUDY DESIGN: We conducted a retrospective review of 64 prenatal lung lesions that were managed at a single fetal care referral center (2001-2011). Sonographic data were analyzed and correlated with perinatal outcomes. RESULTS: Hydrops occurred in only 4 cases (6.3%). Among fetuses without hydrops, the congenital pulmonary airway malformation volume ratio (CVR) was the only variable that was associated significantly with respiratory compromise and the need for lung resection at birth (P < .01). Based on a maximum CVR >1.0, the sensitivity, specificity, positive predictive value, and negative predictive value for respiratory morbidity were 90%, 93%, 75%, and 98%, respectively. CONCLUSION: Nonhydropic fetuses with a maximum CVR >1.0 are a subgroup of patients who are at increased risk for respiratory morbidity and the need for surgical intervention. These patients should be delivered at a tertiary care center with pediatric surgery expertise to ensure optimal clinical outcomes.

Characterization of central venous catheter-associated deep venous thrombosis in infants.

PURPOSE: Deep venous thrombosis (DVT) is a frequent complication in infants with central venous catheters (CVCs). We performed this study to identify risk factors and risk-reduction strategies of CVC-associated DVT in infants. METHODS: Infants younger than 1 year who had a CVC placed at our center from 2005 to 2009 were reviewed. Patients with ultrasonically diagnosed DVT were compared to those without radiographic evidence. RESULTS: Of 333 patients, 47% (155/333) had femoral, 33% (111/333) had jugular, and 19% (64/333) had subclavian CVCs. Deep venous thromboses occurred in 18% (60/333) of patients. Sixty percent (36/60) of DVTs were in femoral veins. Femoral CVCs were associated with greater DVT rates (27%; 42/155) than jugular (11%; 12/111) or subclavian CVCs (9%; 6/64; P < .01). There was a 16% DVT rate in those with saphenofemoral Broviac CVCs vs 83% (20/24) in those with percutaneous femoral lines (P < .01). Multilumen CVCs had higher DVT rates than did single-lumen CVCs (54% vs 6%, P < .01), and mean catheter days before DVT diagnosis was shorter for percutaneous lines than Broviacs (13 ± 17 days vs 30 ± 37 days, P = .02). Patients with +DVT had longer length of stay (86 ± 88 days vs 48 ± 48 days, P < .01) and higher percentage of intensive care unit admission (82% vs 70%, P = .02). CONCLUSIONS: Deep venous thrombosis reduction strategies in infants with CVCs include avoiding percutaneous femoral and multilumen CVCs, screening percutaneous lines, and early catheter removal.

Restorative proctocolectomy without diverting ileostomy in children with ulcerative colitis.

PURPOSE: The safety of performing a restorative proctocolectomy (RP) and J-pouch ileoanal anastomosis (IPAA) without diverting ileostomy for children with ulcerative colitis (UC) is a subject of extensive debate. Our goal was to examine pediatric outcomes of RP and IPAA without ileostomy. METHODS: We performed a single-institution review of UC patients who had RP and IPAA with (+Ostomy) or without (-Ostomy) diverting ileostomy from 2002 to 2010. Surgeon and patient preference determined ileostomy decision. The study included 50 patients (28 +Ostomy, 22 -Ostomy). RESULTS: Preoperative demographics were similar between 2 groups in age (13.5 ± 3.5 years -Ostomy, 14.3 ± 3 years +Ostomy), serum albumin (3.6 ± 0.7 -Ostomy, 3.6 ± 0.7 +Ostomy), body mass index (20.8 ± 6.9 -Ostomy, 21.3 ± 8.6 +Ostomy), and daily corticosteroid dose (22.4 ± 17.7 mg -Ostomy, 23.5 ± 13.7 mg +Ostomy). Operating time was less in -Ostomy with mean times of 6:22 ± 2:04 vs 9:07 ± 2:57. The -Ostomy group required fewer ileoanal anastomotic dilations per patient (0.4 ± 0.8 vs 1.4 ± 1.9). Functional outcomes were not significantly different regarding pouchitis episodes per patient (0.6 ± 1.1 -Ostomy, 0.6 ± 1.1 +Ostomy), daily bowel movements (5.5 ± 1.9 -Ostomy, 6.7 ± 4.0 +Ostomy), and daily postoperative loperamide dose (8.4 ± 4.3 mg -Ostomy, 6.8 ± 4.0 mg +Ostomy). CONCLUSION: Short- and long-term outcomes can be equivalent in patients with and without diverting ileostomy, but questions remain regarding patient selection and quality of life impact.

Acute kidney injury in congenital diaphragmatic hernia requiring extracorporeal life support: an insidious problem.

PURPOSE: Patients with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS) are at increased risk for acute kidney injury (AKI). We hypothesized that AKI would be associated with increased mortality. We further hypothesized that vasopressor requirement, nephrotoxic medications, and infections would be associated with AKI. METHODS: We performed a retrospective chart review in all patients with CDH requiring ECLS from 1999 to 2009 (n = 68). Patient variables that could potentiate renal failure were collected. We used a rise in creatinine from baseline by the RIFLE (risk, 1.5×; injury, 2×; failure, 3×; loss; and end-stage renal disease) criteria to define AKI. Statistical analysis was performed via SPSS (SPSS, Chicago, IL) using Student t test and χ(2) analysis, with P < .05 being considered significant. RESULTS: Survival to hospital discharge was 37 (54.4%) of 68. Acute kidney injury was identified in 48 (71%) of 68 patients, with 15 (22% of all patients) qualifying as injury and 33 (49% of all patients) qualifying as failure by the RIFLE criteria. Patients who qualified as failure by the RIFLE criteria had a significant decrease in survival (27.3% with failure vs 80% without failure; P = .001). Patients who qualified as failure also had increased length of ECLS (314 ± 145 vs 197 ± 115 hours; P = .001) and decreased ventilator-free days in the first 60 days (1.39 ± 5.3 vs 20.17 ± 17.4 days; P = .001). There was no significant difference in survival when patients qualified as risk or injury. CONCLUSIONS: This is the first report using a systematic definition of AKI in patients with CDH on ECLS. There is a high incidence of AKI in these patients, and when it progresses to failure, it is associated with higher mortality, increased ECLS duration, and increased ventilator days. This highlights the importance of recognizing AKI in patients with CDH requiring ECLS and the potential benefit of preventing progression of AKI or early intervention.

Vertical expandable prosthetic titanium rib device insertion: does it improve pulmonary function?

PURPOSE: Vertical expandable prosthetic titanium rib (VEPTR) insertion and expansion has been advocated to increase thoracic volume and pulmonary function in patients with thoracic insufficiency syndrome. We reviewed our experience with VEPTR implantation to determine if lung function and growth is augmented, to determine the children's functional status, and if the scoliosis is controlled. METHODS: From 2006 to 2010, 29 insertions and 57 expansions were performed in 26 patients at our institution. Demographic data were reviewed in conjunction with complications, scoliosis angles, pulmonary function tests (PFTs), and computed tomography-guided 3D reconstructions to determine lung volumes; and quality of life scores were determined using a modified Scoliosis Research Society (SRS) questionnaire preoperatively and postoperatively. The groups were also stratified by age (because of lung growth potential), disease (congenital or infantile scoliosis, Jeune syndrome, neuromuscular, other structural thoracic disorders), and sex. Analyses using SPSS (SPSS, Chicago, Ill) were performed with P < .05 considered significant. RESULTS: Each patient underwent 3.03 ± 1.8 surgeries, spending 0.97 ± 1.8 days in the intensive care unit and 4.41 ± 6 days in the hospital for each procedure. Mean age was 90.7 ± 41 months. Of the 36 complications, most were because of infection (12), half requiring operative repair (hardware removal). The average PFT percent predicted values for forced expiratory volume in 1 second, forced vital capacity, and RV were 54.6 ± 22, 58.1 ± 24, and 145.3 ± 112, respectively, preoperatively and 51.8 ± 20, 55.9 ± 20, and 105.6 ± 31, respectively, postoperatively. The lung volumes measured by computed tomography when corrected for age do not increase significantly postoperatively. The mean Cobb measurement for the preoperative major curves was 64.7° and postoperatively was 46.1° for those curves measured preoperatively, for a 29% curve improvement. All postoperative curves had a mean of 56.4° and 58.1° at final follow-up, a 3% curve increase. The SRS scores for patients remained unchanged and no statistical difference was seen from preoperative to postoperative values. No statistically significant difference was seen in complications, PFT (forced expiratory volume in 1 second, forced vital capacity, RV), lung volumes, scoliosis angles, and SRS scores between sex, age, and disease categories. CONCLUSION: There was mild improvement in scoliosis angles but no improvement in lung function and volume. Scoliosis Research Society scores indicate that the children have near normal function both before and after VEPTR placement. Pulmonary function, lung volume, and patient subjective assessments did not increase dramatically after VEPTR placement, although scoliosis angles improved.

Gastrostomy tube placement in infants and children: is there a preferred technique?

PURPOSE: Advances in percutaneous endoscopic gastrostomy (PEG) and laparoscopic (LAP) techniques, including LAP-assisted PEG, offer alternatives to the standard open gastrostomy technique. This study compares the outcomes of the PEG and LAP techniques. METHODS: All gastrostomy tube placements were reviewed at our institution from January 2004 to October 2008. Demographic, procedural, and outcome data were collected. Univariate and logistic regression statistical analysis was performed with SPSS (SPSS, Chicago, IL), and P < or = .05 considered significant. RESULTS: Of 238 gastrostomy tubes placed, 134 were PEG (56.3%) and 104 were LAP (43.7%). Most tubes were inserted for failure to thrive (74.4%) and feeding difficulties (52.1%). Patient weight and age were increased and operative time decreased for PEG compared with other methods. Percutaneous endoscopic gastrostomy patients also had a statistically higher number of postoperative complications, requiring a return trip to the operating room (P = .02). CONCLUSION: Minimally invasive PEG and LAP techniques have supplanted the open technique for most patients. Operative time for PEG placement is shorter than other methods, and patients chosen for the PEG method of placement are older and of greater weight. However, there were significant and more serious postoperative complications requiring a second operation in the PEG group when compared with the LAP group.

Long-term functional outcome and quality of life in patients with high imperforate anus.

PURPOSE: Anorectal malformations (ARMs) are associated with a large number of functional sequale that may affect a child's long-term quality of life (QOL). The purposes of this study were to better quantify patient functional stooling outcome and to identify how these outcomes related to the QOL in patients with high imperforate anus. METHODS: Forty-eight patients from 2 children's hospitals underwent scoring of stooling after 4 years of life. Scoring consisted of a 13-item questionnaire to assess long-term stooling habits (score range: 0-30, worst to best). These results were then correlated with a QOL survey as judged by a parent or guardian. RESULT: Mean (SD) age at survey was 6.5 (1.6) years. Comparison of QOL and clinical scoring showed no signficant difference between the 2 institutions (P > .05). There was a direct correlation between the QOL and stooling score (Pearson r(2) = 0.827; beta coefficient = 24.7, P < .001). Interestingly, functional stooling scores worsened with increasing age (Pearson r(2) = 0.318, P = .02). Patients with associated congenital anomalies had a high rate of poor QOL (44% in poor range; P = .001). Stooling scores decreased significantly with increasing severity/complexity of the ARM (P = .001). CONCLUSION: A large number of children experience functional stooling problems, and these were directly associated with poor QOL. In contrast to previous perceptions, our study showed that stooling patterns are perceived to worsen with age. This suggests that children with ARMs need long-term follow-up and counseling.

A prospective, randomized comparison of skin adhesive and subcuticular suture for closure of pediatric hernia incisions: cost and cosmetic considerations.

PURPOSE: In this study, we compared the skin adhesive 2-octylcyanoacrylate to subcuticular suture for closure of pediatric inguinal hernia incisions to determine if skin adhesive improves wound cosmesis, shortens skin closure time, and lowers operative costs. METHODS: We prospectively randomized 134 children undergoing inguinal herniorrhaphy at our institution to have skin closure with either skin adhesive (n = 64) or subcuticular closure (n = 70). Data collected included age, sex, weight, type of operation, total operative time, and skin closure time. Digital photographs of healing incisions were taken at the 6-week postoperative visit. The operating surgeon assessed cosmetic outcome of incisions using a previously validated visual analog scale, as well as an ordinate scale. A blinded assessment of cosmetic outcome was then performed by an independent surgeon comparing these photographs to the visual analog scale. Operating room time and resource use (ie, costs) relative to the skin closure were assessed. Comparisons between groups were done using Student's t tests and chi(2) tests. RESULTS: Children enrolled in the study had a mean +/- SE age of 3.7 +/- 0.3 years and weighed 16 +/- 0.8 kg. Patients were predominantly male (82%). Patients underwent 1 of 3 types of open hernia repair as follows: unilateral herniorrhaphy without peritoneoscopy (n = 41; 31%), unilateral herniorrhaphy with peritoneoscopy (n = 55; 41%), and bilateral herniorrhaphy (n = 38; 28%). Skin closure time was significantly shorter in the skin adhesive group (adhesive = 1.4 +/- 0.8 minutes vs suture = 2.4 +/- 1.1 minutes; P = .001). Mean wound cosmesis scores based on the visual analog scale were similar between groups (adhesive = 78 +/- 21; suture=78 +/- 18; P = .50). Material costs related to herniorrhaphy were higher for skin adhesive (adhesive = $22.63 vs suture = $11.70; P < .001), whereas operating room time costs for adhesive skin closure were lower (adhesive = $9.33 +/- 5.33 vs suture = $16.00 +/- 7.33; P < .001). Except for a 7% incidence of erythema in both groups, there were no complications encountered. CONCLUSIONS: There is no difference in cosmetic outcome between skin adhesive and suture closure in pediatric inguinal herniorrhaphy. Material costs are increased because of the high cost of adhesive relative to suture. This is partially offset, however, by the cost savings from reduction in operating room time.

Chylothorax after congenital diaphragmatic hernia repair.

PURPOSE: Chylothorax after congenital diaphragmatic hernia (CDH) repair contributes significantly to morbidity. Our aim was to identify factors contributing to chylothorax and effective treatment strategies. METHODS: We reviewed 171 patients with CDH from 1997 to 2008 and analyzed hernia characteristics, extracorporeal membrane oxygenation (ECMO) use, operative details, and treatment approaches for chylothorax. RESULTS: Ten (7%) patients developed chylothorax; all were left sided. Using univariate analysis, prenatal diagnosis, ECMO use, and patch repair were associated with development of chylothorax. Logistic regression analysis showed that patch repair was the only variable predictive of chylothorax (P = .028; confidence interval, 0.032-0.823). Although survival was not affected, patients with chylothorax had a significant increase in ventilator days and length of stay (t = 3.57; P = .000; t = 2.74; P = .007). All received thoracostomy and total parenteral nutrition. Six patients received octreotide, 5 of whom required pleurectomy because of failed medical management; the remaining patient died of overwhelming sepsis. CONCLUSIONS: The incidence of chylothorax at our institution was relatively low. Patch repair was associated with the formation of chylothorax. Morbidity was substantial, but survival was not significantly affected. Total parenteral nutrition and thoracostomy were appropriate initial treatments. Octreotide was not an effective adjunct. Refractory cases were successfully treated with pleurectomy.

Congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: does timing of repair matter?

PURPOSE: Severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) is associated with high mortality. Timing of CDH repair relative to ECMO therapy remains controversial. Our hypothesis was that survival would significantly differ between those who underwent repair during ECMO and those who underwent repair after ECMO therapy. METHODS: We examined deidentified data from the CDH study group (CDHSG) registry from 1995 to 2005 on patients who underwent repair and ECMO therapy (n = 636). We used Cox regression analysis to assess differences in survival between those who underwent repair during and after ECMO. RESULTS: Five covariates were significantly associated with mortality as follows: timing of repair relative to ECMO (P = .03), defect side (P = .01), ECMO run length (P < .01), need for patch repair (P = .03), birth weight (P < .01), and Apgar score at 5 minutes (P = .03). Birth year, inborn vs transfer status, diaphragmatic agenesis, age at repair, and presence of cardiac or chromosomal abnormalities were not associated with survival. Repair after ECMO therapy was associated with increased survival relative to repair on ECMO (hazard ratio, 1.407; P = .03). CONCLUSION: These data suggest that CDH repair after ECMO therapy is associated with improved survival compared to repair on ECMO, despite controlling for factors associated with the severity of CDH.

Right-sided congenital diaphragmatic hernia: high utilization of extracorporeal membrane oxygenation and high survival.

PURPOSE: The purpose of the study was to assess the treatment strategies and outcome of right-sided congenital diaphragmatic hernia (R-CDH), particularly extracorporeal membrane oxygenation (ECMO). METHODS: We reviewed the cases of 42 patients treated for R-CDH at our institution from 1991 to 2006. We gathered demographic information, documented ECMO use and the type of surgical repair, and compared outcomes with predicted survival as calculated by the CDH Study Group's equation. RESULTS: Of the 35 patients included in our statistical analysis (7 were excluded), 12 (34%) were born at our institution, all of whom were prenatally diagnosed with R-CDH. Nineteen patients (54%) required ECMO therapy. Extracorporeal membrane oxygenation was initiated after repair of the R-CDH in 2 patients (11%). Of those patients who went on ECMO before repair, 4 patients (21%) were repaired on ECMO, 9 patients (47%) underwent repair after ECMO, and 4 patients (21%) underwent ECMO but died before their R-CDH could be repaired. Primary repair of the diaphragm was possible in 15 cases (56%), and primary closure of the abdominal incision was possible in 15 of the 23 open repairs (65%). The mean predicted survival for all 35 patients was 63%, whereas 28 (80%) actually survived. Logistic regression showed a significant association between the presence of cardiac defects and mortality (odds ratio = 0.008, P = .014). CONCLUSIONS: Our data suggest that patients with R-CDH have high ECMO utilization and may experience greater relative benefit from ECMO as evidenced by their higher-than-expected overall survival. Extracorporeal membrane oxygenation may be found to have a distinctive role in managing R-CDH. More high-powered series are needed to elucidate differences between R-CDH and left-sided CDH that may dictate alternate forms of management.

Risk stratification in gastroschisis: can prenatal evaluation or early postnatal factors predict outcome?

PURPOSE: The prenatal or postnatal factors that predict complex gastroschisis in patients (atresia, volvulus, necrotic bowel and bowel perforation) remain controversial. We evaluated the prognostic value of prenatal ultrasonographic parameters and early postnatal factors in predicting clinical outcomes. METHODS: We analyzed maternal and neonatal records of 46 gastroschisis patients treated from 1998 to 2007. Information regarding demographics, prenatal ultrasound data when available, intrapartum and postnatal course was abstracted from medical records. Outcome variables included survival, ventilator days, TPN days, time to full enteral feeds, complications and length of stay. Univariate or multivariate analysis was used, with P < 0.05 considered as significant. RESULT: A total of 75% of complex patients were categorized within 1 week of life. Interestingly, prenatal bowel dilation (>17 mm) and thickness (>3 mm) did not correlate with outcome or risk stratification into simple versus complex (P < 0.05). Complex patients had increased morbidity compared to simple patients (sepsis 58 versus 18%; P = 0.021, NEC 42 versus 9%; P = 0.020, short bowel syndrome 58 versus 3%; P = 0.0001, ventilator days 24 versus 10; P = 0.021; TPN days 178 versus 38; P = 0.0001 and days to full feeds 171 versus 31; P = 0.0001; and length of stay 90 versus 39 days, P = 0.0001). CONCLUSIONS: Prenatal bowel wall dilation and/or thickness did not predict complex patients or adverse outcome. Complex gastroschisis patients can be identified postnatally and have substantial morbidity.

Salvaging the severe congenital diaphragmatic hernia patient: is a silo the solution?

BACKGROUND: Infants with severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) have a high morbidity and mortality. We hypothesized that placement of an abdominal wall silo and staged abdominal wall closure may reduce problems associated with decreased abdominal domain in CDH. METHODS: We performed a retrospective review and identified 7 CDH patients requiring ECMO who had a silastic abdominal wall silo between 2003 and 2006. Variables analyzed included survival, ECMO duration, duration of silo, time to discharge, and long-term outcome. RESULTS: Predicted mean survival for the entire cohort using the published CDH Study Group equation was 47% (range, 9%-86%). All 7 patients (100%) survived. Extracorporeal membrane oxygenation duration averaged 15 days (range, 5-19 days). Four of the patients (58%) were repaired with a silo on ECMO, and 3 (42%) had their repair after ECMO. The abdominal wall defect was closed at a mean of 21 days (range, 4-41 days). Hospital stay after silo placement averaged 54 days (range, 20-170 days) with no infections or wound complications. CONCLUSIONS: Abdominal wall silo placement in infants with CDH requiring ECMO appears to be an effective strategy for decreased abdominal domain. Further studies are warranted to determine the efficacy of such a strategy for these high-risk CDH patients.

Are the long-term results of the transanal pull-through equal to those of the transabdominal pull-through? A comparison of the 2 approaches for Hirschsprung disease.

PURPOSE: The transanal endorectal pull-through (TERPT) is becoming the most popular procedure in the treatment of Hirschsprung disease (HD), but overstretching of the anal sphincters remains a critical issue that may impact the continence. This study examined the long-term outcome of TERPT versus conventional transabdominal (ABD) pull-through for HD. METHODS: Records of 41 patients more than 3 years old who underwent a pull-through for HD (TERPT, n = 20; ABD, n = 21) were reviewed, and their families were thoroughly interviewed and scored via a 15-item post-pull-through long-term outcome questionnaire. Patients were operated on between the years 1995 and 2003. During this time, our group transitioned from the ABD to the TERPT technique. Total scoring ranged from 0 to 40: 0 to 10, excellent; 11 to 20 good; 21 to 30 fair; 31 to 40 poor. A 2-tailed Student t test, analysis of covariance, as well as logistic and linear regression were used to analyze the collected data with confidence interval higher than 95%. RESULTS: Overall scores were similar. However, continence score was significantly better in the ABD group, and the stool pattern score was better in the TERPT group. A significant difference in age at interview between the 2 groups was noted; we therefore reanalyzed the data controlling for age, and this showed that age did not significantly affect the long-term scoring outcome between groups. CONCLUSION: Our long-term study showed significantly better (2-fold) results regarding the continence score for the abdominal approach compared with the transanal pull-through. The stool pattern and enterocolitis scores were somewhat better for the TERPT group. These findings raise an important issue about the current surgical management of HD; however, more cases will need to be studied before a definitive conclusion can be drawn.

Comparison of Karydakis versus midline excision for treatment of pilonidal sinus disease.

Pilonidal sinus disease is associated with a high rate of recurrence and complications. The Karydakis (KAR) method, whereby an asymmetric subcutaneous flap obliterates the anal crease, has been shown to be effective in adults. The goal of this study is to assess the efficacy of the KAR procedure in the operative treatment of children with pilonidal sinus disease compared to those treated via a midline excision (ME). Sixty-eight cases of pediatric pilonidal sinus excision were reviewed over the past 10 years. Data abstracted included surgical approach, complication rate and recurrence rate. Student's t-test or the Chi square test was used for statistical analysis, with P < 0.05 being considered significant. An ME was performed in 44 patients; the KAR method was used in 24 patients. Mean age at diagnosis was 14.4 +/- 4.2 years for the ME group compared to 15.7 +/- 4.3 years for the KAR patients (P = 0.18). Mean operative time was significantly longer with the KAR method (58.7 +/- 25.6 min) compared to 46.3 +/- 18.6 for the primary ME (P = 0.04). Despite the increased operative dissection, there was no difference (P = 0.42) in early post-operative complication rates between groups (25% in the KAR group compared to 34.8% in the ME group). Initial drainage of an abscess had no significant effect upon the recurrence/complication rate in either group. Recurrence rate alone was lower in patients operated on via the KAR approach 0% versus 11.0% using the ME (P = 0.153). Recurrence and complication rates were lower for those patients with a pilonidal sinus treated by the KAR method compared to the ME, but the results did not reach significance. In conclusion, this study does show a potential benefit for children treated with the KAR method for pilonidal sinus. This study mimics the data obtained in adult patients and suggests that a larger study is likely to achieve significance.

Failure of cholecystokinin-octapeptide to prevent TPN-associated gallstone disease.

PURPOSE: Gallstone formation is a common problem in neonates on prolonged courses of total parenteral nutrition (TPN). The authors hypothesized that the use of cholecystokinin-octapeptide (CCK), given at the time of TPN administration, would prevent gallstone formation in a high-risk group of patients with TPN. METHODS: A prospective, randomized, blinded, controlled trial of neonates who were on a prolonged course of TPN for prematurity (25 infants), necrotizing enterocolitis (NEC, 8 infants), or abdominal surgery (5 infants) were selected randomly to receive CCK vs placebo. Patients remained on the study until taking more than 50% of energy enterally. Children were recalled between 2 and 4 years after completing TPN for ultrasonographic examination of their hepatobiliary tree. RESULTS: Neonates (38 studied) required a mean (+/-SD) of 33 +/- 16 days of TPN. Cholelithiasis was detected in 4 (10%) infants. Cholecystokinin-octapeptide was not effective in preventing the formation of gallstones (3 stones in infants receiving CCK, P = .51). Diagnosis (P = .56), birth weight (P = .54), gestational age (P = .18), and duration of TPN (P = .53) did not correlate with gallstone formation. To address the management of these stones, all 4 were placed on a prolonged course of ursodeoxycholic acid (mean duration, 11.6 +/- 5.4 months). Two additional infants (not in the original study) with TPN-associated gallstone disease were also given a trial of ursodeoxycholic acid. Serial ultrasounds were performed every 6 months. No patient achieved any degree of stone dissolution. One patient underwent cholecystectomy for symptomatology. CONCLUSIONS: Total parenteral nutrition-associated gallstones were detected in 10% of children, and most are nonsymptomatic. Cholecystokinin-octapeptide prophylaxis was not effective in preventing TPN-associated gallstones. In addition, the use of ursodeoxycholic acid did not dissolve gallstones, once identified. Future methods will be needed to address the prevention and treatment of these stones.