Implication of folate deficiency in CYP2U1 loss of function.

Hereditary spastic paraplegias are heterogeneous neurodegenerative disorders. Understanding of their pathogenic mechanisms remains sparse, and therapeutic options are lacking. We characterized a mouse model lacking the Cyp2u1 gene, loss of which is known to be involved in a complex form of these diseases in humans. We showed that this model partially recapitulated the clinical and biochemical phenotypes of patients. Using electron microscopy, lipidomic, and proteomic studies, we identified vitamin B2 as a substrate of the CYP2U1 enzyme, as well as coenzyme Q, neopterin, and IFN-α levels as putative biomarkers in mice and fluids obtained from the largest series of CYP2U1-mutated patients reported so far. We also confirmed brain calcifications as a potential biomarker in patients. Our results suggest that CYP2U1 deficiency disrupts mitochondrial function and impacts proper neurodevelopment, which could be prevented by folate supplementation in our mouse model, followed by a neurodegenerative process altering multiple neuronal and extraneuronal tissues.

Brimapitide Reduced Neuronal Stress Markers and Cognitive Deficits in 5XFAD Transgenic Mice.

Alzheimer's disease (AD) is characterized by accumulations of amyloid-ß (Aß42) and hyperphosphorylated tau proteins, associated with neuroinflammation, synaptic loss, and neuronal death. Several studies indicate that c-Jun N-terminal kinase (JNK) is implicated in the pathological features of AD. We have investigated in 5XFAD mice, the therapeutic effects of Brimapitide, a JNK-specific inhibitory peptide previously tested with higher concentrations in another AD model (TgCRND8). Three-month-old 5XFAD and wild-type littermate mice were treated by intravenous injections of low doses (10 mg/kg) of Brimapitide every 3 weeks, for 3 or 6 months (n = 6-9 per group). Cognitive deficits and brain lesions were assessed using Y-maze, fear-conditioning test, and histological and biochemical methods. Chronic treatment of Brimapitide for 3 months resulted in a reduction of Aß plaque burden in the cortex of 5XFAD treated mice. After 6 months of treatment, cognitive deficits were reduced but also a significant reduction of cell death markers and the pro-inflammatory IL-1ß cytokine in treated mice were detected. The Aß plaque burden was not anymore modified by the 6 months of treatment. In addition to modulating cognition and amyloid plaque accumulation, depending on the treatment duration, Brimapitide seems experimentally to reduce neuronal stress in 5XFAD mice.

SCAMP 3 is a novel regulator of endosomal morphology and composition.

Secretory Carrier Membrane Proteins (SCAMPs) are transmembrane proteins that function in the plasma membrane, endosomes, and trans-Golgi network. Here we show that SCAMP 3 is a novel regulator of endosomal morphology and composition. Under certain nutrient-starved conditions, SCAMP 3 concentrates in enlarged early endosomes. The enlarged contain ubiquitylated and non-ubiquitylated SCAMP 3 as well as other SCAMPs, EEA1, and the ESCRT-0 protein Hrs. We demonstrate that SCAMP 3 is sufficient to recruit Hrs to the enlarged endosomes. Taken together, our results suggest a novel role for SCAMP 3 in modifying endosome structure through interactions that involve its ubiquitylation and ESCRT proteins.

Optimizing fluid resuscitation in hypertrophic pyloric stenosis.

BACKGROUND: Hypertrophic pyloric stenosis (HPS) is the most common diagnosis requiring surgery in infants. Electrolytes are used as a marker of resuscitation for these patients prior to general anesthesia induction. Often multiple fluid boluses and electrolyte panels are needed, delaying operative intervention. We have attempted to predict the amount of IV fluid boluses needed for electrolyte correction based on initial values. METHODS: A single center retrospective review of all patients diagnosed with HPS from 2008 through 2014 was performed. Abnormal electrolytes were defined as chloride <100mmol/L, bicarbonate ≥30mmol/L or potassium >5.2 or <3.1mmol/L. Patients with abnormal electrolytes were resuscitated with 20ml/kg saline boluses and continuous fluids at 1.5 times maintenance rate. RESULTS: During the study period 542 patients were identified with HPS. Of the 505 who were analyzed 202 patients had electrolyte abnormalities requiring IV fluid resuscitation above maintenance, and 303 patients had normal electrolytes at time of diagnosis. Weight on presentation was significantly lower in the patients with abnormal electrolytes (3.8 vs 4.1kg, p<0.01). Length of stay was significantly longer in the patients with electrolyte abnormalities, 2.6 vs 1.9days (p<0.01). Fluid given was higher over the entire hospital stay for patients with abnormal electrolytes (106 vs 91ml/kg/d, p<0.01). The number of electrolyte panels drawn was significantly higher in patients with initial electrolyte abnormalities, 2.8 vs 1.3 (p<0.01). Chloride was the most sensitive and specific indicator of the need for multiple saline boluses. Using an ROC curve, parameters of initial Cl(-)80mmol/L and the need for 3 or more boluses AUC was 0.71. Modifying the parameters to initial Cl(-) ≤97mmol/L and 2 boluses AUC was 0.65. A patient with an initial Cl(-)85 will need three 20ml/kg boluses 73% (95% CI 52-88%) of the time. A patient with an initial Cl(-) ≤97 will need two 20ml/kg boluses at a rate of 73% (95% CI 64-80%). CONCLUSION: Children with electrolyte abnormalities at time of diagnosis of HPS have a longer length of stay; require more fluid resuscitation and more lab draws. This study reveals high sensitivity and specificity of presenting chloride in determining the need for multiple boluses. We recommend the administration of two 20ml/kg saline boluses separated by an hour prior to rechecking labs in patients with initial Cl(-) value ≤97mmol/L. If the presenting Cl(-) <85 three boluses of 20ml/kg of saline separated by an hour are recommended. If implemented these modifications have potential to save time by not delaying care for extraneous lab results and money in the form of fewer lab draws.

Inflammatory myofibroblastic tumors in children.

BACKGROUND: Inflammatory myofibroblastic tumor (IMFT) is an uncommon neoplasm in children. METHODS: Retrospective review from 1993 to 2014 of patients ≤18years of age with a histopathologic diagnosis of IMFT treated at two tertiary centers. RESULTS: Thirty-two patients were diagnosed with IMFT. Mean (±SD) age was 9.3±5.7years at diagnosis. Tumor location was variable: abdomen/pelvis (28%), head/neck region (22%), intrathoracic (22%), genitourinary (9%), bowel (6%) liver (6%), and musculoskeletal (6%). Median follow-up was 2.6±4.6years, with 3 recurrences and 2 deaths, which occurred only after recurrence. Positive microscopic margin after resection was associated with recurrence, compared to those that had a negative margin (40% vs. 0%, p=0.04). Recurrence was associated with increased mortality (67% vs 0%, p=0.01). Time from first symptoms to resection was shorter in those with recurrence (25.8±22 vs. 179±275days, p=0.01) and in nonsurvivors (44.0±8.0 vs. 194.3±53.4days, p=0.02). Adjuvant chemotherapy, not including steroid monotherapy, either given before or after resection, was administered more often to nonsurvivors (100% vs 4%, p=0.009), and use of corticosteroids was also higher in the nonsurvivors (100% vs. 15%, p=0.04). CONCLUSIONS: IMFT is a rare pediatric neoplasm with variable locations. Complete excision is critical for cure. Proposed guidelines for diagnosis, treatment and surveillance of theses tumors in children are reported.

Epidural versus Patient-Controlled Analgesia after Pediatric Thoracotomy for Malignancy: A Preliminary Review.

Introduction The use of thoracic epidural is standard in adult thoracotomy patients facilitating earlier mobilization, deep breathing, and minimizing narcotic effects. However, a recent randomized trial in pediatric patients who undergo repair of pectus excavatum suggests patient-controlled analgesia (PCA) produces a less costly, minimally invasive postoperative course compared with epidural. Given that thoracotomy is typically less painful than pectus bar placement, we compared the outcomes of epidural to PCA for pain management after pediatric thoracotomy. Methods A retrospective review of 17 oncologic thoracotomies was performed at a children's hospital from 2004 to 2013. Data points included operative details, epidural or PCA use, urinary catheterization, days to regular diet, days to oral pain regimen, postoperative pain scores, length of stay, and anesthesia charges. Patients were excluded if they did not have epidural or PCA following thoracotomy. Results Six thoracotomies were managed with an epidural and 11 with a PCA. Three epidural patients were opiate naïve compared with two with a PCA. The most common indication for thoracotomy was metastatic osteosarcoma (n = 13). When comparing epidural to PCA, there was no significant difference in days to removal of Foley catheter, regular diet, oral pain control, length of stay, or total operating room time. Postoperative pain scores were also comparable. The mean anesthesia charges were significantly higher in patients with an epidural than with a PCA. Conclusion Epidural catheter and PCA provided comparable pain relief and objective recovery course in children who underwent thoracotomy for oncologic disease; however, epidural catheter placement was associated with increased anesthesia charges, suggesting that PCA is a noninvasive, cost-effective alternative.

The Role of Medication in Spontaneous Gastrocutaneous Fistula Closure.

PURPOSE: Factors precipitating persistence of gastrocutaneous fistulas (GCFs) are not clearly understood. The role of proton pump inhibitors (PPIs) or histamine receptor antagonists in GCF closure is not yet studied. We aimed to identify whether these medications influence spontaneous GCF closure. METHODS: Retrospective review was performed on children who underwent gastrostomy tube insertion and removal from January 2010 to February 2013. Spontaneous GCF closure rates and medication use during gastrostomy tube removal were investigated. RESULTS: Of the 97 patients included, 48 had spontaneous GCF closure, whereas 49 required operative closure. When comparing these two groups, no significant difference existed in spontaneous GCF closure rates among patients who were on ranitidine, PPIs, or both (p = 0.09, p = 0.83, p = 0.06 respectively). Spontaneous closure occurred more in older patients (2.7 ± 4.1 vs. 0.9 ± 1.6 years, p < 0.01) and in patients without fundoplication at time of tube insertion (12.5 vs. 30.6%, p = 0.05). There were more laparoscopic placements in the group that closed spontaneously (83 vs. 61%, p = 0.02). Mean gastrostomy tube presence was longer in patients who required surgery than those with spontaneous closures (18.7 ± 10.3 vs. 35.5 ± 36.6 months, p < 0.01). CONCLUSION: Ranitidine or PPI use upon removal of gastrostomy tubes does not seem to facilitate spontaneous GCF closure in children.

Influence of body mass index on skin grafting in pediatric burns.

BACKGROUND: There is heterogeneous literature on the association of obese and underweight body habitus on burn outcomes in adult and pediatric literature. We examine the effect of standardized pediatric body mass index (BMI) categories skin graft utilisation. METHODS: A retrospective chart review was performed on children who underwent burn treatment from January 1995 to November 2011. BMI was categorized by standard definitions: underweight (<5%), normal (5-85%), overweight (85-95%), obese (>95%). RESULTS: There were 1164 patients: 77 underweight, 604 normal, 215 overweight, and 268 obese patients. No differences existed between group demographics. Grafts were performed in 39% of underweight, 27% of normal, 22% of overweight, and 27% of obese patients. Underweight children had nearly a 2 fold increase in their risk of full thickness burns and were 1.8 times more likely to undergo skin grafting than normal BMI children. Overweight children had a significant decrease in the incidence skin grafting by 23% then compared to normal weight children. There were no differences in percent TBSA burned or percent TBSA grafted using ANOVA. CONCLUSIONS: Underweight pediatric burn victims have an increased risk for skin grafting while mildly overweight children are slightly protected from skin grafting.

Single-incision laparoscopic ileocecectomy in children with Crohn's disease.

INTRODUCTION: We previously reported our experience with standard laparoscopic ileocecectomy, but we have recently used a single-incision laparoscopic approach. PATIENTS AND METHODS: We conducted a retrospective review of a single surgeon's experience from 2009 to 2013. RESULTS: Twenty-eight children 11-18 years of age (mean, 15.5 years) with a mean body mass index of 18.9±3 kg/m(2) underwent single-incision laparoscopic ileocecectomy for Crohn's disease. Mean operative time was 86.5±25.9 minutes (range, 56-166 minutes). There were no extra ports placed or conversions to open surgery. Five children (18%) were on parenteral nutrition at time of surgery, 14 (50%) were on steroids, and 9 (32%) were on tumor necrotic factor inhibitors. A stapled extracorporeal anastomosis was performed in all children. Complications included abscess (n=4), small bowel obstruction (n=3), superficial wound infection (n=3), and small bowel perforation (n=1). Some patients had more than one complication. Of those with complications, 5 (56%) were on steroids, 5 (56%) were on tumor necrotic factor inhibitors, and 1 patient was on both. The perforation occurred at a point of adhesiolysis also involved with Crohn's disease. There were no anastomotic leaks. Median follow-up was 17 months (range, 1-47 months). CONCLUSIONS: Single-incision laparoscopic ileocecectomy is safe and feasible in pediatric patients with Crohn's disease.

Radiation exposure - how do CT scans for appendicitis compare between a free standing children's hospital and non-dedicated pediatric facilities?

BACKGROUND: We compare the amount of radiation children receive from CT scans performed at non-dedicated pediatric facilities (OH) versus those at a dedicated children's hospital (CH). METHODS: Using a retrospective chart review, all children undergoing CT scanning for appendicitis at an OH were compared to children undergoing CT imaging for appendicitis at a CH between January 2011 and November 2012. RESULTS: One hundred sixty-three children underwent CT scans at 42 different OH. Body mass index was similar between the two groups (21.00±6.49kg/m(2), 19.58±5.18kg/m(2), P=0.07). Dose length product (DLP) was 620±540.3 at OH and 253.78±211.08 at CH (P < 0.001). OH CT scans accurately diagnosed appendicitis in 81%, while CT scans at CH were accurate in 95% (P=0.026). CTDIvol was recorded in 65 patients with subset analysis showing CTDIvol of 16.98±15.58 and 4.89±2.64, a DLP of 586.25±521.59 and 143.54±41.19, and size-specific dose estimate (SSDE) of 26.71±23.1 and 3.81±2.02 at OH and CH, respectively (P<0.001). CONCLUSION: Using SSDE as a marker for radiation exposure, children received 86% less radiation and had improved diagnostic accuracy when CT scans are performed at a CH.

Burden of complications from needle penetration of plastic ports in children.

BACKGROUND: Complications of totally implanted venous access ports are well documented. A concerning mechanical complication we have encountered is posterior penetration of plastic ports with the access needle. The purpose of this study is to investigate the burden of posterior penetrations. METHODS: We performed a retrospective review of all ports placed between November 2007 and December 2011 at a single institution. RESULTS: There were 247 children who received a port. 117 children (47%) received a port with a plastic posterior wall, 95 children (38%) received a port with a metal posterior wall, and 35 children (14%) had ports that were unable to be identified as plastic or metal. Posterior port penetrations occurred 8 times (3.2% overall, 6.8% of plastic ports). All perforations occurred in plastic ports of a single brand and product code. Average time from port insertion to penetration was 11.2±21.3 months (range 0.3 to 63.4 months). Other complications included catheter malfunction (14), infection (9), pain (2), inability to draw/aspirate (4), leak (3), port migration (2), and malfunctioning not otherwise specified (15). CONCLUSIONS: There is an unacceptably high risk of needle penetration of the posterior wall of plastic ports. We recommend utilizing ports with metal backing to avoid this complication.

Laparoscopic pyloromyotomy: comparing the arthrotomy knife to the Bovie blade.

BACKGROUND: Laparoscopic pyloromyotomy was performed at our institution using an arthrotomy knife until it became unavailable in 2010. Thus, we adapted the use of the blunt Bovie tip, which can be used with or without electrocautery to perform the myotomy. This study compared the outcomes between using the arthrotomy knife versus the Bovie blade in laparoscopic pyloromyotomies. MATERIALS AND METHODS: Retrospective review was performed on all laparoscopic pyloromyotomy patients from October 2007 to September 2012. Arthrotomy knife pyloromyotomy patients were compared with those performed with the Bovie blade. Patient demographics, diagnostic measurements, electrolyte levels, length of stay, operative time, and complications were compared. RESULTS: A total of 381 patients were included, with 191 in the arthrotomy group and 190 in the Bovie blade group. No significant differences existed between groups in age, weight, gender, pyloric dimensions, electrolyte levels, or length of stay. Mean operative times were 15.8±5.6 min with knife and 16.4±5.3 min for Bovie blade (P=0.24). In the arthrotomy knife group, there was one incomplete pyloromyotomy and one omental herniation. There was one wound infection in each group. Readmission rate was greater in the arthrotomy knife group (5.7%) versus the Bovie blade group (3.1%). CONCLUSIONS: The Bovie blade appears to offer no objective disadvantages compared with the arthrotomy knife when performing laparoscopic pyloromyotomy.

Ceftriaxone-induced pseudolithiasis in children treated for perforated appendicitis.

PURPOSE: Ceftriaxone has been associated with development of pseudolithiasis. In our institution, it is used for treatment of perforated appendicitis in children. This study evaluated the occurrence of ceftriaxone-related pseudolithiasis in this population. METHODS: After obtaining IRB approval, we performed a retrospective chart review over 51 months. We included patients undergoing laparoscopic appendectomy for perforated appendicitis. All patients were treated with ceftriaxone post-operatively. Patients without initial or post-treatment gallbladder imaging available for review were excluded. RESULTS: There were 71 patients who met inclusion criteria with a mean (±SD) age of 10.8 ± 3.8 years. Of these, 14 % (n = 10) developed stones or sludge in the gallbladder. The mean duration of ceftriaxone therapy was 8.7 ± 3.8 days. The average time to post-antibiotic imaging was 11.5 ± 10.3 days from initiation of antibiotics. There was no significant difference in duration of ceftriaxone therapy in the children that developed pseudolithiasis or sludge (10.0 ± 4.9 days) compared to those that did not (8.5 ± 3.6, p = 0.26). One child (10 %) with pseudolithiasis went on to become symptomatic, requiring a laparoscopic cholecystectomy. CONCLUSIONS: In our experience, ceftriaxone use for perforated appendicitis is associated with a significant incidence of biliary pseudolithiasis, and is unrelated to duration of ceftriaxone therapy.

Reduced use of computed tomography in patients treated with interval appendectomy after implementing a protocol from a prospective, randomized trial.

BACKGROUND: In 2009, we instituted a protocol to standardize care for patients undergoing interval appendectomy based on results from a prospective trial that demonstrated a reduction in the mean number of computed tomography (CT) scans performed. The goal of this study was to determine if our current practice now resulted in fewer CT scans as a result of this trial. METHODS: A retrospective review of all patients undergoing interval appendectomy for perforated appendicitis from March 2009 to March 2011 was performed. Demographics and outcomes were compared to previously collected data from a retrospective study prior to institution of the protocol and to the prospective trial. RESULTS: During the study period, 45 patients underwent interval appendectomy. There were no differences in demographics among the three studies. Similar numbers of patients underwent aspiration or percutaneous drainage. There continues to be a significant reduction in the number of CT scans (3.5 ± 2.0 vs. 2.1 ± 1.3, P = 0.0001) and health care visits (7.6 ± 2.8 vs. 4.5 ± 1.4, P = 0.0001) when comparing management prior to the prospective trial to management since its completion. CONCLUSION: A protocol for management of patients undergoing interval appendectomy care results in fewer health care visits and CT scans.

Implications of Foley catheterization in children with perforated appendicitis.

BACKGROUND: We treat patients after appendectomy for perforated appendicitis with patient controlled analgesia (PCA) using a background continuous dose. We usually place urinary catheters in these patients because of concerns of urinary retention. The objective of the present study was to determine the rate of urinary retention in this patient population when a catheter was not used or was removed before the continuous PCA infusion was discontinued. METHODS: We performed a retrospective review of all patients who had received PCA postoperatively for perforated appendicitis from December 2008 to May 2011. The demographics, need for replacement of a Foley catheter, number of recorded nursing calls to physicians, and the incidence of urinary tract infection was recorded. Patients who had received a urinary catheter intraoperatively were compared with those who had not. Subgroups were also created according to whether the patients with a Foley catheter had undergone removal of their catheter before or after cessation of the continuous PCA infusion. RESULTS: Of 242 patients, 20 (8.3%) did not have a catheter postoperatively, 1 of whom required a catheter because of retention. Of the 222 patients who started with a catheter, 2 required reinsertion for retention (P = 0.59). Age, gender, and body mass index were similar for patients with and without a catheter. In the subgroup analysis, of the patients with a catheter, 48 (21.6%) had their catheter removed before discontinuation of the continuous PCA infusion and none required Foley catheter replacement. Of the 174 patients whose catheters were removed after discontinuation of the continuous PCA dose, 2 required catheter replacement (P = 0.46). A significantly higher percentage of telephone calls was generated for patients with a catheter than for patients without a Foley catheter (41.4% versus 10%, P = 0.007). No patients with a catheter developed a urinary tract infection. CONCLUSIONS: Patients with a perforated appendicitis who receive a continuous PCA have a low rate of urinary retention whether or not a catheter has been placed intraoperatively.

Patient scar assessment after single-incision versus four-port laparoscopic cholecystectomy: long-term follow-up from a prospective randomized trial.

BACKGROUND: The single-incision laparoscopic approach for cholecystectomy has been reported to be cosmetically superior in the traditional four-port technique in several case series; however, prospective comparative data are lacking. We conducted a 60-patient, prospective, randomized trial comparing single-incision laparoscopic cholecystectomy with standard four-port cholecystectomy, including validated scar assessment evaluation around 6 weeks and 18 months after the operation in an effort to determine if a cosmetic advantage existed. PATIENTS AND METHODS: Patients over 12 years of age and parents of patients under 12 years of age enrolled in the trial were asked to complete the validated Patient Scar Assessment Questionnaire (PSAQ). The PSAQ consists of four subscales: Appearance, Consciousness, Satisfaction with Appearance, and Satisfaction with Symptoms. The Symptoms subscale is omitted from analysis per PSAQ instructions because of insufficient reliability. Each subscale is a set of items with 4-point categorical responses (from 1=most favorable to 4=least favorable). The sum of the questions quantifies each subscale. Data are expressed as mean±standard deviation values. RESULTS: Eighteen single-site patients and 8 four-port patients completed early questionnaires, in which there was no difference in overall scar assessment (P=.17). Telephone follow-up was accomplished for 17 single-site patients and 24 four-port patients and revealed that the overall scar assessment significantly favored the single-site approach (P=.04). CONCLUSIONS: Patients or parents of patients do not identify an overall superior scar assessment at early follow-up after single-site laparoscopic versus four-port cholecystectomy. However, they do perceive a superior scar assessment at long-term follow-up, suggesting that there is a cosmetic benefit favoring the single-site approach.

Accuracy of American College of Surgeons National Surgical Quality Improvement Program Pediatric for laparoscopic appendectomy at a single institution.

BACKGROUND: In October 2008, the American College of Surgeons revealed the National Surgical Quality Improvement Program (NSQIP) Pediatric in an effort to improve quality of surgical care in children. A 5% disagreement rate of data reported between institutions is accepted. The two goals of this study were to (1) determine if the random sampling performed with NSQIP data collection was representative of the population, and (2) verify that data captured in NSQIP was accurate. METHODS: For children undergoing laparoscopic appendectomy from April 2010-April 2011, demographic data, length of stay (LOS), and rates of surgical site infection (SSI) and postoperative abscess recorded in NSQIP (group 1) were compared with data from chart review (group 2). Secondarily, all NSQIP data were examined for accuracy by comparing relevant data points to existing databases. All disagreements were further examined with review of the medical chart. Unpaired t-test and χ(2) with Fisher's exact test were used in the statistical analysis. RESULTS: NSQIP Pediatric captured data from 126 children (group 1); group 2 had 525 children. There were no significant differences in age, body mass index, gender, race or LOS between the two groups. Rate of SSI was 1.6% in group 1 and 1.7% in group 2 (P = 0.92). Abscess rate was 1.6% in group 1 and 3.4% in group 2 (P = 0.28). There were six errors in the NSQIP database. One child was listed as having two SSI. One child with postoperative abscess was missed and another was not counted as they were not categorized correctly. Recorded LOS was incorrect for two children and the other had incorrect age. CONCLUSIONS: NSQIP Pediatric captured a representative sample of patients undergoing laparoscopic appendectomy. Errors were found in the reporting of outcomes for SSI and postoperative abscess in children undergoing laparoscopic appendectomy. Given the low incidence of these outcomes, there is little effect on percentages of complications reported.

The prevalence of Hirschsprung disease in premature infants after suction rectal biopsy.

BACKGROUND: The prevalence of Hirschsprung disease (HD) in the premature infant population is not well documented. However, delayed passage of stool is common in premature infants, and suction rectal biopsy (SRB) is often used to evaluate for HD in this population. The use of SRB is unknown. Therefore, we evaluated the role of SRB in premature infants with abnormal stooling patterns. METHODS: After Institutional Review Board approval, a retrospective study was conducted on all infants having an SRB performed to exclude HD from January 2000 to December 2010. Infants were divided into two groups according to gestational age (premature < 37 wk; term ≥ 37 wk). Demographics, diagnosis, treatments, and outcomes were collected. A subset analysis was performed on patients diagnosed with HD. RESULTS: Two hundred sixty-nine infants were identified (113 premature and 156 term). Six premature infants (5.3%) and 79 term infants (50.6%) were found to have HD (P < 0.01). As expected, gestational age was significantly different between groups (31.7 versus 38.9 wk, P < 0.01) (Table 1). Premature infants were less likely to have prenatal care (35% versus 55%, P < 0.01) and had longer lengths of hospital stay (45.6 versus 17.6 d, P < 0.01). The most common location of aganglionosis was rectosigmoid in both groups (group 1, 50%; group 2, 33%, P = 0.7). CONCLUSIONS: HD occurs significantly less often in premature infants than in term infants. Alternative diagnoses should be investigated in this population when delayed stooling patterns are encountered. SRB should be used more selectively in this group.

Clinical outcomes following bowel resection versus reduction of intussusception.

BACKGROUND: Intussusception is most commonly managed with air-contrast reduction. However, when this fails, emergent operation with resection or manual reduction is indicated. It is not known if there are advantages to resection compared with manual reduction. METHODS: A retrospective review of all patients receiving operative care for intussusception from February 2000 to December 2011. Patients undergoing intestinal resection were compared with those treated with manual reduction alone. RESULTS: Of 111 patients, 49 underwent resection and 62 underwent manual reduction. Mean (±SD) time to oral intake favored manual reduction (2.1 ± 1.2 versus 2.6 ± 1.2 d, respectively, P=0.05). Manual reduction was associated with a greater need for repeat imaging (47% versus 18%, P=0.002) and the only recurrences were with manual reduction (8% versus 0%, P=0.1). Mean duration of stay was no different (P=0.36), nor was the need for reoperation (P=0.9). CONCLUSIONS: Patients undergoing manual reduction have an increased number of radiographic imaging procedures. The surgeon should have a low threshold for resection for intussusceptions requiring operative management.