Role of the PI3K/AKT pathway and PTEN in otitis media.

Mucosal hyperplasia is common sequela of otitis media (OM), leading to the secretion of mucus and the recruitment of leukocytes. However, the pathogenic mechanisms underlying hyperplasia are not well defined. Here, we investigated the role of the AKT pathway in the development of middle mucosal hyperplasia using in vitro mucosal explants cultures and an in vivo rat model. The Akt inhibitor MK2206 treatment inhibited the growth of middle ear mucosal explants in a dose-dependent manner. In vivo, MK2206 also reduced mucosal hyperplasia. Unexpectedly, while PTEN is generally thought to act in opposition to AKT, the PTEN inhibitor BPV reduced mucosal explant growth in vitro. The results indicate that both AKT and PTEN are mediators of mucosal growth during OM, and could be potential therapeutic targets.

Airway Obstruction during Sleep due to Diaphragm Pacing Precludes Decannulation in Young Children with CCHS.

Children with congenital central hypoventilation syndrome (CCHS) have a PHOX2B mutation-induced control of breathing deficit necessitating artificial ventilation as life support. A subset of CCHS families seek phrenic nerve-diaphragm pacing (DP) during sleep with the goal of tracheal decannulation. Published data regarding DP during sleep as life support in the decannulated child with CCHS and related airway dynamics in young children are limited. We report a series of 3 children, ages 3.3-4.3 years, who underwent decannulation. Sleep endoscopy performed during DP revealed varied (oropharynx, supraglottic, glottic, etc.) levels of complete airway obstruction despite modification of pacer settings. Real-time analysis of end tidal CO2 and SpO2 confirmed inadequate gas exchange. Because the families declined re-tracheostomy, all 3 patients rely on noninvasive mask ventilation as a means of life support while asleep. These results emphasize the need for extreme caution in proceeding with tracheal decannulation in young children with CCHS who expect to use DP during sleep as life support. Parents and patients should anticipate that they will depend on noninvasive mask ventilation (rather than DP) during sleep after undergoing decannulation. This information may improve management and guide expectations regarding potential decannulation in young paced children with CCHS.

Perioperative anesthetic management of children with congenital central hypoventilation syndrome and rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation undergoing thoracoscopic phrenic nerve-diaphragm pacemaker implantation.

BACKGROUND: Congenital Central Hypoventilation Syndrome and Rapid-Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation are rare neurocristopathies characterized by autonomic dysregulation including bradyarrhythmias, abnormal temperature control, and most significantly, abnormal control of breathing leading to tracheostomy and ventilator dependence as life support. Surgical advancements have made phrenic nerve-diaphragm pacemakers available, to eliminate the tether to a mechanical ventilator for 12-15 hours each day. The thoracoscopic approach to implantation has allowed for a less invasive approach which may have implications for pain control and recovery time. However, thoracoscopic implantation of these devices presents several challenges to the anesthesiologist in these complex ventilator-dependent patients, including, but not limited to, sequential lung isolation, prevention of hypothermia, and management of arrhythmias. Postoperative challenges may also include strategies to treat hemodynamic instability, managing the ventilator following lung derecruitment, and providing adequate pain control. AIMS: We aimed to describe the anesthetic management of Congenital Central Hypoventilation Syndrome and Rapid-Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation patients undergoing thoracoscopic phrenic nerve-diaphragm pacemaker implantation and the nature and incidence of perioperative complications. METHODS: A retrospective chart review was performed of 14 children with Congenital Central Hypoventilation Syndrome and Rapid-Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation undergoing phrenic nerve-diaphragm pacemaker implantation at a single academic pediatric hospital between 2009 and 2017. Demographic information, intraoperative management, and perioperative complications were analyzed from patient records. RESULTS: Twelve of 14 patients (86%) underwent an inhalational induction via tracheostomy. Lung isolation was achieved via fiberoptic guidance of a single lumen endotracheal tube sequentially into the right or left mainstem bronchi for 12 patients (86%). Double lumen endotracheal tubes were utilized in two patients (7%) and bronchial blockers in two patients (7%) for lung isolation. Anesthesia was maintained using a balanced technique of volatile agents (sevoflurane/isoflurane) and opioids (fentanyl). Bradyarrhythmias developed in six patients (43%) during surgery, 5 (36%) responded to anticholinergics and one patient (7%) required backup cardiac pacing using a previously implanted bipolar cardiac pacemaker. Intraoperative hypothermia (<35.5°C) was present in five patients (36%) despite the use of warming devices. Hypercarbia (>50 mm Hg) during lung isolation was present in eight patients (57%) and hemoglobin desaturation (<90%) in four patients (29%). Postoperatively, oxygen desaturation was a common complication with nine patients (64%) requiring supplemental oxygen administration via mechanical ventilator or manual bag ventilation. Opioids via patient-controlled analgesia devices (12 patients, 86%) or intermittent injection (two patients, 14%) were administered to all patients for postoperative pain control. Phrenic nerve-diaphragm pacemaker placement was successful thoracoscopically in all patients with no perioperative mortality. CONCLUSION: The main anesthetic challenges in patients with Congenital Central Hypoventilation Syndrome and Rapid-Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation include hemodynamic instability, the propensity to develop hypothermia, hypercarbia/hypoxemia, and the need to perform bilateral sequential lung isolation requisite to the thoracoscopic implantation technique. Most anesthetic agents can be used safely in these patients; however, adequate knowledge of the susceptibility to complications, coupled with adequate preparation and understanding of the innate disease characteristics, are necessary to treat anticipated complications.

Incidental GIST after appendectomy in a pediatric patient: a first instance and review of pediatric patients with CD117 confirmed GISTs.

A 7-year-old boy underwent uncomplicated laparoscopic appendectomy for acute appendicitis. Incidentally, he was found to have a spindle cell tumor with CD117 immunopositivity, consistent with gastrointestinal stromal tumor (GIST) in the appendix. Although commonly reported in adults, pediatric GISTs are rare gastrointestinal malignancies that occur in only 1.4-2.7% of children and adolescents. Due to the paucity of reports, data are insufficient to adequately characterize tumor behavior, recurrence, and survival. We present the first case of pediatric GIST in the appendix. In addition, a review of the literature for CD117 confirmed pediatric GISTs was conducted to summarize its clinical features and current treatment options.

Nonconvulsive seizures are common in children treated with extracorporeal cardiac life support.

OBJECTIVES: The prevalence of electrographic seizures or nonconvulsive status epilepticus and the effect of such seizures in children treated with extracorporeal cardiac life support are not known. We investigated the occurrence of electrographic abnormalities, including asymmetries in amplitude or frequency of the background rhythm and interictal activity in children undergoing extracorporeal cardiac life support and their association with seizures. We compared mortality and radiologic evidence of neurologic injury between patients with seizures and those without seizures. DESIGN: Retrospective review of medical records and the Extracorporeal Life Support Organization database. SETTING: PICU at a single institution. PATIENTS: All pediatric patients up to 18 years old, who had extracorporeal cardiac life support and continuous electroencephalography monitoring between the years 2006 and 2011. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Nineteen patients treated with extracorporeal cardiac life support underwent continuous electroencephalography monitoring. Seizures occurred in four patients (21%) and were exclusively nonconvulsive in three patients. Two of these four patients had nonconvulsive status epilepticus. Interictal discharges on electroencephalography were associated with seizures (odds ratio, 19.5 [95% CI, 1.29-292.75]; p = 0.03). Only 50% of the seizures were detected in the first hour of monitoring, whereas all seizures were detected within 24 hours. All patients with seizures had structural abnormalities seen on neuroimaging. Seizures were not significantly associated with increased mortality. To evaluate for ascertainment bias, we compared outcomes between patients who underwent extracorporeal cardiac life support and received continuous electroencephalography monitoring and those patients who underwent extracorporeal cardiac life support during the study period but did not receive electroencephalography (n = 30). CONCLUSIONS: Seizures are common in children during extracorporeal cardiac life support, and most seizures are nonconvulsive. In patients undergoing extracorporeal cardiac life support, clinical features are unreliable indicators of the presence of seizures. The presence of seizures is suggestive of CNS injury. This study is limited by the exclusion of neonates, a feature of the clinical use of electroencephalography at our institution. Although seizures were not associated with increased mortality, further prospective studies in larger populations are needed to assess the long-term morbidity associated with seizures during extracorporeal cardiac life support.

Measuring the Efficacy of Thymectomy for Pediatric Myasthenia Gravis Across Tertiary Children's Hospitals.

BACKGROUND: Thymectomy is a treatment for pediatric myasthenia gravis, but the efficacy over time is unknown. Multi-institutional data are also lacking. Therefore, the objective of this study was to determine the efficacy of thymectomy for pediatric myasthenia gravis using medication burden and health care utilization as proxies for disease severity. METHODS: This was a cross-sectional study of the Pediatric Health Information System database among children who underwent thymectomy at one of 49 children's hospitals from 2004 to 2022. Differences in annual median number of doses of myasthenia-related medications, admissions, and health care costs in the year before thymectomy to three years after were compared. A comparison cohort that did not undergo thymectomy was utilized. Medians were compared using the Wilcoxon signed-rank test. Generalized linear regression estimated the effect of surgical approach on outcomes. RESULTS: A total of451 patients (238 patients who underwent thymectomy and 213 nonthymectomy patients) were identified. Following thymectomy, the decrease in annual median total number of myasthenia-related doses was 12.0 (interquartile range: 6 to 31) (P < 0.001). The decrease in number of annual admissions was 2.0 (1 to 4) (P < 0.001), which represented a cost difference of $5292 ($3533 to $8681) (P < 0.001). No differences were observed in the control cohort. In a generalized linear regression model, surgical approach was not associated with the efficacy of thymectomy (P = 0.55). CONCLUSIONS: Thymectomy is an effective treatment for pediatric myasthenia gravis, evidenced by the decreased medication burden and health care utilization after surgery. Surgical approach did not influence the success of surgery. Thymectomy should be considered earlier in the treatment algorithm.

An unusual cause of pulmonary infiltrates mimicking pulmonary edema: metastatic calcifications.

Metastatic calcification is an uncommon condition with underlying abnormal bone and calcium metabolism, leading to ectopic deposition of calcium in soft tissues. The lung is a common site of involvement, and can mimic pulmonary edema in radiographic findings, especially in clinical context of end-stage renal failure. Recognition of such condition is important to avoid unnecessary diuretic therapy and extra dialysis sessions and guides correct treatment of underlying metabolic disorder. This article, therefore, serves to illustrate such condition with emphasis to radiological and clinical features suggestive of such condition.

Unilateral groin surgery in children: will the addition of an ultrasound-guided ilioinguinal nerve block enhance the duration of analgesia of a single-shot caudal block?

BACKGROUND: Inguinal hernia repair, hydrocelectomy, and orchidopexy are commonly performed surgical procedures in children. Postoperative pain control is usually provided with a single-shot caudal block. Blockade of the ilioinguinal nerve may lead to additional analgesia. The aim of this double-blind, randomized controlled trial was to evaluate the efficacy of an adjuvant blockade of the ilioinguinal nerve using ultrasound (US) guidance at the end of the procedure with local anesthetic vs normal saline and to explore the potential for prolongation of analgesia with decreased need for postoperative pain medication. METHODS: Fifty children ages 1-6 years scheduled for unilateral inguinal hernia repair, hydrocelectomy, orchidopexy, or orchiectomy were prospectively randomized into one of two groups: Group S that received an US-guided ilioinguinal nerve block with 0.1 ml x kg(-1) of preservative-free normal saline and Group B that received an US-guided nerve block with 0.1 ml x kg(-1) of 0.25% bupivacaine with 1 : 200,000 epinephrine at the conclusion of the surgery. After induction of anesthesia but prior to surgical incision, all patients received caudal anesthesia with 0.7 ml x kg(-1) of 0.125% bupivacaine with 1 : 200,000 epinephrine. Patients were observed by a blinded observer for (i) pain scores using the Children and Infants Postoperative Pain Scale, (ii) need for rescue medication in the PACU, (iii) need for oral pain medications given by the parents at home. RESULTS: Forty-eight patients, consisting of 46 males and two females, with a mean age of 3.98 (SD +/- 1.88) were enrolled in the study. Two patients were excluded from the study because of study protocol violation and/or alteration in surgical procedure. The average pain scores reported for the entire duration spent in the recovery room for the caudal and caudal/ilioinguinal block groups were 1.92 (SD +/- 1.59) and 1.18 (SD +/- 1.31), respectively. The average pain score difference was 0.72 (SD +/- 0.58) and was statistically significant (P < 0.05). In addition, when examined by procedure type, it was found that the difference in the average pain scores between the caudal and caudal/ilioinguinal block groups was statistically significant for the inguinal hernia repair patients (P < 0.05) but not for the other groin surgery patients (P = 0.13). For all groin surgery patients, six of the 23 patients in the caudal group and eight of the 25 patients in the caudal/ilioinguinal block group required pain rescue medications throughout their entire hospital stay or at home (P = 0.76). Overall, the caudal group received an average of 0.54 (SD +/- 1.14) pain rescue medication doses, while the caudal/ilioinguinal block group received an average of 0.77 (SD +/- 1.70) pain rescue medication doses; this was, however, not statistically significant (P = 0.58). CONCLUSIONS: The addition of an US-guided ilioinguinal nerve block to a single-shot caudal block decreases the severity of pain experienced by pediatric groin surgery patients. The decrease in pain scores were particularly pronounced in inguinal hernia repair patients.

Endoloop as the first line tool for appendiceal stump closure in children with appendicitis.

PURPOSE: The objective of this study is to compare outcomes for pediatric patients undergoing laparoscopic appendectomy (LA) performed either (1) using an endostapler (ES) to divide the appendix and mesoappendix or (2) using endoloops (ELs) to close the appendiceal stump and electrocautery to divide the mesoappendix. METHODS: We conducted a retrospective chart review of all patients who underwent LA for suspected appendicitis 4 years at a free standing children's hospital. The use of EL and ES was compared separately in patients with perforated and nonperforated appendicitis. We compared patient characteristics and outcomes. RESULTS: There were no significant differences in rate of postoperative abscess, rate of subsequent small bowel obstruction requiring operation or rate of intraperitoneal hematoma between the ES and EL groups for both nonperforated and perforated appendectomy cases. Superficial wound infection was more common in the nonperforated EL group (17/309, 5.5%) than in the nonperforated ES group (2/235, 0.9%; p = 0.007). Operative time for the EL technique (52.2 ± 15.8 minutes; p = 0.047) was shorter than for the ES technique (58 ± 23.2 minutes) for patients with perforated appendicitis. CONCLUSION: EL stump closure and mesoappendix cauterization during LA is safe and effective in children with appendicitis, including perforated appendicitis.

Accessory hemi diaphragm.

BACKGROUND/PURPOSE: Accessory hemi diaphragm (AHD) is an uncommon condition. Its clinical features, diagnosis and management have not been clearly defined. METHODS: We reviewed the world literature and added a case of our own. RESULTS: There are 40 proven cases in the literature. The lesion occurs almost exclusively in the right hemithorax. Twenty seven cases presented with cardiovascular and/or respiratory symptoms while 3 had symptoms pertaining to other systems. In the remaining 10 patients the diagnosis was either made accidentally or information pertaining to their presentation is missing. Abnormalities of pulmonary and systemic blood vessels often accompany the abnormality. The diagnosis is usually suspected because of an abnormal chest radiograph and confirmed by a variety of tests. CONCLUSIONS: If the AHD prevents proper aeration of the lung it should be excised. If vascular abnormalities coexist they should be treated along with the AHD. Asymptomatic patients may be observed after confirming the diagnosis.

Validation of measures from a thoracoscopic esophageal atresia/tracheoesophageal fistula repair simulator.

PURPOSE: A validated high fidelity simulation model would provide a safe environment to teach thoracoscopic EA/TEF repair to novices. The study purpose was to evaluate validity evidence for performance measures on an EA/TEF simulator. METHODS: IRB-exempt data were collected from 12 self-reported "novice" and 8 "experienced" pediatric surgeons. Participants evaluated the EA/TEF repair simulator using survey ratings that were analyzed for test content validity evidence. Additionally, deidentified operative performances were videotaped and independently rated by two surgeons using the Objective Structured Assessment for Technical Skills (OSATS) instrument. Novice and experienced OSATS were compared with p<.05 significant. RESULTS: Participants had high overall simulator ratings. Internal structure was supported by high interitem consistency (α=.95 and .96) and interrater agreement (ICC) [.52, .84] for OSATS ratings. Experienced surgeons performed at a significantly higher level than novices for all five primary and two supplemental OSATS items (p<.05). CONCLUSION: Favorable participant ratings indicate the simulator is relevant to clinical practice and valuable as a learning tool. Further, performance ratings can discriminate experienced and novice performances of EA/TEF repair. These findings support the use of the simulator for performance assessment, representing the first validated measures from a simulator intended for pediatric surgical training.

Severe menorrhagia, unilateral ovarian mass, elevated inhibin levels, and severe hypothyroidism: an unusual presentation of Van Wyk and Grumbach syndrome.

A 10 year old female presented with a 3 week history of persistent vaginal bleeding, lower abdominal pain, and generalized fatigue. Further work-up determined that she had severe autoimmune hypothyroidism, precocious puberty, and a large left cystic ovarian mass, consistent with Van Wyk and Grumbach syndrome. In addition, the patient had elevated inhibin A and B levels, which may be elevated in granulosa cell tumor. The patient was managed with thyroid hormone replacement therapy and careful observation. Complete resolution of the ovarian mass and interval normalization of the inhibin levels were achieved. In females with severe primary hypothyroidism, ovarian masses, and elevated tumor markers, operative resection may be avoided with appropriate thyroid replacement therapy and close observation.

Current diagnosis and management of urachal remnants.

PURPOSE: The purposes of this study are to review our experience with patients who were found to have urachal remnants. We discuss their diagnosis and management and we also evaluate post-operative complications in our cohort of patients with this entity. METHODS: We performed a retrospective review of all patients diagnosed with a urachal remnant (UR) between January 2006 and December 2010. Patient variables included age, gender, presenting symptoms and signs, diagnostic modality, and type of urachal remnant. Operative management, post-operative complications, and non-operative follow-up were abstracted. RESULTS: We identified 103 pediatric patients with either a urachal cyst (n = 38), urachal diverticulum (n = 13), urachal sinus (n = 11), patent urachus (n = 21), or a non-specific atretic urachal remnant (n = 20). Seventy-eight patients had symptomatic URs. Thirty-four symptomatic patients underwent surgical excision of the UR and 44 symptomatic patients were observed. Eighteen URs were incidentally diagnosed in asymptomatic patients, none of whom underwent surgical excision. In 7 patients symptoms could not be determined from the records. No patients with non-specific atretic remnants were operated upon. Nineteen of the observed patients were reimaged subsequently. In fifteen (78.9%) of these patients, the URs had resolved spontaneously. None of the patients who were observed required subsequent excision of the UR. Post-operative complications occurred in 14.7% of patients in whom URs were excised. CONCLUSION: Review of our patients diagnosed with URs indicates there is a subset of patients in whom spontaneous resolution could be expected. Complications are not uncommon after surgical excision.

Fate of the uterus in 46XX cloacal exstrophy patients.

PURPOSE: Genetically female cloacal exstrophy (46XX CE) patients develop complications later in life due to their abnormal uterine anatomy, resulting in various invasive gynecologic procedures. Furthermore, they have difficulty becoming pregnant, and if they do conceive, they are unlikely to carry the pregnancy to term. We performed this review to determine the rate of gynecological complications, the fate of the uterus, and the rate of pregnancy in 46XX cloacal exstrophy patients. METHODS: All charts for 46XX CE patients treated by us were reviewed following IRB approval. Patient age at last follow-up, surgical management of the uterus, uterine complications, and pregnancies were recorded. RESULTS: The charts of all 16 of our 46XX CE patients who survived past the neonatal period were reviewed. Two patients underwent hemi-hysterectomy (HH): 1 for an atretic hemi-uterus at birth, another for abnormal uterine insertion at 3 years. A third patient initially had HH for hydrometrocolpos leading to ureteral and colonic obstruction at 14 years but she required a completion hysterectomy a year later. Four patients underwent total hysterectomy (TH) at the outset: 2 neonates for a diminutive uterus with extreme disparity in the halves, another for uterine prolapse at 1 month of age, and a fourth for hematometrocolpos at 16 years of age. Six patients reached adulthood without requiring gynecologic intervention; one of these six is now being managed at another institution. Two patients are prepubertal and one was lost to follow-up. The only patient in the series who became pregnant miscarried at 11 weeks gestation. CONCLUSION: Out of 13 post-pubertal patients 6 have retained the entire uterus and another 2 had a hemi-hysterectomy. One patient who became pregnant miscarried at 11 weeks gestation. We believe it is appropriate to avoid ablative genital surgery as far as possible and for these patients to become pregnant after detailed discussion with physicians experienced in the care of 46XX CE patients.

Delayed repeated enema and operative findings after unsuccessful primary enema for intussusception.

INTRODUCTION: The purpose of this study was (1) to evaluate the role for and the potential morbidity associated with delayed repeated enema (DRE) and (2) to compare surgical findings for patients undergoing operative exploration after failed primary enema and DRE reduction attempts for intussusception. METHODS: After obtaining approval from the Internal Review Board, we completed a retrospective review of all patients treated for intussusception at a large, tertiary care pediatric hospital. We evaluated the success of primary reduction enema, DRE, and compared surgical findings after failed enema reduction. RESULTS: A total of 135 intussusceptions underwent an attempt at enema reduction following diagnosis. Of these, 83 (61.5%) intussusceptions were reduced with primary enema. Thirty-four patients (25.2%) proceeded directly to surgical exploration after a failed primary enema and 4 more patients were explored after successful reduction due to suspicion for a pathologic lead point. Thirty-four of these operations were therapeutic. In this, four patients had a negative exploration; eight patients required a bowel resection due to necrotic bowel, perforation, or persistence of an irreducible intussusception. Eighteen patients underwent a DRE after a failed primary enema. Two DREs revealed the intussusception had completely reduced before the study, and 11 DREs were successful in reducing persistent intussusceptions. Five patients underwent operative exploration after a failed DRE and were all found to have a persistent intussusception which was successfully reduced intraoperatively. There were no negative laparotomies, pathologic lead points, or instances of necrotic bowel or perforation in patients explored following unsuccessful DRE and no bowel resections were required. CONCLUSIONS: The use of DRE in select patients with persistence of an intussusception may result in successful subsequent reduction with no apparent increase in morbidity. A DRE may lead to improved outcomes for patients with intussusception through avoidance of unnecessary surgical reductions and negative explorations.

Utility of the computed tomography severity index (Balthazar score) in children with acute pancreatitis.

BACKGROUND: Previous studies in children with acute pancreatitis have demonstrated that clinical scoring systems such as the Ranson, modified Glasgow, and pediatric acute pancreatitis scores are of value in predicting severity of the disease. The aim of this study was to determine the predictive value of the computed tomography severity index (CTSI or Balthazar score) in pediatric patients. METHODS: All children (≤ 18 years) admitted to our institution with acute pancreatitis from 2000 through 2009 were reviewed. Contrast-enhanced computed tomographic (CT) images at presentation were retrospectively reviewed by 2 pediatric radiologists. Peripancreatic fluid and the extent of necrosis were assessed to determine the CTSI. The predictive value of the CTSI was calculated and compared with clinical scoring systems. RESULTS: Of 211 children with acute pancreatitis, 64 underwent contrast-enhanced CT at presentation. The median age was 12.3 years. Etiology of pancreatitis was idiopathic (35.9%), gallstone (17.2%), medication-induced (20.3%), posttransplant (9.4%), traumatic (6.3%), structural (1.6%), and other (9.4%). The sensitivity, specificity, positive predictive value, and negative predictive value of the CTSI (using a cutoff score of 4+) were 81%, 76%, 62%, and 90%, respectively, which compared favorably to the results of the pediatric acute pancreatitis (53%, 72%, 41%, 80%), Ranson (71%, 87%, 67%, 89%), and modified Glasgow (71%, 87%, 67%, 89%) scores. CONCLUSION: The CTSI is superior to clinical scoring systems for identifying children with acute pancreatitis at heightened risk for developing serious complications.

Laparoscopic repair of traumatic abdominal wall hernia from handlebar injury.

A 14-year-old boy was seen at an outside hospital after falling over the handlebar of his bicycle and was discharged home. He was subsequently seen in our emergency department with complaints of persistent abdominal pain. A computed tomography scan of the abdomen revealed disruption of the muscles of the upper right abdominal wall containing the hepatic flexure of the colon, with a small amount of intraperitoneal free fluid noted. The patient underwent laparoscopic exploration using 3 ports (2-5 mm and 1-12 mm) and 2 separate stab incisions. The traumatic abdominal wall hernia was repaired with interrupted sutures placed with an ENDO CLOSE (Covidien, Mansfield, MA) device, and a mesenteric defect in the colon was approximated with intracorporeal sutures. The trocar sites were sutured closed. The patient recovered well and was discharged home. Follow-up examination revealed no abdominal wall defect and resolution of his symptoms. Laparoscopic repair of a traumatic abdominal wall defect and exploratory laparoscopy after trauma is feasible and safe in the pediatric patient. It should be considered as an alternative approach with potentially less morbidity than an exploratory laparotomy for handlebar injuries in a stable patient.

Acute pancreatitis in children: spectrum of disease and predictors of severity.

BACKGROUND: The aim of this study was to describe the spectrum of disease in children with acute pancreatitis and assess predictors of severity. METHODS: Children (≤ 18 years) admitted to a single institution with acute pancreatitis from 2000 to 2009 were included. The accuracy of the Ranson, modified Glasgow, and pediatric acute pancreatitis severity (PAPS) scoring systems for predicting major complications was assessed. RESULTS: The etiology of pancreatitis in these 211 children was idiopathic (31.3%), medication-induced (19.9%), gallstones (11.8%), trauma (7.6%), transplantation (7.6%), structural (5.2%), and hemolytic-uremic syndrome (3.3%). Fifty-six patients (26.5%) developed severe complications. Using the cutoff thresholds in the PAPS scoring system, only admission white blood cell count more than 18,500/µL (odds ratio [OR], 3.1; P = .010), trough calcium less than 8.3 mg/dL (OR, 3.0; P = .019), and blood urea nitrogen rise greater than 5 mg/dL (OR, 4.1; P = .004) were independent predictors of severe outcome in a logistic regression model. The sensitivity (51.8%, 51.8%, 48.2%) and negative predictive value (83.2%, 83.5%, 80.5%) of the Ranson, modified Glasgow, and PAPS scores were, respectively, insufficient to guide clinical decision making. CONCLUSION: Commonly used scoring systems have limited ability to predict disease severity in children and adolescents with acute pancreatitis. Careful and repeated evaluations are essential in managing these patients who may develop major complications without early signs.

Pyloric duplication with communication to the pancreas in a neonate simulating hypertrophic pyloric stenosis.

An 11-day-old neonate presents with worsening nonbilious emesis and radiographic findings suggestive of pyloric stenosis. Using a laparoscopic approach, a slightly asymmetric and hypertrophied pylorus was visualized. The unusual age of presentation and appearance of the pylorus led to the decision to perform an open exploration for suspicion of a pyloric duplication. An incision of the pyloric muscle exposed and confirmed a duplication cyst. Further exposure revealed accessory pancreatic tissue communicating from the cyst to the main body of the pancreas. The duplication cyst and accessory pancreas were resected, and the patient had an uneventful recovery. Pyloric duplication is a rare cause of congenital extrinsic obstruction in the newborn. Even rarer is a communication of the duplication cyst with the pancreas. Resection of the duplication cyst and the aberrant pancreatic tissue and duct is recommended.

Laparoscopic Collis-Nissen for recurrent severe reflux in pediatric patients with esophageal atresia and recurrent hiatal hernia.

PURPOSE: Patients following esophageal atresia repair may often have a congenitally short esophagus, leading to severe reflux and failed fundoplications. This report evaluates the efficacy of a laparoscopic Collis-Nissen fundoplication in this group of patients who have failed a previous fundoplication. METHODS: From January 2005 to February 2010, 6 patients born with esophageal atresia presented with recurrent gastroesophageal reflux disease (GERD) and hiatal hernia (H/H). Patient's ages ranged from 5 to 12 years and weights from 17 to 32 kg. All patients had undergone at least three previous fundoplications and hiatal hernia repair. Four of 5 patients had at least one open laparotomy for their previous repair. Patient 6 had had a previous open Collis gastroplasty. The procedure was performed through five ports and consisted of a takedown of the previous fundoplication, elongation of the esophagus using an endoscopic stapler, closure of the hiatus using Teflon pledgets to buttress the repair, and formation of the fundoplication. RESULTS: All procedures were completed successfully laparoscopically. Procedure times ranged from 180 to 300 minutes. A nasogastric (NG) tube was left for an average of 3 days in 5 patients. Patient 6 had a delayed perforation on day 3 and required reexploration and an NG tube that was left for 10 days. Five of 6 patients were started on feeds on day 4 and were discharged on day 5. Patient 6 with the perforation was discharged on day 14. At an average follow-up of 42 months, all patients have intact wraps and no recurrence of their hiatal hernia. CONCLUSIONS: A laparoscopic Collis-Nissen in a child with previous failed fundoplication is a complex, but efficacious, procedure. Elongating the esophagus in patients with esophageal atresia may decrease the high recurrence rate of hiatal hernia in these patients and should be considered if the patient fails their primary repair.