Feeding behavior modifies the circadian variation in RR and QT intervals by distinct mechanisms in mice.

Rhythmic feeding behavior is critical for regulating phase and amplitude in the ≈24-h variation of heart rate (RR intervals), ventricular repolarization (QT intervals), and core body temperature in mice. We hypothesized changes in cardiac electrophysiology associated with feeding behavior were secondary to changes in core body temperature. Telemetry was used to record electrocardiograms and core body temperature in mice during ad libitum-fed conditions and after inverting normal feeding behavior by restricting food access to the light cycle. Light cycle-restricted feeding modified the phase and amplitude of 24-h rhythms in RR and QT intervals, and core body temperature to realign with the new feeding time. Changes in core body temperature alone could not account for changes in phase and amplitude in the ≈24-h variation of the RR intervals. Heart rate variability analysis and inhibiting ß-adrenergic and muscarinic receptors suggested that changes in the phase and amplitude of 24-h rhythms in RR intervals were secondary to changes in autonomic signaling. In contrast, changes in QT intervals closely mirrored changes in core body temperature. Studies at thermoneutrality confirmed that the daily variation in QT interval, but not RR interval, primarily reflected daily changes in core body temperature (even in ad libitum-fed conditions). Correcting the QT interval for differences in core body temperature helped unmask QT interval prolongation after starting light cycle-restricted feeding and in a mouse model of long QT syndrome. We conclude feeding behavior alters autonomic signaling and core body temperature to regulate phase and amplitude in RR and QT intervals, respectively.NEW & NOTEWORTHY We used time-restricted feeding and thermoneutrality to demonstrate that different mechanisms regulate the 24-h rhythms in heart rate and ventricular repolarization. The daily rhythm in heart rate reflects changes in autonomic input, whereas daily rhythms in ventricular repolarization reflect changes in core body temperature. This novel finding has major implications for understanding 24-h rhythms in mouse cardiac electrophysiology, arrhythmia susceptibility in transgenic mouse models, and interpretability of cardiac electrophysiological data acquired in thermoneutrality.

New role for cardiomyocyte Bmal1 in the regulation of sex-specific heart transcriptomes.

It has been well established that cardiovascular diseases exhibit significant differences between sexes in both preclinical models and humans. In addition, there is growing recognition that disrupted circadian rhythms can contribute to the onset and progression of cardiovascular diseases. However little is known about sex differences between the cardiac circadian clock and circadian transcriptomes in mice. Here, we show that the the core clock genes are expressed in common in both sexes but the circadian transcriptome of the mouse heart is very sex-specific. Hearts from female mice expressed significantly more rhythmically expressed genes (REGs) than male hearts and the temporal pattern of REGs was distinctly different between sexes. We next used a cardiomyocyte-specific knock out of the core clock gene, Bmal1, to investigate its role in sex-specific gene expression in the heart. All sex differences in the circadian transcriptomes were significantly diminished with cardiomyocyte-specific loss of Bmal1. Surprisingly, loss of cardiomyocyte Bmal1 also resulted in a roughly 8-fold reduction in the number of all the differentially expressed genes between male and female hearts. We conclude that cardiomyocyte-specific Bmal1, and potentially the core clock mechanism, is vital in conferring sex-specific gene expression in the adult mouse heart.

Feeding Behavior Modifies Daily Rhythms in Autonomic Signaling and Core Body Temperature to Shape 24-hour Rhythms in Cardiac Electrophysiology.

Circadian rhythms in physiology and behavior are intrinsic ~24-hour cycles regulated by biological clocks (i.e., circadian clocks) that optimize organismal homeostasis in response to predictable environmental changes. Studies suggest that circadian clock signaling in the suprachiasmatic nucleus of the hypothalamus and cardiomyocytes shape day/night rhythms in cardiac electrophysiology (i.e., RR and QT intervals). However, studies also show that the day/night rhythm of the RR and QT intervals depends on the timing of feeding in mice. This study determined the mechanisms for how feeding impacts day/night rhythms in the RR and QT intervals in mice. Telemetry was used to record electrocardiograms, core body temperature, and activity in mice during ad libitum-fed conditions and after inverting normal feeding behavior by restricting the timing of feeding to the light cycle. Light-cycle restricted feeding caused a simultaneous realignment of RR, QT, and PR intervals and body temperature to the new feeding time. Correcting the QT interval for body temperature eliminated the 24-hour rhythm in the QT interval. Estimating the impact of temperature on RR intervals did not account for the daily change in the RR interval during light-cycle restricted feeding. Cross-correlation analysis suggested daily rhythm in RR intervals correlated with heart rate variability measures but not activity. Injecting mice undergoing light cycle-restricted feeding with propranolol and atropine caused a complete loss in the 24-hour rhythm in the RR interval. We conclude that feeding behavior impacts body temperature and autonomic regulation of the heart to generate 24-hour rhythms in RR and QT intervals.

Robotic Surgery for Pediatric Choledochal Cysts: An American Case Series and Literature Review.

INTRODUCTION: Choledochal cysts are rare congenital biliary cystic dilations. The US incidence rate varies between 5 and 15 cases per 1,000,000 people. In contrast, Asians, which are a large subset of the population of Hawaii, have an incidence of approximately one in every 1000 births. We report our experience with robot-assisted laparoscopic surgical management with biliary reconstruction of choledochal cysts which to date is the largest American case series to be reported. MATERIALS AND METHODS: From 2006 to 2021, patients diagnosed with a choledochal cyst(s) at a tertiary children's hospital were retrospectively reviewed. Perioperative analysis was performed. Complications were defined as immediate, early, or late. The data underwent simple descriptive statistics. RESULTS: Nineteen patients underwent choledochal cystectomy and hepaticoduodenostomy. Thirteen underwent a robotic approach while the rest were planned laparoscopic. Eighteen of 19 were female with 15/19 of Asian descent. The ages ranged from 5 mo to 21 y. Presenting diagnoses included jaundice, primary abdominal pain, pancreatitis, and cholangitis. Sixty eight percent had type 1 fusiform cysts while the rest were type 4a. Operative time and length of stay for robotic versus laparoscopic were 321 versus 267 min and 8.2 versus 17.3 d, respectively. For the robotic group, there was one immediate complication due to peritonitis. One-year follow-up revealed two patients requiring endoscopic retrograde cholangiopancreatography with dilation/stenting for an anastomotic stricture. There were no anastomotic leaks. CONCLUSIONS: Robot-assisted laparoscopic choledochal cystectomy with hepaticoduodenostomy is associated with overall good outcomes with the most common long-term complication being anastomotic stenosis.

The Evolving Use of Magnets in Surgery: Biomedical Considerations and a Review of Their Current Applications.

The novel use of magnetic force to optimize modern surgical techniques originated in the 1970s. Since then, magnets have been utilized as an adjunct or alternative to a wide array of existing surgical procedures, ranging from gastrointestinal to vascular surgery. As the use of magnets in surgery continues to grow, the body of knowledge on magnetic surgical devices from preclinical development to clinical implementation has expanded significantly; however, the current magnetic surgical devices can be organized based on their core function: serving as a guidance system, creating a new connection, recreating a physiologic function, or utilization of an internal-external paired magnet system. The purpose of this article is to discuss the biomedical considerations during magnetic device development and review the current surgical applications of magnetic devices.

The role of the cardiomyocyte circadian clocks in ion channel regulation and cardiac electrophysiology.

Daily variations in cardiac electrophysiology and the incidence for different types of arrhythmias reflect ≈24 h changes in the environment, behaviour and internal circadian rhythms. This article focuses on studies that use animal models to separate the impact that circadian rhythms, as well as changes in the environment and behaviour, have on 24 h rhythms in heart rate and ventricular repolarization. Circadian rhythms are initiated at the cellular level by circadian clocks, transcription-translation feedback loops that cycle with a periodicity of 24 h. Several studies now show that the circadian clock in cardiomyocytes regulates the expression of cardiac ion channels by multiple mechanisms; underlies time-of-day changes in sinoatrial node excitability/intrinsic heart rate; and limits the duration of the ventricular action potential waveform. However, the 24 h rhythms in heart rate and ventricular repolarization are primarily driven by autonomic signalling. A functional role for the cardiomyocyte circadian clock appears to buffer the heart against perturbations. For example, the cardiomyocyte circadian clock limits QT-interval prolongation (especially at slower heart rates), and it may facilitate the realignment of the 24 h rhythm in heart rate to abrupt changes in the light cycle. Additional studies show that modifying rhythmic behaviours (including feeding behaviour) can dramatically impact the 24 h rhythms in heart rate and ventricular repolarization. If these mechanisms are conserved, these studies suggest that targeting endogenous circadian mechanisms in the heart, as well as modifying the timing of certain rhythmic behaviours, could emerge as therapeutic strategies to support heart function against perturbations and regulate 24 h rhythms in cardiac electrophysiology.

Timing of food intake in mice unmasks a role for the cardiomyocyte circadian clock mechanism in limiting QT-interval prolongation.

Cardiac electrophysiological studies demonstrate that restricting the feeding of mice to the light cycle (time restricted feeding or TRF) causes a pronounced change in heart rate and ventricular repolarization as measured by the RR- and QT-interval, respectively. TRF slows heart rate and shifts the peak (acrophase) of the day/night rhythms in the RR- and QT-intervals from the light to the dark cycle. This study tested the hypothesis that these changes in cardiac electrophysiology are driven by the cardiomyocyte circadian clock mechanism. We determined the impact that TRF had on RR- and QT-intervals in control mice or mice that had the cardiomyocyte circadian clock mechanism disrupted by inducing the deletion of Bmal1 in adult cardiomyocytes (iCSΔBmal1-/- mice). In control and iCSΔBmal1-/- mice, TRF increased the RR-intervals measured during the dark cycle and shifted the acrophase of the day/night rhythm in the RR-interval from the light to the dark cycle. Compared to control mice, TRF caused a larger prolongation of the QT-interval measured from iCSΔBmal1-/- mice during the dark cycle. The larger QT-interval prolongation in the iCSΔBmal1-/- mice caused an increased mean and amplitude in the day/night rhythm of the QT-interval. There was not a difference in the TRF-induced shift in the day/night rhythm of the QT-interval measured from control or iCSΔBmal1-/- mice. We conclude that the cardiomyocyte circadian clock does not drive the changes in heart rate or ventricular repolarization with TRF. However, TRF unmasks an important role for the cardiomyocyte circadian clock to prevent excessive QT-interval prolongation, especially at slow heart rates.

Telehealth Delivery of Outpatient Pediatric Surgical Care in Hawai'i: An Opportunity Analysis.

In the state of Hawai'i, nearly all pediatric surgical care is delivered on the main island of O'ahu at the state's primary tertiary children's hospital. Outpatient clinic visits require patients and families to travel to O'ahu. The direct and opportunity costs of this can be significant. The objective of this study was to characterize potential telehealth candidates to estimate the opportunity for telehealth delivery of outpatient pediatric surgical care. A retrospective chart review including all patients transported from neighbor islands for outpatient consultation with a pediatric surgeon on O'ahu over a 4-year period was performed. Each patient visit was examined to determine if the visit was eligible for telehealth services using stringent criteria. Direct, insurance-based costs of the travel necessary were then determined. Demographic data was used to characterize the patients potentially affected. A total of 1081 neighbor island patients were seen in the pediatric surgery clinic over 4 years. Thirty-one percent of these patients met criteria as candidates for telehealth visits. The majority of patients came from Hawai'i and Maui. Most patients were identified as Native Hawaiian or Asian. The average cost per trip was $112.53 per person, leading to a potential direct cost savings of $37,697 over 4 years. Over 30% of outpatient pediatric surgical encounters met stringent criteria as candidates for telehealth delivery of care. Given the significant number of patients that met our criteria, we believe there is an opportunity for direct, travel-based cost savings with the implementation of telehealth delivery of outpatient pediatric surgical care in Hawai'i.

The Pediatric Surgical Team: a Model for Increased Surgeon Index Case Exposure.

BACKGROUND/PURPOSE: High surgical volume for both surgeons and hospital systems has been linked to improved outcomes for many surgical problems, yet case volumes per pediatric surgeon are diminishing nationally in complex pediatric surgery. We therefore sought to review our experience in a geographically isolated setting where a surgical team approach has been used to improve per-surgeon exposure to index pediatric surgical cases. METHODS: As a surgical group, we incorporated a surgical team approach to complex pediatric surgical cases in 2010. We obtained institutional review board approval to review our pediatric surgeon index case volume experience. We then compared our surgeon experience to published surgical volumes for complex pediatric surgical cases. RESULTS: A surgical team approach (2 or 3 board certified pediatric surgeons/urologists working as co-surgeons or assistant surgeon) was used in the majority of cases for tracheoesophageal fistula/esophageal atresia (77%), congenital pulmonary airway malformation (73.5%), cloaca (75%), anorectal malformation (43.6%) biliary atresia (77.8%), Hirschsprung's disease (51.9%), congenital diaphragmatic hernia (67.6%), robotic choledochal cyst (100%), and complex oncology (adrenal tumors, neuroblastoma, Wilms tumor and Hepatoblastoma surgery) (85-100%). Over the 5-year period, surgeon index case exposure for all index pediatric surgical cases was above the published national median for pediatric surgeons, except for in splenic operations when contrasted to published experience. CONCLUSIONS: A surgical team approach to complex pediatric surgery may help maintain exposure to adequate index case volumes. This model may be useful for maintaining competence in geographically-isolated practice settings and low-volume pediatric hospitals that provide surgical care; the model has implications for systems development and workforce allocation within pediatric surgery. LEVEL OF EVIDENCE: 4.

Cryoanalgesia in Patients Undergoing Nuss Repair of Pectus Excavatum: Technique Modification and Early Results.

PURPOSE: The Nuss procedure for surgical correction of pectus excavatum often causes severe postoperative pain. Cryoanalgesia of intercostal nerves is an alternative modality for pain control. We describe our modification of the cryoICE™ probe that allows for nerve ablation through the ipsilateral chest along with early results utilizing this technique. METHODS: To allow for ipsilateral nerve ablation, a 20-French chest tube was cut and secured to the cryoICE probe, thus providing insulation for the malleable end of the probe. A 3-year retrospective review of patients undergoing Nuss repair at our institution was performed. Patients who received cryoanalgesia (cryo, n = 6) were compared with a historical control cohort who did not receive cryoanalgesia (nocryo, n = 13) during Nuss repair. Hospital length of stay, postoperative narcotic requirement (PNR), and highest postoperative pain score were collected. RESULTS: Both cohorts were similar regarding age, BMI, and pectus index. The cryo group had a significantly less PNR (6.4 versus 17.9 doses, P = .05) and was discharged on average >1 day earlier than nocryo patients (3.7 versus 2.2 days, P = .01). No complications occurred in either group. CONCLUSIONS: Our technique modification simplifies previously described approaches to intercostal nerve cryoablation. Patients undergoing this adjunct benefit with less PNR and a faster discharge time.

Possible etiologies of increased incidence of gastroschisis.

PURPOSE: Gastroschisis incidence has increased over the past decade nationally and in Hawaii. Pesticides have been implicated as potential causative factors for gastroschisis, and use of restricted use pesticides (RUPs) is widespread in Hawaii. This study was conducted to characterize gastroschisis cases in Hawaii and determine whether RUP application correlates with gastroschisis incidence. METHODS: Gastroschisis patients treated in Hawaii between September, 2008 and August, 2015 were mapped by zip code along with RUP use. Spatial analysis software was used to identify patients' homes located within the pesticide application zone and agricultural land use areas. RESULTS: 71 gastroschisis cases were identified. 2.8% of patients were from Kauai, 64.8% from Oahu, 16.9% from Hawaii, 14.1% from Maui, and 1.4% from Molokai. RUPs have been used on all of these islands. 78.9% of patients lived in zip codes overlapping agricultural land use areas. 85.9% of patients shared zip codes with RUP-use areas. CONCLUSION: The majority of gastroschisis patients were from RUP-use areas, supporting the idea that pesticides may contribute to the development of gastroschisis, although limited data on specific releases make it difficult to apply these findings. As more RUP-use data become available to the public, these important research questions can be investigated further.

Magnetic Compression Stricturoplasty For Treatment of Refractory Esophageal Strictures in Children: Technique and Lessons Learned.

INTRODUCTION: Esophageal stricture is the most common complication following repair of esophageal atresia (EA). In general, these strictures are successfully managed using endoscopic techniques including bougie and balloon dilation, stenting, and chemotherapeutic agent application. If these techniques are unsuccessful, patients require segmental esophageal resection and reanastomosis or esophageal replacement. Magnetic compression anastomosis has been described in children. Herein we report our experience with magnetic compression stricturoplasty to treat refractory strictures after EA repair. METHODS: We reviewed our experience using magnets to treat refractory strictures in 2 patients. Both patients failed multiple standard interventions. Because of near complete esophageal obstruction, both patients were candidates for esophageal replacement or segmental resection/anastamosis. In both patients, we applied neodymium-iron-boron magnets using fluoroscopic and endoscopic guidance. RESULTS: The magnets were successfully positioned in both cases. Magnets were left in place for 7 and 10 days allowing for gradual compression stricturoplasty/anastamosis. Upon removal of the magnets, recanalization was visualized endoscopically and self-expanding stents were placed. There were no leaks or significant early complications. By 31 months post-magnetic stricturoplasty, both patients achieved durable esophageal patency without dysphagia. CONCLUSION: Magnetic stricturoplasty was successful at establishing early patency of the esophagus in 2 patients with recalcitrant EA strictures. Fundamental knowledge of magnetism was critical in configuring magnet arrays for surgery. In both cases, early follow-up is promising. Further follow-up will define the long-term success of this technique.

Thoracoscopic pleural clipping for the management of congenital chylothorax.

PURPOSE: Medical management of congenital chylothoraces consists of total parental nutrition and tube thoracostomy. However, these infants are exposed to significant fluid shifts and the related leukopenia carries a high infection risk. The purpose of this review is to describe the technique of parietal pleural clipping as a surgical treatment of congenital chylothorax. METHODS: The medical records of all patients with a chylothorax diagnosis during the study period of January 2002 to April 2014 were retrospectively reviewed. RESULTS: Six of 14 infants identified underwent thoracoscopic parietal pleural clipping to disrupt the pleural lymphatic channel flow as visualization of the thoracic duct and lymphatics was not possible. Nearly all surgical patients had bilateral disease (5/6). Resolution of chylous leakage was dramatic following parietal clipping. In the surgical patients, chest tube output 2 days prior to surgery averaged 86.96 ml/kg/day. After parietal clipping, chest tube output dropped to an average of 6.5 ml/kg/day on post op day 2. Thereafter, chest tube output remained low to negligible and chest tubes were removed variably as enteral feeds were started. CONCLUSIONS: We describe a straightforward technique of thoracoscopic parietal pleural clipping as a safe and successful option for treatment of congenital chylothoraces.

The feasibility of using haptic devices to engage people with chronic traumatic brain injury in virtual 3D functional tasks.

BACKGROUND: The primary aim of this study was to assess the level of engagement in computer-based simulations of functional tasks, using a haptic device for people with chronic traumatic brain injury. The objectives were to design functional tasks using force feedback device and determine if it could measure motor performance improvement. METHODS: A prospective crosssectional study was performed in a biomedical research facility. The testing environment consisted of a single, interactive, stylus-driven computer session navigating virtual scenes in 3D space. Subjects had a haptic training session (TRAIN) and then had three chances to perform each virtual task: (i) remove tools from a workbench (TOOL), (ii) compose 3 letter words (SPELL), (iii) manipulate utensils to prepare a sandwich (SAND), and (iv) tool use (TUSE). Main Outcome Measures included self-report of engagement in the activities, improved performance on simulated tasks and observer estimate as measured by time to completion or number of words completed from baseline, correlations among performance measures and self-reports of boredom, neuropsychological symptom inventory (NSI), and The Purdue Peg Motor Test (PPT). RESULTS: Participants were 19 adults from the community with a 1 year history of non-penetrating traumatic brain injury (TBI) and were able to use computers. Seven had mild, 3 moderate and 9 severe TBIs. Mean score on the Boredom Proneness Scale (BPS): 107 (normal range 81-117); mean NSI:32; mean PPT 54 (normal range for assembly line workers >67). Responses to intervention: 3 (15%)subjects did not repeat all three trials of the tasks; 100% reported they were highly engaged in the interactions; 6 (30%) reported they had a high level of frustration with the tasks, but completed them with short breaks. Performance measures: Comparison of baseline to post training: TOOL time decreased by (mean) 60 sec; SPELL increased by 2.7 words; TUSE time decreased by (mean) 68 sec; and SAND time decreased by (mean) 72 sec. PPT correlated with TOOL (r=-0.65, p=0.016) and TUSE time (r=-0.6, p=0.014). SPELL correlated with Boredom score (r=0.41, p=0.08) and NSI (r=-.49, p=0.05). CONCLUSION: People with chronic TBI of various ages and severity report being engaged in using haptic devices that interact with 3D virtual environments. Haptic devices are able to capture objective data that provide useful information about fine motor and cognitive performance.

Negative pressure dressing assisted healing in pediatric burn patients.

BACKGROUND/PURPOSE: Pediatric burn patients traditionally require multiple dressing changes and significant amounts of narcotics. Negative pressure dressings (NPDs) have emerged as an effective wound therapy that may represent an alternative primary dressing for these patients. METHODS: This is a single institution, retrospective study of pediatric burn patients treated with NPDs over a defined 2 year period. Twenty-two patients were identified and their charts reviewed for age, sex, mode of injury, location of injury, degree of burn, length of stay, length of dressing required, number of dressing changes, and narcotic use between dressing changes. RESULTS: The average patient was 3.5 years old (range of 8 months to 10 years old) with partial thickness burns involving 8.5% (range 3-18%) body surface area. The average treatment regimen was 3.5 dressing changes more than 6.6 days, with a mean hospital stay of 9.6 days. The average child received 9.4 total doses of delivered narcotics during their inpatient care. DISCUSSION: The use of NPD in pediatric burn patients does require sedation and narcotics which limits its usefulness in the general pediatric burn population. Yet, they adhere well and stay in place even on active children, they capture and quantify fluid losses, they only require changes every 2-4 days and promote the adherence of split thickness skin grafts making them useful in various clinical situations. CONCLUSIONS: NPDs are a viable option for both partial and full thickness burns in pediatric patients that do not require transfer to a burn unit. NPDs may be advantageous in highly active children, those with extensive fluid losses, those that require sedation for dressing changes and those that will require grafting.

Increased risk of cardiovascular perforation during ECMO with a bicaval, wire-reinforced cannula.

PURPOSE: Cardiac or major vascular perforation is a rare but serious risk of ECMO. We sought to determine if perforation rates are related to cannula design. METHODS: We utilized three methods to evaluate perforation on ECMO. 1. The ELSO registry was queried to establish the historical rate of hemorrhagic pericardial tamponade. 2. ELSO centers were surveyed regarding cannula related perforation events and brands of cannulas used over a four year time period (January 2008-March 2012). 3. The FDA's MAUDE database was reviewed looking for adverse events related to ECMO cannulas. RESULTS: The historical rate of hemorrhagic pericardial tamponade in the ELSO registry was 0.53% (~1985-2010, ELSO registry). In the survey there were eleven reports of cannula-related perforation, 0.74% (11/1482 p-value=0.29) at 7 different ELSO centers with 23 ELSO centers responding (17% response rate). The incidence of perforation was much higher for the wire-reinforced bicaval design 3.6% (10/279) as compared to catheters designed for the atrial position, 0.1% (1/1203, p-value<0.0001). Review of the FDA's MAUDE database revealed 19 adverse events related to the bicaval cannula design, 16 of which were hemorrhagic pericardial effusions or tamponade. CONCLUSION: These findings suggest a relatively high rate of cardiac perforation associated with the dual lumen bicaval cannula. This may be related to inherent differences in cannula design or the IVC positioning required by the design.

First STEPs: serial transverse enteroplasty as a primary procedure in neonates with congenital short bowel.

BACKGROUND: Since its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty (STEP) has been increasingly used as the surgical treatment of choice for patients with refractory short bowel syndrome (SBS). While primary STEP for the treatment of congenital conditions was proposed in the original description of the procedure, emphasis was placed on a delayed or staged approach to these patients. To date, a comprehensive review of the outcomes from this sub-population has not been reported by the International STEP Data Registry. METHODS: A retrospective review of the International STEP Data Registry was performed to identify all patients who underwent STEP as a primary operative procedure for the treatment of congenital SBS. Changes in pre- and post-STEP values were assessed using paired t-tests with significance set at p<0.05. Data are presented as mean ± standard deviation. RESULTS: Fifteen patients underwent primary STEP for congenital SBS between September 1, 2004, and April 10, 2012. Thirteen patients had follow-up information available. Causes of congenital SBS included closing gastroschisis, small bowel atresia, and midgut volvulus. Twelve patients had pre- and post-STEP bowel measurements taken. Average pre- and post-STEP bowel lengths were 32 ± 16 cm and 47 ± 22 cm, respectively. Intestinal length was increased by a mean of 15 ± 12 cm for a relative small bowel length increase of 50.4 ± 27.3% (p<0.001). Only one patient required an ostomy at the time of primary STEP. A second patient required a temporary ostomy at 3months of age that was later closed. There was one death from intestinal failure associated liver disease (IFALD). Another patient experienced IFALD progression and required liver and intestinal transplantation. The most commonly reported complication following primary STEP was obstruction or bowel re-dilatation requiring additional operative interventions. Nine patients underwent second STEP procedures under these circumstances. Eight patients remain dependent on parenteral nutrition, while three patients achieved enteral autonomy. CONCLUSIONS: Primary STEP is a feasible and safe surgical option for the treatment of congenital conditions resulting in SBS. Primary STEP establishes early bowel continuity, creates intestinal length from congenitally dilated bowel, and appears to obviate the need for interval stomas and their associated loss of bowel length in neonates with congenital SBS. However, with recent changes in SBS management emphasizing intestinal rehabilitation, additional studies are needed to assess the long-term impact on intestinal adaptation of STEP performed in the neonatal period prior to adoption of this technique.

Appendicitis as the presenting manifestation of Kawasaki disease.

In cases of Kawasaki's disease (KD) presenting as acute surgical abdomen, rarely has the presence of acute appendicitis been found. We report two cases of histologically confirmed acute appendicitis in the presence of KD and a review of the literature as it pertains to acute abdomen and atypical presentations of KD.

Successful treatment of rare-earth magnet ingestion via minimally invasive techniques: a case series.

Cases of rare-earth magnet ingestions have been increasingly reported in the literature. However, these descriptions have focused on the severity of the injuries, rather than the clinical presentation and/or therapeutic approach. We report a series of eight children, ranging in age from 2 to 10 years, who ingested powerful rare-earth magnets. The rare-earth magnets were marketed in 2009 under the trade name Buckyballs(®) (Maxfield & Oberton, New York, NY). They are about 5 mm in size, spherical, and brightly colored, making them appealing for young children to play with and place in their mouths. Three children presented within hours of ingestion, and the magnets were successfully removed via endoscopy in two, whereas the third child required laparoscopy. No fistulas were found in these children. A fourth child presented 2 days after ingestion with evidence of bowel wall erosion, but without fistula formation; the magnets were removed via laparoscopy. A fifth child ingested nine magnets in a ring formation, which were removed via colonoscopy without evidence of injury or fistula formation. The three remaining children presented late (5-8 days after ingestion) and were found to have associated fistulas. They were treated successfully with a combination of endoscopy and laparoscopy with fluoroscopy. None of the children in our series required an open surgical procedure. All children were discharged home without complications. This case series highlights the potential dangers of rare-earth magnet ingestion in children. Our experience suggests that prompt intervention using minimally invasive approaches can lead to successful outcomes.