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.

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.

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.

The Assessment and Management of Biliary Atresia in Hawai'i, 2009-2023.

Although biliary atresia (BA) is a rare neonatal disorder, it remains the leading cause of pediatric end-stage liver disease. Early diagnosis of BA and treatment with the Kasai procedure can significantly reduce the need for pediatric liver transplant. Current data suggests that performing the Kasai procedure at 30-45 days of life is associated with longer native liver survival rates and reduction of the need for liver transplant. The incidence rate of BA in the state of Hawai'i is nearly double the incidence rate in the continental US. International studies have demonstrated that screening programs for BA reduce the age at diagnosis and treatment. However, there has been no statewide analysis on the ages at diagnosis or at Kasai, nor does a statewide screening program for BA exist. The purpose of this study is to review the age of diagnosis and treatment of BA to determine if the current practice in Hawai'i is in line with the published data. A retrospective chart review of all patients diagnosed with BA at the state's primary children's hospital was performed (2009-2023) and 19 patients who underwent the Kasai procedure were identified. The mean age at diagnosis is 71.4 days (n=19) and the mean age at Kasai procedure is 72.0 days (n=19). Both the average age at diagnosis and treatment for BA in Hawai'i is significantly higher than published data suggesting best outcomes at 30-45 days of life. This review suggests that the implementation of a statewide screening program for BA in Hawai'i is warranted.

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.

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.

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.

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.

Laparoscopic drainage of intraabdominal abscess after appendectomy: an alternative to laparotomy in cases not amenable to percutaneous drainage.

PURPOSE: Postoperative abscesses after appendectomy occur in 3% to 20% of cases and are more common in cases of perforated appendicitis. Smaller abscesses are often amenable to antibiotic therapy, but surgical drainage remains the mainstay of treatment for larger collections. Surgical options generally include percutaneous drainage and open laparotomy. Laparoscopic drainage of these abscesses has not been well characterized in the pediatric population. OBJECTIVE: The aim of this study was to describe our experience with laparoscopic drainage of postappendectomy abscesses that were not amenable to percutaneous drainage. METHODS: This study is a retrospective review of all pediatric patients who underwent laparoscopic appendectomy for acute appendicitis at a tertiary pediatric medical center during a 4-year period (2006-2009). The review focuses on patients who developed abscesses after appendectomy, were unable to undergo percutaneous drainage, and were treated with laparoscopic abscess drainage. RESULTS: Twelve patients (7 male and 5 female) underwent laparoscopic drainage of postappendectomy abscesses. The mean age was 8.5 years old (range, 3-14 years). A clinical diagnosis of postoperative abscess was made when fevers, pain, and leukocytosis persisted despite broad-spectrum antibiotics. Computed tomography was performed in all patients. Abscesses ranged between 3 and 11 cm in size. The mean length of time between initial appendectomy and drainage procedure was 10 days. There were no complications specifically related to the laparoscopic drainage procedure. The mean length of the drainage procedure was 77 minutes (range, 30-196 minutes). The mean hospital length of stay after laparoscopic drainage was 6.5 days (range, 3-13 days) with patients maintained on intravenous antibiotics until afebrile and without leukocytosis. CONCLUSION: Laparoscopic drainage is a safe and effective alternative for intraabdominal abscesses that occur after laparoscopic appendectomy. We recommend it as an alternative to open laparotomy when percutaneous drainage is not an option.

Thoracoscopic segmentectomy for treatment of congenital lung malformations.

PURPOSE: Congenital lung malformations (CLM) predispose patients to recurrent respiratory tract infections and pose a rare risk of malignant transformation. Although pulmonary lobectomy is the most common treatment of a CLM, some advocate segmental resection as a lung preservation strategy. Our study evaluated lung-preserving thoracoscopic segmentectomy as an alternative to lobectomy for CLM resection. METHODS: We conducted a retrospective review of patients who underwent thoracoscopic segmentectomy for CLM from 2007 to 2010. RESULTS: Fifteen patients underwent thoracoscopic segmentectomy for CLM. There were five postoperative complications: three asymptomatic pneumothoraces and a small air leak that resolved without intervention. One patient developed a bronchopulmonary fistula requiring thoracoscopic repair. At follow-up, all patients are asymptomatic. One patient has a small amount of residual disease on postoperative computed tomography (CT), and re-resection has been recommended. CONCLUSIONS: Thoracoscopic segmentectomy for CLM is a safe and effective means of lung parenchymal preservation. The approach spares larger airway anatomy and has a complication rate that is comparable with that of thoracoscopic lobectomy. Residual disease can often only be appreciated on postoperative CT scan and may require long-term follow-up or reoperation in rare cases. This lung preservation technique is best suited to smaller lesions.

Thoracoscopic treatment of pediatric lung abscesses.

OBJECTIVE: Lung abscesses in the pediatric population are relatively rare. We present our consecutive series of thoracoscopically treated pediatric lung abscesses. METHODS: A retrospective review of children who underwent thoracoscopic drainage of intraparenchymal lung abscesses between October 2006 and January 2009 at a tertiary referral center. All patients had associated parapneumonic empyema and underwent drainage of the abscess concurrently with thoracoscopic treatment of the empyema. RESULTS: Eleven children (4 boys and 7 girls) had thoracoscopic intervention for lung abscesses. A total of seventeen abscesses were drained. All procedures were completed thoracoscopically. There were no mortalities or long-term bronchopleural fistulas. No child required a formal thoracotomy, lung resection or a second operation. Mean duration of postoperative hospital stay was 11.0 days (range, 3-36). Mean length of stay was 19.5 days (range, 6-77 days). Mean duration of postoperative chest tube was 3.6 days (range, 2-8). Mean length to defervescence was 4.8 days (range, 1-11 days). Mean duration of postoperative antibiotics was 23.6 days (range, 3-56). Eight children had organisms identified from intraoperative cultures. CONCLUSIONS: Thoracoscopic drainage of pediatric lung abscesses is a viable and safe treatment option. Thoracoscopic abscess drainage is associated with minimal morbidity and may result in faster recovery and a shorter course of antibiotics.

Thoracoscopic decortication for advanced pediatric empyema.

BACKGROUND: Although tube thoracostomy with fibrinolytic agents and video-assisted thoracoscopic (VATS) decortication are relatively equivalent in the treatment of empyema with regard to time to patient defervescence and hospital discharge, the potential for greater benefit with VATS decortication in the setting of advanced empyema has not been explored fully. This paper describes our transition from a policy of drainage and antibiotics to primary operative management with VATS. We wanted to assess the safety and efficacy of primary operative management as a first-line treatment for advanced disease. METHODS: A retrospective review was conducted of 25 patients treated for stage 3 or 4 empyema. The primary endpoints were morbidity and death. The secondary endpoints were conversion to an open procedure, time to defervescence, and length of hospital stay. RESULTS: The morbidity rate was 16% with no deaths. No patient required conversion to open decortication. The mean time to defervescence was 3.2 days, and on average, patients were discharged on postoperative day 9. Patients were discharged home earlier when managed primarily with VATS. CONCLUSIONS: Video-assisted thoracoscopic decortication is a safe and effective treatment for pediatric stage 3 or 4 empyema.

Solid cervical ectopic thymus in an infant.

Cervical ectopic thymus presenting as a neck mass is rare in a neonate. Just more than 100 cases have been reported in the literature with less than 10% occurring in infants. We report a case of solid cervical ectopic thymus in an asymptomatic 2-month-old boy. We review the literature and discuss the embryology, pathophysiology, diagnosis, and management of an infantile ectopic thymus.

Neonatal thoracoscopic repair of congenital diaphragmatic hernia: selection criteria for successful outcome.

BACKGROUND/PURPOSE: Complications of open conversion, hypercarbia, and intestinal injury have plagued minimally invasive approaches to congenital diaphragmatic hernia (CDH) repair in neonates. To safely begin using minimally invasive techniques for neonatal CDH repair, we formulated preoperative selection criteria and operative techniques that would enhance chances for successful thoracoscopic primary diaphragm repair and uncomplicated outcome. METHODS: During the period from January 2003 to October 2004, neonates were selected for thoracoscopic CDH repair using anatomic and physiologic criteria. Anatomically, all patients were required to have stomach in the abdomen by radiography. Physiologically, all patients were required to be on minimal ventilator support with preoperative ventilator peak inspiratory pressures in the low 20s mm Hg. No patient could have clinical evidence of pulmonary hypertension at the time of surgery. Thoracoscopic CDH repair was performed using 3 trocars (3 and 5 mm). The hernia contents were reduced into the abdomen using 5-mm Hg insufflation, and the diaphragms were repaired primarily using interrupted 3-0 Ethibond simple sutures (Ethicon, Inc, Piscataway, NJ). Posterolateral diaphragm stitches were passed around the posterolateral ribs and tied extracorporeally. RESULTS: Thirty neonates with CDH were admitted to Children's Hospital Boston and Vanderbilt Children's Hospital during the study period. Eight patients (27%) met selection criteria and 7 underwent thoracoscopic CDH repair. Primary diaphragmatic repair was successfully accomplished thoracoscopically in all neonates without perioperative complication. Preoperative anatomic criteria correlated accurately with intact esophageal hiatus and primary diaphragm repair. Physiologically, each patient tolerated intrathoracic insufflation and CDH repair without clinical pulmonary hypertension or blood pressure lability. Three patients had intraoperative respiratory acidosis that was reversed with ventilator changes. Operative times averaged 152 minutes and ranged from 212 to 106 minutes. Postoperative mechanical ventilation ranged from 0 to 7 days, and the length of hospitalization ranged from 5 to 32 days. Longest follow-up has been 17 months. One patient required reoperation for recurrent CDH at 10 months after repair, but there have been no other long-term complications. CONCLUSIONS: Neonatal thoracoscopic CDH repair is safe in selected patients who have good preoperative pulmonary function and anatomy amenable to primary diaphragmatic repair. A wider range of neonates may be acceptable for thoracoscopic CDH repair with increasing surgical experience.

Contouring buttock reconstruction after sacrococcygeal teratoma resection.

PURPOSE: Massive sacrococcygeal teratomas are typically resected and closed in a "chevron" fashion. The resultant scar may leave protuberant "dog ears" and extend across and below the infragluteal creases down onto the posterior thighs, causing undesirable buttock deformity. Given the redundant skin often available, the authors sought to develop a closure technique to minimize deformity and unpleasant scars. METHODS: At the time of resection of 2 sacrococcygeal teratomas, attention was directed to minimizing redundant skin, restoring normal buttock contour, and avoiding scars crossing the infragluteal crease. After properly securing the anal location, serial polygonal skin excisions were performed, working the excess tissue centrally rather than peripherally, leaving 2 right-angled scars on each buttock. RESULTS: Each infant underwent successful reconstruction with a normal buttock contour without redundancy. All scars on the buttocks can be easily covered by bathing attire. CONCLUSIONS: The excess skin expanded by large sacrococcygeal teratomas affords an opportunity to apply straightforward tissue rearrangement principles to reconstruct the buttocks with a normal contour and hidden scars.

The split abdominal wall muscle flap--a simple, mesh-free approach to repair large diaphragmatic hernia.

PURPOSE: The authors present a technique to repair large diaphragmatic hernias that is simple, can be done primarily, and offers a durable closure with living tissue. METHODS: A review of congenital diaphragmatic hernias was performed for the period between January 1991 and August 2000. Repair types included primary repair, synthetic patch, or a split abdominal wall muscle flap. The split abdominal wall muscle flap is performed by downward rotation of the internal oblique and transversalis abdominal wall muscles. This repair requires that the initial subcostal incision be positioned at least 4 to 5 cm below the costal margin, low enough to insure an adequate length of muscle will be available to fill the defect. RESULTS: Eight of 158 children with congenital diaphragmatic hernia underwent repair by abdominal wall muscle flap. Five of 8 had complete agenesis. Five survived the newborn period. Of the survivors, 4 of 5 required an additional surgical procedure, but none have had a recurrent hernia. CONCLUSIONS: The split abdominal wall muscle flap is an effective technique to close large diaphragmatic hernias. The repair was carried out successfully in 8 patients with massive defects. In the surviving patients, the repair has proven to be a durable restoration of the diaphragm.