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.

Decrease in injuries from fireworks in Hawaii: associations with a county policy to limit access.

OBJECTIVE: To examine trends in fireworks-related injuries (FRI) before and after enactment of an ordinance to limit access in the City and County of Honolulu (the island of Oahu). METHODS: Surveillance of FRI treated in all emergency departments in the state, for 18 new year's periods (31 December through 1 January) from 2004 to 2021. Prelaw (2004 to 2011) and postlaw (2012 to 2021) number of FRI were compared, by patient age and county. RESULTS: The average annual number of FRI for all ages decreased significantly in Oahu, from 74 during the prelaw period to 27 during the postlaw period (p<0.01), but not in the remaining neighbour islands (p=0.07). Decreases were particularly evident for Oahu paediatric patients (under 18 years), among whom FRI declined from 42 to 10 per year (p<0.01). FRI were approximately halved for older Oahu patients and neighbour island paediatric patients. CONCLUSIONS: Legislation requiring permits for a specified number and type of fireworks, and limiting access to persons 18 years and older was associated with significant decreases in FRI in the City and County of Honolulu.

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.

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.

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.

Distension enterogenesis: increasing the size and function of small intestine.

INTRODUCTION: The purpose of this study is to evaluate the feasibility of using saline infusion to lengthen small bowel while preserving intestinal enzymatic function. METHODS: Male Sprague-Dawley rats had a 3-cm jejunal segment taken out of continuity. A catheter was inserted in the proximal end, and the distal end was oversewn. Continuous infusion of saline into the isolated jejunal segment was started 2 weeks postoperatively. Segments were harvested 1 week later. Segment weights and lengths were measured preoperatively and at the time of harvest. Histology of harvested segments was performed. Alkaline phosphatase (ALP) and lactase assays were performed. Comparisons were made with normal jejunum from control animals. RESULTS: A 32% increase in length was achieved with saline distension of small intestine. The segment weight to length ratio was significantly increased by saline distension; however, the total protein-to-weight ratio was unchanged. Specific activities of ALP and lactase were not affected by saline distension. Because of the increased length and weight of the distended jejunal segments, total segment activities for both enzymes were significantly increased. CONCLUSIONS: Saline infusion appears to be a viable method for increasing small intestinal length without compromising enzymatic function. This phenomenon may provide a new method for the treatment of patients with short bowel syndrome in the future, and further study is warranted.

Enterogenesis by mechanical lengthening: morphology and function of the lengthened small intestine.

BACKGROUND/PURPOSE: The purpose of this study is to evaluate the function of the mechanically lengthened small intestine. METHODS: A jejunal segment was separated from intestinal continuity in rats. A screw was inserted into its proximal end, and the distal end was oversewn. The screw was advanced into the jejunal segment by 5 mm every other day. The jejunal segments were retrieved after 2 weeks. The length, weight, muscular thickness, alkaline phosphatase, and lactase activities of the jejunal segments were determined. Comparisons were made among normal jejunum, isolated jejunal segments without lengthening, and lengthened jejunal segments. RESULTS: Jejunal segments doubled in length after gradual mechanical stretching compared with the normal and isolated controls. The thickness of the muscular layer increased in both the isolated and lengthened groups. The total activity of alkaline phosphatase increased in jejunal segments that were lengthened, whereas the total lactase activity remained the same. CONCLUSIONS: Mechanical force is a viable method for increasing intestinal length while preserving the intestinal function. This phenomenon may provide a new method for the treatment of patients with short bowel syndrome.