Continuous local anaesthetic wound infusion of bupivacaine for postoperative analgesia in neonates: a randomised control trial (CANWIN Study).

OBJECTIVE: To determine the effect of continuous wound infusion of local anaesthetic drug (bupivacaine) on total amount of systemic opioid use in the first 72 hours in newborn infants undergoing laparotomy. DESIGN: A two-arm parallel, open-label randomised controlled trial. SETTING: A quaternary newborn intensive care unit. PATIENTS: Infants>37 weeks of gestation undergoing laparotomy for congenital or acquired abdominal conditions. INTERVENTIONS: Continuous wound infusion of local anaesthetic (bupivacaine) for the first 72 hours along with systemic opioid analgesia (catheter group) or only systemic opioid analgesia (opioid group). MAIN OUTCOME: Total amount of systemic opioid used within the first 72 hours post laparotomy. RESULTS: The study was underpowered as only 30 of the expected sample size of 70 infants were enrolled. 16 were randomised to catheter group and 14 to opioid group. The two groups were similar at baseline. There was no significant difference between the groups for the primary outcome of median total systemic opioid use in the first 72 hours post laparotomy (catheter 431.5 µg/kg vs opioid 771 µg/kg, difference -339.5 µg/kg, 90% CIhigh 109, p value 0.28). There was no significant difference between the groups for any of the secondary outcomes including pain scores, duration of mechanical ventilation, time to reach full feeds and duration of hospital stay. There were no adverse events noted. CONCLUSION: Continuous wound infusion of local anaesthetic along with systemic opioid analgesia is feasible. The lack of a difference in total systemic opioid use in the first 72 hours cannot be reliably interpreted as the study was underpowered. TRIAL REGISTRATION NUMBER: ACTRN12610000633088.

Liver transplantation in children with inborn errors of metabolism: 30 years experience in NSW, Australia.

BACKGROUND: Inborn errors of metabolism (IEM) are a diverse group of genetic disorders that can result in significant morbidity and sometimes death. Metabolic management can be challenging and burdensome for families. Liver transplantation (LT) is increasingly being considered a treatment option for some IEMs. IEMs are now considered the second most common reason for pediatric LT. AIM: To review the data of all children with an IEM who had LT at The Children's Hospital at Westmead (CHW), NSW, Australia between January 1986 and January 2019. METHODS: Retrospective data collected from the medical records and genetic files included patient demographics, family history, parental consanguinity, method of diagnosis of IEM, hospital and intensive care unit admissions, age at LT, graft type, clinical outcomes and metabolic management pre and post-LT. RESULTS: Twenty-four LT were performed for 21 patients. IEM diagnoses were MSUD (n = 4), UCD (n = 8), OA (n = 6), TYR type I (n = 2) and GSD Ia (n = 1). Three patients had repeat transplants due to complications. Median age at transplant was 6.21 years (MSUD), 0.87 years (UCD), 1.64 years (OA) and 2.2 years (TYR I). Two patients died peri-operatively early in the series, one died 3 months after successful LT due to septicemia. Eighteen LTs have been performed since 2008 in comparison to six LT prior to 2008. Dietary management was liberalized post LT for all patients. CONCLUSIONS: Referral for LT for IEMs has increased over the last 33 years, with the most referrals in the last 10 years. Early LT has resulted in improved clinical outcomes and patient survival.

Pediatric third liver transplantation-A single-center experience.

BACKGROUND: Pediatric retransplantation is an accepted practice for graft failure and complications in Australasia. As 15% of children require a third transplant, this is a growing cohort with limited data in the literature. METHODS: We review nine patients from the commencement of our transplantation program in 1986 up to 2020 assessing demographics, prognosis, and outcome measures. RESULTS: Third transplant patient survival was comparative to first and second transplant patient survival at 5 years. All deaths were within the post-operative period and secondary to sepsis. Operative times and transfusion volumes were increased at third transplant (1.8 and 4.5 times compared to first transplant, respectively). Learning difficulties and psychological disturbances were prevalent (83% and 66.6%, respectively). CONCLUSIONS: While recent mortality outcomes appear comparable to undergoing a second liver transplant, third transplant operations were more complex. Neurological impairment and psychological disturbance appear to be prevalent and need to be considered in pre-transplant counseling.

Minimally invasive surgical treatment of pilomatrixoma.

BACKGROUND: Pilomatrixoma is a benign skin tumour often presenting as a firm irregular mass in the paediatric population. The most common site is on the head and neck. Traditionally, a wide local excision has been the method of management. We propose an incision and curettage (I&C) technique for an improved cosmetic outcome. METHODS: A retrospective review of children who underwent I&C for pilomatrixoma was done between January 2010 and June 2020. The I&C technique involved making a small incision over or near the lesion in a discrete location such as behind the hairline and removing the tumour piecemeal. Four to six weeks of routine post-operative follow-up was conducted. Patients and families were also subsequently contacted via a survey to assess for late recurrence, any other complications and ascertain their level of satisfaction with the outcome. RESULTS: Twenty lesions were removed in 11 patients over this time with a female predominance (seven) and most lesions were on the face (11). No patients had a recurrence in a mean follow-up time of 6 years (1-10 years). All parents are very satisfied with the cosmetic result. CONCLUSION: I&C may be an effective and cosmetically pleasing method to removing pilomatrixoma.

Complications of surgical mesh patches after kidney transplantation in children-A case series.

BACKGROUND: Abdominal compartment syndrome after kidney transplantation in pediatric recipients is a recognized complication relating to size discrepancy requiring abdominal wall closure over a large adult allograft. In order to circumvent this problem, our center implemented use of a surgical mesh, Surgisis® (Cook Surgical, Bloomington, IN), for abdominal wall closure in very small children to increase the surface covering over the organ and prevent compression. In this article, we report on the complications encountered following the use of these mesh patches. METHODS: A retrospective case review was conducted of all pediatric kidney transplants from September 2006 to December 2018 and divided into abdominal wall closure with and without implantation of Surgisis® mesh patch. Review of clinical notes was performed to identify information with respect to clinical course and post-operative outcomes. RESULTS: A surgical mesh patch was used in 7 pediatric recipients, of which 5 (71%) presented with post-operative complications. Three recipients were found to have bowel obstruction related to the surgical patch, necessitating bowel resection in one child. In addition, three children developed large serous fluid collections between the subcutaneous layers and the surgical mesh, requiring surgical drainage in two. CONCLUSIONS: In view of these findings, we recommend close surveillance for potential complications in this cohort. Future research is needed to explore the safety of different approaches to achieve abdominal wall closure in this group.

Portosystemic shunt for portal hypertension after Kasai operation in patients with biliary atresia.

PURPOSE: Many biliary atresia (BA) patients will eventually develop liver failure even after a successful Kasai portoenterostomy. A common complication of long-term BA survivors with their native liver is problematic portal hypertension. The aim of this study was to defend the view that portosystemic shunts can delay or negate the need for transplantation in these children. METHODS: A retrospective single center review of the efficacy of portosystemic shunts in BA patients after a successful Kasai portoenterostomy was conducted. RESULTS: From 1991 to 2017, 11 patients received portosystemic shunts. Median age of Kasai operation was 48 (36-61) days. Shunts were performed at the median age of 6.2 (4.1-6.8) years. Three of these eleven patients required subsequent liver transplantation. OS at 5 and 10 years were 90.9% and 81.8%, respectively. TFS at 5 and 10 years were 90.9% and 72.7%, respectively. Long-term complications included mild encephalopathy in 2 patients, hypersplenism in 3, and cholestasis in 1. CONCLUSION: Portosystemic shunt for the treatment of portal hypertension in carefully selected BA patients is an effective option in delaying or negating the need for liver transplantation.

Double barrel enteroplasty for the management of short bowel syndrome in children.

BACKGROUND: Currently, there are two well-established methods of bowel lengthening in patients with short bowel syndrome (SBS)-longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) [1-4]. Both procedures may carry a high reported morbidity and mortality of 30.2% and 14.4%, respectively [5]. We report the outcomes of a novel technique: double barrel enteroplasty (DBE) for autologous intestinal reconstruction. METHODS: We performed a retrospective review of all ten patients who underwent DBE at our institution since 2011. All patients have SBS and were dependent on parenteral nutrition (PN) at the time of surgery. Etiologies were gastroschisis (n = 4), bowel atresia (n = 3), necrotising enterocolitis (n = 1), volvulus (n = 1), and near-total intestinal aganglionosis (n = 1). Patient survival, complications, and subsequent enteral autonomy were evaluated. RESULTS: All patients are alive with normal liver function. Five children achieved enteral autonomy, while the remaining are on weaning PN. There was no bleeding, anastomotic leak, perforation, infective complications, or intestinal necrosis. No patient has required a liver and/or intestinal transplant. CONCLUSIONS: Double barrel enteroplasty is technically feasible and safe. It has similar efficacy and may have fewer complications when compared with other methods of autologous intestinal reconstruction.

Outcomes for children after second liver transplantations are similar to those after first transplantations: a binational registry analysis.

OBJECTIVE: To assess long term graft and patient survival after donor liver retransplantation in children in Australia and New Zealand during 1986-2017; to determine the factors that influence survival. DESIGN: Retrospective cohort analysis (registry data). SETTING, PARTICIPANTS: Australia and New Zealand Liver Transplant Registry data for all liver retransplantations in children (under 18 years of age), 1986-2017, in all four paediatric and six adult liver transplantation centres in the two countries. MAIN OUTCOME MEASURES: Graft and patient survival at one, 5, 10 and 15 years. RESULTS: 142 liver retransplantations were undertaken in children (59 during 1986-2000, 83 during 2001-2017). Kaplan-Meier survival analysis indicated that survival was significantly greater during 2001-2017 than 1986-2000 (P < 0.001). During 2001-2017, graft survival one year after retransplantation was 84%, at 5 years 75%, at 10 years 70%, and at 15 years 54%; patient survival was 89% at one year, 87% at 5 years, 87% at 10 years, and 71% at 15 years. Median time between transplantations was 0.2 years (IQR, 0.03-1.4 years) during 1986-2000, and 1.8 years (IQR, 0.1-6.8 years) during 2001-2017 (P = 0.002). The proportion of graft failures that involved split grafts was larger during 2001-2017 (35 of 83, 42%) than 1986-2000 (10 of 59, 17%). Graft type, cause of graft failure, and number of transplants did not influence survival following retransplantation. CONCLUSION: Survival for children following retransplantation is excellent. Graft survival is similar for split and whole grafts. Children on the liver waiting list requiring retransplantation should have the same access to donor grafts as children requiring a first transplant.

Use of a donor iliac vein graft for reconstruction of the inferior vena cava in liver transplantation for hepatoblastoma with caval extension.

Complete microscopic tumor resection is critical for successful treatment of hepatoblastoma, and this may include when liver transplantation is required. For tumors involving the IVC or PV, complete resection should include the involved IVC or PV to ensure full tumor clearance. When this is required, the venous reconstruction at transplant or post-excision can be challenging. We present the management of an 18-month-old girl with PRETEXT Stage IV (P, V, F) hepatoblastoma and IVC involvement, where native caval resection and reconstruction was required. The preoperative staging following neoadjuvant chemotherapy was POSTTEXT Stage IV (P, V, F). An orthotopic liver transplantation was performed using a left lateral segment graft from a deceased adult donor. With native hepatectomy, retrohepatic IVC resection from just above the hepatic venous confluence to just above the entry of the right adrenal vein was performed. For caval reconstruction, a venous graft from a deceased donor was used. The graft included the lower IVC with the right common iliac vein and a short stump of the left common iliac vein. The common iliac was a perfect size match for the IVC, and the three natural ostia matched the upper cava, lower cava, and the outflow from the donor left hepatic vein. The patient had an uneventful postoperative course and remains well and disease-free 2 years after transplant with continued patency of the reconstructed cava. When indicated, a donor iliac vein graft with its natural ostia should be considered in caval reconstruction for pediatric liver transplantation.

Successful treatment of a metastatic hepatocellular malignant neoplasm, not otherwise specified with chemotherapy and liver transplantation.

Hepatocellular malignant neoplasm, not otherwise specified (HCN-NOS) is a provisional entity describing a subset of rare malignant pediatric liver tumors with overlapping features of hepatoblastoma and hepatocellular carcinoma. We present a case illustration of metastatic HCN-NOS successfully treated with a backbone of hepatoblastoma chemotherapy, pulmonary metastastectomy, and liver transplantation, along with a literature review of the clinical outcomes of HCN.

Intra-abdominal Complications After Pediatric Kidney Transplantation: Incidence and Risk Factors.

BACKGROUND: The incidence and types of intra-abdominal complications after pediatric transplantation are not well established, and specific risk groups have not been clearly identified. METHODS: A retrospective chart review of all pediatric transplant recipients between 1995 and 2016 was undertaken. Intra-abdominal complications were grouped into 4 categories: fluid collections, gastrointestinal, vascular, and urogenital. Donor, recipient, and transplant characteristics were evaluated using univariate and multivariate logistic regressions. RESULTS: There were 146 transplants meeting the inclusion criteria. The mean follow-up time was 4.6 ± 3.7 years (range, 0.3-18 y). The mean weight at transplantation was 31.5 ± 16.5 kg (range, 9-78), with 24 (16%) recipients being <15 kg and 23% younger than 5 years. Thirty-four (23%) patients had previous abdominal surgery. There were 32 complications identified in 27 (18%) transplant recipients. Fluid collections requiring surgical drainage developed in 9 (6.2%), gastrointestinal surgical complications in 12 (8.2%), vascular complications in 5 (3.5%), and urogenital complications in 6 (4.1%). There were only 3 graft losses due to abdominal complications, all after renal vein thrombosis. Weight <15 kg at the time of transplant (P = 0.016), previous abdominal surgery (P = 0.047), and intraperitoneal surgical technique (P = 0.008) were risk factors in the univariate analysis using Cox regression models, whereas only weight <15 kg (P = 0.003) and previous abdominal surgery (P = 0.008) were retained in the multivariate analysis. CONCLUSIONS: Intraabdominal complications occur in almost 1 in 5 pediatric renal transplant recipients. Weight <15 kg and previous abdominal surgery are risk factors for developing such complications.

A bibliometric analysis of pediatric liver transplantation publications.

Citation counts can identify landmark papers. The aim of this study was to identify and characterize the top-cited articles in the pediatric liver transplantation literature. A search strategy for the Scopus® database was designed for pediatric liver transplantation publications from 1945 to 2014. The 50 top-cited articles were analyzed. Author co-citation analysis was performed using VOSviewer techniques. There were 2896 articles published between 1969 and 2015. The mean citation count of the top 50 cited articles was 166 (range 95-635). There were three case reports in this top-cited list. There were 15 collaborations in this top-cited list with nine being international. The top-cited publications originated in 12 countries, with the USA and the UK contributing 31 and seven articles, respectively. There were 14 authors with four or more publications in this list. There was a single author with nine publications in the top-cited list. These top-cited papers were found in 16 journals, with three journals collectively publishing over 50% of these publications. Pediatric liver transplantation research is an evolving entity. Surgical techniques and case reports are influential articles. Collaborations at a national and international level produce highly cited articles, which are found in influential journals.

Reducing the incidence of hepatic artery thrombosis in pediatric liver transplantation: Effect of microvascular techniques and a customized anticoagulation protocol.

We aimed to assess the incidence of HAT over three eras following implementation of microvascular techniques and a customized anticoagulation protocol in a predominantly cadaveric split liver transplant program. We retrospectively reviewed pediatric liver transplants performed between April 1986 and 2016 and analyzed the incidence HAT over three eras. In E1, 1986-2008, each patient received a standard dose of 5 U/kg/h of heparin and coagulation profiles normalized passively. In E2, 2008-2012, microvascular techniques were introduced. In E3, 2012-2016, in addition, a customized anticoagulation protocol was introduced which included replacement of antithrombin 3, protein C and S, and early heparinization. A total of 317 liver transplants were completed during the study period, with a median age of 31.7 months. In E1, 22% of grafts were cadaveric in situ split grafts, while the second and third eras used split grafts in 59.0% and 64.9% of cases, respectively. HAT occurred in 9.5% in the first era, 11.5% (P=.661) in the second, and dropped to 1.8% in the third era (P=.043). A routine anticoagulation protocol has significantly reduced the incidence of HAT post-liver transplantation in children in a predominantly cadaveric in situ split liver transplant program.

Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia.

OBJECTIVES: Most infants with biliary atresia (BA) require liver transplantation (LT) after hepatoportoenterostomy (HPE), including those who initially clear jaundice. The aim of the present study was to identify clinical and routine laboratory factors in infants with BA post-HPE that predict native liver survival at 2 years. METHODS: A retrospective cohort study was conducted in 217 patients with BA undergoing HPE in Sydney, Australia and Toronto, Canada between January 1986 and July 2009. Univariate and multivariate logistic regression using backwards-stepwise elimination identified variables at 3 months after HPE most associated with 2-year native liver survival. RESULTS: Significant variables (P < 0.05) on univariate analysis included serum total bilirubin (TB) and albumin at 3 months post-HPE, bridging fibrosis or cirrhosis on initial liver biopsy, ascites of <3 months post-HPE, type 3 BA anatomy, age at HPE of >45 days, change in length z scores within 3 months of HPE, and center. On multivariate analysis, TB (P < 0.0001) and albumin (P = 0.02) at 3 months post-HPE, and center (P = 0.0003) were independently associated with native liver survival. Receiver operating characteristic analysis revealed an optimal cut-off value of TB <74 µmol/L (4.3 mg/dL; area under the receiver operating characteristic curve 0.8990) and serum albumin level >35 g/L (3.5 mg/dL; area under the receiver operating characteristic curve 0.7633) to predict 2-year native liver survival. TB and albumin levels 3 months post-HPE defined 3 groups (1: TB ≤74 µmol/L, albumin >35 g/L; 2: TB ≤74 µmol/L, albumin ≤35 g/L; 3: TB >74 µmol/L) with distinct short- and long-term native liver survival rates (log-rank P < 0.001). Length z scores 3 months post-HPE were poorer for group 2 than group 1 (-0.91 vs -0.30, P = 0.0217) with similar rates of coagulopathy. CONCLUSIONS: Serum TB and albumin levels 3 months post-HPE independently predicted native liver survival in BA when controlling for center. Serum albumin level <35 g/L in infants with BA who were no longer jaundiced at 3 months post-HPE was a poor prognostic indicator. Poorer linear growth and absence of significant coagulopathy suggest a role for early aggressive nutritional therapy in this group.

Papillary thyroid cancer in childhood: is parental screening helpful?

BACKGROUND: Familial non-medullary thyroid cancer (FNMTC) mandates family screening with ultrasound; however, the need for screening paediatric thyroid cancer with no family history has not been addressed. METHODS: This study conducted a retrospective cohort study in a tertiary hospital. The study group is composed of patients ≤18 years undergoing surgery for papillary thyroid cancer (PTC) from 2008 to 2014. RESULTS: During the study period, 15 paediatric patients had PTC. Only one patient had a prior family history of PTC involving two second-degree relatives. Of the 30 parents, four were lost to follow-up, with the remaining 26 undergoing screening thyroid ultrasound. Nodular thyroid disease was documented in eight ultrasounds performed. Subsequently, one has not undergone fine-needle biopsy, five were benign and two underwent surgical resection: one having a benign follicular adenoma and the other a papillary microcarcinoma with nodal micrometastasis. CONCLUSIONS: Routine ultrasound screening of parents of children presenting with PTC and no family history do not demonstrate an increased incidence of FNMTC. Screening of affected families is likely to demonstrate the expected background incidence of nodular thyroid disease and incidental papillary microcarcinoma. As such, it is unlikely to be useful.

Inspissated bile syndrome; Safe and effective minimally invasive treatment with percutaneous cholecystostomy in neonates and infants.

PURPOSE: Inspissated bile syndrome (IBS) is a rare cause of obstructive jaundice in neonates and infants with several treatment options reported. We present our experience with the use of minimally invasive ultrasound-guided percutaneous cholecystostomy drain catheter placement with ongoing saline lavage in neonates and infants. METHODS: Retrospective chart review of patients treated with percutaneous cholecystostomy, from February 2010 till June 2015. We reviewed the technical and clinical success along with complications of the procedure. RESULTS: There were 6 patients, mean age 17weeks (range 4-40). Most had significant risk factors for IBS presenting with biliary obstruction. A total of 7 procedures performed on the 6 patients, with a technical success rate of 6/7. One patient required cannulation of the intrahepatic biliary system. Drains were flushed for a median of 26days (10-70). Clinical success was achieved in all patients. 3 had displacement of the drain, one of which required re-insertion. Another developed a small sub-hepatic collection post procedure with pyrexia. On long term follow up one was found to have a forme fruste choledochal cyst. CONCLUSION: Centers with suitable interventional radiology services ultrasound-guided percutaneous cholecystostomy drain catheter placement with ongoing saline lavage is a safe and effective minimally invasive treatment for IBS in neonates and infants.

Hepatocellular Carcinoma in Children: Does Modified Platinum- and Doxorubicin-Based Chemotherapy Increase Tumor Resectability and Change Outcome? Lessons Learned From the SIOPEL 2 and 3 Studies.

INTRODUCTION: The aim of this article is to present an experience of two prospective studies from the International Childhood Liver Tumor Strategy Group (SIOPEL 2 [S2] and SIOPEL [S3]) trials and to evaluate whether modified platinum- and doxorubicin-based chemotherapy is capable of increasing tumor resectability and changing patient outcomes. METHODS: Between 1995 and 2006, 20 patients with hepatocellular carcinoma (HCC) were included in the S2 trial and 70 were included in the S3 trial. Eighty-five patients remained evaluable. RESULTS: Response to preoperative chemotherapy was observed in 29 of 72 patients (40%) who did not have primary surgery, whereas 13 patients underwent upfront surgery. Thirty-three patients had a delayed resection. Thirty-nine tumors never became resectable. Complete tumor resection was achieved in 34 patients (40%), including seven of those treated with liver transplantation (LTX). After a median follow-up period of 75 months, 63 patients (74%) had an event (a progression during treatment, a relapse after treatment, or death from any cause). Sixty patients died. Twenty-three of 46 patients (50%) who underwent tumor resection died. Eighteen of 27 patients (63%) with complete tumor resection (without LTX) and 20 of 34 patients (59%) with LTX survived. Only one of seven patients (14%) with microscopically involved margins survived. Overall survival at 5 years was 22%. CONCLUSION: Survival in pediatric HCC is more likely when complete tumor resection can be achieved. Intensification of platinum agents in the S2 and S3 trials has not resulted in improved survival. New treatment approaches in pediatric HCC should be postulated.

Delayed primary closure and the incidence of surgical complications in pediatric liver transplant recipients.

PURPOSE: The purpose of this study was to analyze the general surgical complications in pediatric liver transplant recipients and the safety of delayed primary closure at a single tertiary center. METHODS: A retrospective review of all liver transplant recipients between April 1986 and May 2014 was performed. All general and gastrointestinal complications were recorded and analyzed. The incidence and risk of these complications were compared between children who had a primary versus those who had a delayed closure, with or without the use of Surgisis®, of their abdomen. RESULTS: 242 patients underwent 281 liver transplants. The median age of the children was 31months. Whole (77), reduced size (91), split (96), and living related grafts (17) were used. General surgical complications were observed in 33 cases (11.7%). 135 cases underwent delayed primary closure (DPC) of their abdomen, 35 with Surgisis®. Patients with biliary atresia had a higher rate (4.6%) of bowel perforation (p=0.013). The majority of complications occurred within 3months of transplantation. CONCLUSION: General surgical complications postpediatric liver transplantation were common but usually not life threatening. Delayed primary closure was safe, had no significant long-term issues, and was not associated with higher incidence of wound related complications.

Focused image-guided parathyroidectomy in the current management of primary hyperparathyroidism.

OBJECTIVE: Primary hyperparathyroidism (PHPT) in childhood and adolescence has been considered a different disease to that seen in adults, with predominantly familial aetiology mandating open exploration to exclude parathyroid hyperplasia in contrast to the adoption of focused image-guided parathyroidectomy (FP) in adults. STUDY DESIGN: A retrospective cohort study in a tertiary referral hospital setting of all children and adolescents (<18 years) undergoing parathyroid surgery for PHPT. Data were obtained from a dedicated endocrine surgery database and hospital medical records. RESULTS: Over the 35-year study period (1980-2014), there were 31 patients who underwent parathyroidectomy for PHPT. 3 patients were from known multiple endocrine neoplasia type 1 syndrome (MEN1) families, 3 had an isolated family history of PHPT and 25 were sporadic. In the sporadic group, 24 (96%) presented with symptomatic hypercalcaemia, affecting the gastrointestinal, musculoskeletal, genitourinary or neuropsychiatric systems. In the 25 patients with sporadic PHPT, nine (36%) had FP with a single adenoma removed with a 100% initial cure rate. Sixteen patients (64%) in the sporadic group had an open exploration: 14 had single gland disease while 2 patients required a second procedure to achieve a final cure rate of 100%. Of the three patients with MEN1, one was cured, one has persistent hyperparathyroidism after FP and the third has permanent hypoparathyroidism after open exploration. CONCLUSIONS: The majority of children and adolescents with PHPT have symptomatic disease due to a single adenoma. They can therefore be managed in a similar fashion to their adult counterparts with preoperative localisation studies aiming to permit FP in a day case setting.

Risk of malignancy for each Bethesda class in pediatric thyroid nodules.

PURPOSE: The Bethesda classification for cytology is used to classify thyroid nodules into one of six categories, and for each category there is an implied cancer risk and also recommendation for management. Despite lack of data in children, the American thyroid association promotes the use of the same management guidelines as in adults. Our aim was to study the risk of malignancy for each Bethesda class in children with thyroid nodules. METHODOLOGY: We included all patients ≤18years of age that had underwent a thyroid fine needle aspiration (FNA) at one of two centers between January 1998 and July 2013. FNA results were reclassified according to the Bethesda criteria. Histological, repeat cytological, radiological and clinical follow-up were recorded. RESULTS: Fifty-six patients (66 nodules) underwent FNB. Mean age was 13.6 years. Numbers of nodules reported as BI-BVI were 7, 38, 11, 4, 3 and 3, respectively. Follow-up was achieved for 55 (83%) nodules. Twelve (18%) nodules were malignant by histology and revealed papillary (n=7), follicular (n=3) or insular thyroid cancer (n=2), The proportion of nodules with malignancy for BI-BVI was: 0%, 0%, 18%, 100%, 100% and 100%. CONCLUSION: The rate of malignancy in thyroid nodules in children seems to be higher than reported in adults. The Bethesda criteria seem to accurately identify benign nodules, but other categories have a very high rate of malignancy and BIII nodules pose a particular challenge.