Perinatal airway management in neonatal goiter: A healthcare cost and utilization project (HCUP) kids' inpatient database analysis.

INTRODUCTION: Fetal goiter is a rare congenital disorder that can present with life-threatening neonatal airway obstruction. Lifesaving and function-preserving airway management strategies are available, but routine delivery affords a limited window for intervention. Accordingly, fetal goiter is reported among the most common indications for ex-utero intrapartum treatment (EXIT). While EXIT prolongs the window for airway intervention to benefit the neonate, it elevates the risk to the pregnant person and requires extensive resources; therefore, data to guide ideal treatment selection are essential. This study aims to compare perinatal airway interventions between individuals with a birth hospitalization discharge diagnosis (BHDD) of goiter and the general population. MATERIALS AND METHODS: Individuals with and without BHDD of goiter were identified in the Healthcare Cost and Utilization Project (HCUP) Kids' Inpatient Database from 2000 to 2019. The frequency of airway interventions on day of life (DOL) 0 or 1 were compared using the Rao-Scott chi-square test. Additionally, gestational age, type of intervention, complications, mortality, birth weight, and length of stay were examined for the goiter cohort. RESULTS: Two-hundred eighty-seven weighted cases of goiter were identified in the study period. The population was 61 % male, 55 % White, and median birthweight was 3.3 kg. The median length of stay was 4.3 days, and average total charges were $42,332. Airway intervention on DOL 0 or 1 was performed in 16.9 % of individuals with goiter compared to 1.6 % in neonates without goiter (p < 0.001). Interventions in the goiter cohort included endotracheal intubation in 16 % of cases, laryngoscopy/bronchoscopy in 1-5% of cases, and tracheostomy in <1 % of cases. Fewer than 1 % of individuals undergoing intubation additionally had mass decompression/resection on DOL 0 or 1. No neonates received extracorporeal membrane oxygenation cannulation or cardiopulmonary resuscitation. Hypoxic encephalopathy occurred in <1 % of cases, among which endotracheal intubation was the only airway intervention performed. There were no mortalities among neonates with goiter. CONCLUSION: Individuals with BHDD of goiter receive significantly higher rates of perinatal airway intervention. In most cases, endoscopic interventions alone were sufficient to avoid hypoxic neurological complications. These findings contribute to data to aid in clinical counseling and empower patients to make informed decisions according to their values and treatment goals.

Extracorporeal Membrane Oxygenation in a Patient with Biliary Atresia: Case and Review of Extracorporeal Life Support Organization Data.

Biliary atresia is a newborn cholangiopathy that may lead to portopulmonary hypertension and cirrhosis-induced cardiomyopathy while awaiting liver transplantation. Extracorporeal life support and hepatic toxin filtration are life-saving interventions that provide cardiopulmonary support and hepatic dialysis to allow resolution of a child's illness. We utilized a combination of these extreme measures to bridge an infant with biliary atresia to transplantation. We reviewed cases of extracorporeal life support utilization in transplantation recipients in the Extracorporeal Life Support Organization database and determined that ours was the only use of pretransplant extracorporeal life support in biliary atresia.

The management of pediatric renovascular hypertension: a single center experience and review of the literature.

INTRODUCTION: Renal artery occlusive disease is poorly characterized in children; treatments include medications, endovascular techniques, and surgery. We aimed to describe the course of renovascular hypertension (RVH), its treatments and outcomes. METHODS: We performed literature review and retrospective review (1993-2014) of children with renovascular hypertension at our institution. Response to treatment was defined by National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents at most-recent follow-up. RESULTS: We identified 39 patients with RVH. 54% (n=21) were male, with mean age of 6.93 ± 5.27 years. Most underwent endovascular treatment (n=17), with medication alone (n=12) and surgery (n=10) less commonly utilized. Endovascular treatment resulted in 18% cure, 65% improvement and 18% failure; surgery resulted in 30% cure, 50% improvement and 20% failure. Medication alone resulted in 0% cure, 75% improvement and 25% failure. 24% with endovascular treatment required secondary endovascular intervention; 18% required secondary surgery. 20% of patients who underwent initial surgery required reoperation for re-stenosis. Mean follow-up was 52.2 ± 58.4 months. CONCLUSIONS: RVH treatment in children includes medications, surgical or endovascular approaches, with all resulting in combined 79% improvement in or cure rates. A multidisciplinary approach and individualized patient management are critical to optimize outcomes. TYPE OF STUDY: Retrospective comparative study LEVEL OF EVIDENCE: Level III.

Surgical management and surveillance of pediatric appendiceal carcinoid tumor.

PURPOSE: Appendiceal carcinoid tumors are rare neuroendocrine neoplasms. The aim of this study was to determine if postoperative oncologic follow-up was necessary for this tumor. METHODS: A retrospective review was performed of patients with appendiceal carcinoid 2000-2015. RESULTS: 8382 patients underwent appendectomy 2000-2015. 30 (0.3%) had appendiceal carcinoid. 70% were female (n=21) with an average age of 13.5±2.8 years (range 8-18). Most presented with abdominal pain (n=29, 97%). 20% (n=6) had appendiceal perforation. Mean tumor size was 5.4±4mm (range microscopic - 15mm) with most at the appendiceal tip (n=18, 60%). No node infiltration was found, although 10% (n=3) had perineural and 3% (n=1) had lymphovascular invasion. Five were transmural (17%). Most patients were referred to oncology (n=19, 63%) for staging and surveillance including ultrasonography (n=11, 65%), MRI (n=7, 41%), and CT (n=6, 35%). The majority (79%, n=15) underwent serial 5-HIAA testing. All surveillance was found to be normal, and no patients required further treatment. Mean follow-up was 36±34 months, with 58% (n=11) continuing surveillance. Medical charges ranged $8500-$44,000. No recurrences have been identified. CONCLUSION: Appendectomy is an adequate treatment for pediatric appendiceal carcinoid <16 mm despite presence of histological risk factors. More aggressive surgery and extensive oncologic follow up are of limited value. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Retrospective comparative study.

Cystic Biliary Atresia and Choledochal Cysts Are Distinct Histopathologic Entities.

Cystic biliary atresia (CBA), a rare cystic expansion of atretic extrahepatic bile ducts in young infants, overlaps in age at presentation and imaging features with early choledochal cysts (CC). Treatment and prognosis differ; histologic differences are unsettled. We compared 10 patients with CBA, 1975 to 2015, to an age-similar cohort of 13 infants, and to older patients who had surgery for CC. Operative details, imaging, and clinical courses were correlated to pathologic specimens. Immunostains for smooth muscle actin and myosin heavy chain were used to evaluate cyst walls and atretic segments. CBA cysts typically lacked epithelium and inflammation; cyst walls had an inner, dense cicatricial layer associated with myofibroblastic (MF) hyperplasia that often delaminated producing a grossly visible inner cyst wall. Seven proximal biliary remnants in CBA featured circumferential peribiliary MF hyperplasia/fibrosis with little or no inflammation, similar to isolated BA. Extrahepatic atresia was usually both proximal and distal to the cyst. Features in 10/13 CC from infants and 8/8 CC in older patients had mostly preserved uninjured epithelium and no subepithelial cicatrix. Mural smooth muscle (absent in CBA) was present to some extent in CC at all ages. Unexpectedly, focal MF hyperplasia and laminar sclerosis was present in a few CC in infants, resembling CBA. CBA and infant CC are distinct histologic entities that occasionally overlap. CBA bile duct injury mimics non-CBA. Cystification is an aberrant manifestation of stromal proliferation in BA. The current management approach assuming CBA and CC in infants are 2 separate disease processes is supported but caution is advised.

The presentation and management of choledochocele (type III choledochal cyst): A 40-year systematic review of the literature.

BACKGROUND: Choledochoceles may cause biliary obstruction and harbor malignancy. We conducted a 40-year systematic review of the literature for this rare anomaly. METHODS: PubMed and Cochrane databases were accessed 1975-2015 using terms "choledochocele" or "choledochal cyst". Studies reviewed that met the following criteria: English language, published 1975-2015 with human subjects. RESULTS: 325 patients with a choledochocele were identified, including 71 case reports and 254 cases within institutional reviews. 13 pediatric case reports of choledochocele exist, with abdominal pain being the most common symptom (n=11). The most frequent diagnostic and treatment modalities were ultrasound (n=10), and endoscopic sphincterotomy (n=5). No malignancies were reported. 58 adult case reports exist, with the most common presenting symptom being abdominal pain (n=54). Ultrasound was the frequently employed diagnostic modality (n=32). Open procedures were performed more often (n=30). Malignant lesions were identified in 5. In 42 institutional reviews, the frequency of choledochocele was 0.7%. Of those for whom treatment was reported, 69% underwent endoscopic sphincterotomy. CONCLUSION: Choledochocele is a rare malformation. Similarities exist between pediatric and adult patients, but malignancy has only been reported in adults. An algorithm based on patient age, cyst size, lining and amenability to endoscopic resection may be considered as a treatment strategy for this uncommon condition.

Blood pressure changes after intravenous fosphenytoin and levetiracetam in patients with acute cerebral symptoms.

PURPOSE: To study the incidence and extent of the occasionally noted hypotension after intravenous (IV) infusions of fosphenytoin (FOS) and levetiracetam (LEV) in patients presenting with acute cerebral symptoms. METHODS: Retrospective data collection of consecutive patients with acute cerebral symptoms who received IV infusions of a single dose of 750 mg or more of either fosphenytoin or levetiracetam and had documented blood pressure values in the 2h prior and the 2h after their IV infusion. RESULTS: More than 10 mmHg drop in the systolic, diastolic and MBP was observed in the FOS group following the IV infusion (-16.82 mmHg, -11.60 mmHg, and 13.34 mmHg, respectively). However, there was not a significant change in the MBP after LEV infusion (1.54 mmHg, 1.84 mmHg, and 1.74 mmHg for systolic, diastolic and MBP change, respectively). The difference in the systolic, diastolic and MBP changes between the two groups was statistical significant (all p values are <0.0001) after adjusting for age, clinical presentations of the patients and if they were on any antihypertensive medication in the hospital. Sixty two percent of patients who received FOS had >10 mmHg decrease in their MBP. In the LEV group, only 2 of the 50 patients (4%) had >10 mmHg decrease in their MBP. The difference in proportion of the patients with >10 mmHg drop in MBP between the two study groups is also statistically significant (p<0.001) for age, clinical presentations of the patients and if they were on any antihypertensive medication in the hospital. CONCLUSIONS: IV infusion of FOS in subjects presenting with acute cerebral symptoms may cause significant decreases in their blood pressure. This was not seen in patients receiving IV LEV infusions. Since maintaining adequate cerebral perfusion pressure is a key point in the management of patients with acute cerebral symptoms, the results of this study may carry a clinical impact on the management of this subgroup of patients.