Validity of the American College of Radiology Thyroid Imaging Reporting and Data System in Children.

OBJECTIVE: To assess the validity of the American College of Radiology Thyroid Imaging Reporting and Data System (ACR TI-RADS) for evaluating thyroid nodules in children. METHODS: Patients aged <19 years with thyroid nodule(s) evaluated by ultrasound (US) from 2007-2018 at a tertiary children's hospital were included. Two radiologists scored de-identified thyroid US images using ACR TI-RADS (from 1, "benign" to 5, "highly suspicious"). The radiologists recorded size and rated vascularity for each nodule. Ultrasound findings were compared to pathology results (operative cases, n = 91) and clinical follow-up without disease progression (non-operative cases, n = 15). RESULTS: Thyroid images from 115 patients were reviewed. Nine patients were excluded due to the absence of an evaluable nodule. Forty-seven benign and 59 malignant nodules were included. Median age at ultrasound was 15 years (range 0.9-18 years). Twenty (18.9%) patients were male. There was moderate agreement between TI-RADS levels assigned by the two raters (kappa = 0.57, p < 0.001). When the raters' levels were averaged, >3 as the threshold for malignancy correctly categorized the greatest percentage of nodules (68.9%). Eleven (18.6%) malignant nodules received a TI-RADS level of 2 (n = 3) or 3 (n = 8). Sensitivity, specificity, and positive and negative predictive values were 81.4%, 53.2%, 68.6%, and 69.4%, respectively. Although not part of TI-RADS, vascularity was similar between benign and malignant nodules (p = 0.56). CONCLUSION: In a pediatric population, TI-RADS can help distinguish between benign and malignant nodules with comparable sensitivity and specificity to adults. However, the positive and negative predictive values suggest TI-RADS alone cannot eliminate the need for FNA. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:2394-2401, 2023.

Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation.

A seven-yr-old boy presented with persistent oxygen requirement following a respiratory infection. Physical exam was remarkable for orthodeoxia and digital clubbing. Laboratory evaluation showed elevated A-a oxygen gradient of 48 mmHg and mildly elevated transaminases. Sonography showed a 13 cm multilobulated liver mass and a biopsy revealed histological findings consistent with focal nodular hyperplasia. MAA scan revealed 23% right to left shunting. Abdominal CTA and MRV demonstrated the absence of the intrahepatic portal vein with an extrahepatic portocaval shunt. Abernethy malformation is a rare anomalous intra- or extrahepatic communication between portal blood flow and systemic venous return. In rare cases, Abernethy malformation results in HPS. Ours is the sixth case report to describe the co-existence of these two entities. Surgical correction of anomalous hepatic vasculature or liver transplant is imperative to restoration of lung function and also to prevent progression of possible malignant liver tumors. We describe the second patient with Abernethy and HPS who underwent liver transplant with complete resolution of HPS.

An unusual cause of rectal bleeding and intestinal obstruction in a child with peripheral vascular malformations.

Vascular malformations of the small bowel are rare yet important causes of abdominal pain and rectal bleeding in children. This report describes a 6-year-old girl with a known history of musculoskeletal vascular anomalies who presented with recurrent abdominal pain and rectal bleeding after seemingly minor trauma. A diagnosis of enteric vascular malformation was established, and the patient was treated by a combined laparoscopic and open approach. This case highlights the fact that in the child with known vascular anomalies who presents with sudden gastrointestinal bleeding and intestinal obstruction, the diagnosis of gastrointestinal vascular anomaly should be suspected, and exploratory laparoscopy should be considered. The current management of enteric vascular anomalies is reviewed, and the differences between vascular malformations and hemangiomas are analyzed.

Mesenteric inflammatory pseudotumor as a cause of abdominal pain in a teenager: presentation and literature review.

Inflammatory pseudotumor (IP) is an unusual cause of chronic abdominal pain in children. The management of these lesions is complicated by controversies surrounding their appropriate classification and the numerous alternate names with which they are described. Successful treatment requires careful radiologic and pathologic evaluation to distinguish IPs from other lesions, along with complete surgical resection. We present the case of a 15-year-old boy with IP and review the literature in an attempt to simplify the description of these tumors.

Gastric volvulus as a complication of liver transplant.

We report a patient who developed mesenteroaxial gastric volvulus after a liver transplantation. We hypothesize that this complication may have been related to the ligation of the hepatogastric ligament done to mobilize the liver during hepatectomy.

Predictors of outcome for children with perforated appendicitis initially treated with non-operative management.

BACKGROUND: Initial non-operative therapy for children with perforated appendicitis has become increasingly popular with the advent of powerful broad-spectrum antibiotics. However, there is no consensus regarding which patients may be managed effectively with this strategy. We reviewed all children with perforated appendicitis who were treated initially with non-operative therapy to determine those characteristics that may predict a successful outcome. METHODS: We reviewed the medical records of children admitted to our hospital between January 1, 2000 and May 1, 2003 with the diagnosis of perforated appendicitis. Only those who were treated initially with a single broad-spectrum antibiotic (piperacillin-tazobactam), with the intention of performing an interval appendectomy, were included in this study. Patients were divided into two groups based on whether they were managed successfully with non-operative therapy: Responders and non-responders. Non-responders were defined as patients who either did not improve with antibiotic therapy or who required appendectomy prior to their electively scheduled time (six weeks). Demographic data, duration and type of presenting symptoms, initial white blood cell count (WBC), percent bands, percent neutrophils (PMNs), computed tomography (CT) interpretation, and interventions/operations were abstracted. Categorical data were compared using Chi-square analysis or the Fisher exact test; continuous variables were compared using the Student t-test and the Mann-Whitney U-test. RESULTS: Overall, 26% (19/73) of patients treated initially non-operatively required appendectomy prior to the electively scheduled date. There was no difference between responders (n = 54) and non-responders (n = 19) with respect to age, gender, initial WBC, percent bands, percent PMNs, or duration and type of presenting symptoms. However, responders were more likely to have a phlegmon on CT scan compared to non-responders (11/54 vs. 0/19, p = 0.03). Non-responders were twice as likely to undergo drainage of an abscess by interventional radiology (10/19 vs. 13/54, p = 0.02) compared to responders. Among all patients who required percutaneous drainage, the failure rate of non-operative management was 43% (10/23). CONCLUSIONS: Children with perforated appendicitis can be managed effectively with nonoperative therapy, even in the presence of intra-abdominal abscesses. However, the need for abscess drainage increases the failure rate, perhaps due to inadequate source control. Those patients with a phlegmon on CT scan as opposed to an abscess, are most likely to respond to non-operative management. Initial non-operative therapy of perforated appendicitis in children is appropriate under certain clinical circumstances, especially when the body itself or interventional radiology can achieve adequate source control.

The influence of the synergistic anion on iron chelation by ferric binding protein, a bacterial transferrin.

Although the presence of an exogenous anion is a requirement for tight Fe(3+) binding by the bacterial (Neisseria) transferrin nFbp, the identity of the exogenous anion is not specific in vitro. nFbp was reconstituted as a stable iron containing protein by using a number of different exogenous anions [arsenate, citrate, nitrilotriacetate, pyrophosphate, and oxalate (symbolized by X)] in addition to phosphate, predominantly present in the recombinant form of the protein. Spectroscopic characterization of the Fe(3+)anion interaction in the reconstituted protein was accomplished by UV-visible and EPR spectroscopies. The affinity of the protein for Fe(3+) is anion dependent, as evidenced by the effective Fe(3+) binding constants (K'(eff)) observed, which range from 1 x 10(17) M(-1) to 4 x 10(18) M(-1) at pH 6.5 and 20 degrees C. The redox potentials for Fe(3+)nFbpXFe(2+)nFbpX reduction are also found to depend on the identity of the synergistic anion required for Fe(3+) sequestration. Facile exchange of exogenous anions (Fe(3+)nFbpX + X' --> Fe(3+)nFbpX' + X) is established and provides a pathway for environmental modulation of the iron chelation and redox characteristics of nFbp. The affinity of the iron loaded protein for exogenous anion binding at pH 6.5 was found to decrease in the order phosphate > arsenate approximately pyrophosphate > nitrilotriacetate > citrate approximately oxalate carbonate. Anion influence on the iron primary coordination sphere through iron binding and redox potential modulation may have in vivo application as a mechanism for periplasmic control of iron delivery to the cytosol.