Noninvasive Follicular Thyroid Neoplasm With Papillary-Like Nuclear Features in Children: An Institutional Experience and Literature Review.

BACKGROUND: Papillary thyroid carcinoma (PTC) in children has a distinctive set of clinicopathologic features and molecular signature compared to their adult counterparts. The recent recommendation to reclassify encapsulated follicular variant of papillary thyroid carcinoma (EFVPTC) without invasion as noninvasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) is based on evidence derived almost exclusively from studies in adults. Clinicopathologic studies restricted to pediatric NIFTP are limited. METHODS: We retrospectively analyzed all pediatric PTC and NIFTP diagnosed and treated in our institution from 1999 to 2016 (n = 31). RESULTS: Using recently published consensus diagnostic criteria, we identified 3 NIFTP and 2 infiltrative follicular variants of papillary thyroid carcinoma (FVPTC) among 31 cases. Two of the NIFTP cases were initially diagnosed as EFVPTC. All 3 patients with NIFTP had unifocal tumors of lower American Joint Committee on Cancer (AJCC) classification (T2 or lower) and were free of lymph node or distant metastasis. Total (n = 1) or completion (n = 2) thyroidectomy was performed in all cases, and only 1 NIFTP patient received subsequent radioablative therapy. No residual or recurrent disease has been observed during follow-up (15-138 months) in patients with NIFTP. CONCLUSIONS: Our experience with NIFTP in children is similar to outcomes reported in adult studies, suggesting that pediatric NIFTP behave indolently as evidenced by the absence of local recurrence in our cohort.

Thoracoscopic Resection of a Rare Congenital Lung Lesion With a Tracheal Bronchus.

A tracheal bronchus is a rare anatomic variant characterized by a bronchus originating from the trachea rather than the carina. These are most commonly asymptomatic and found incidentally but can cause recurrent pneumonias in children. Here, we present a case of a thoracoscopic resection of an azygous lobe with a tracheal bronchus in a 9-year-old female.

Robotic-assisted laparoscopic repair of a congenital rectourethral fistula with duplicated urethra.

OBJECTIVE: To describe our experience with robot-assisted laparoscopic transperitoneal repair of a congenital rectourethral fistula in a pediatric patient with a urethral duplication. METHODS: The patient is a 2-year-old male with a past medical history of Tetralogy of Fallot presenting with a febrile urinary tract infection (UTI). He was diagnosed with urethral duplication and a rectourethral fistula by voiding cystourethrogram (VCUG). The parents were counseled on various options and agreed to proceed with a robotic repair. RESULTS: Robotic-assisted transperitoneal rectourethral fistula repair was performed. The procedure time was 229 min with an estimated blood loss (EBL) of 15 mL. His postoperative course was unremarkable. At his 2-week follow-up, the urethral catheter was removed and the patient was voiding normally and having normal bowel movements. CONCLUSION: Congenital rectourethral fistula with urethral duplication is a rare anomaly with only a few reports in the literature. Pediatric robotic-assisted transperitoneal rectourethral fistula repair is a technically feasible approach in infants with minimal morbidity that allows for excellent visualization and avoids open repair.

Incidence and malignancy rates of indeterminate pediatric thyroid nodules.

BACKGROUND: The American Thyroid Association guidelines task force currently recommends definitive thyroidectomy or lobectomy after an indeterminate thyroid biopsy in children. This recommendation is based on evidence of a greater incidence and a higher risk of malignancy compared with adults in earlier pediatric studies. Such management may lead to overtreatment and unnecessary surgery for many children in the United States. METHODS: The objective of the current study was to re-evaluate pediatric thyroid nodules and assess the overall percentages and malignancy rates for indeterminate thyroid biopsies in children. In total, 302 pediatric thyroid fine-needle aspirations (FNAs) were analyzed retrospectively (2001-2018). Distribution percentages and malignancy rates were calculated for each category of The Bethesda System for Reporting Thyroid Cytopathology (TBSRTC). RESULTS: Two indeterminate TBSRTC groups (atypia of undetermined significance/follicular lesion of undetermined significance and follicular neoplasm/suspicious for a follicular neoplasm) had much lower distribution percentages and malignancy rates compared with earlier pediatric series and American Thyroid Association guidelines. A meta-analysis further supported these findings and demonstrated distinctly different malignancy rates for the indeterminate groups (atypia of undetermined significance/follicular lesion of undetermined significance, follicular neoplasm/suspicious for a follicular neoplasm, and suspicious for malignancy), suggesting the need for TBSRTC category-specific management recommendations rather than a nondiscriminatory, up-front surgical approach. CONCLUSIONS: Adult patients with indeterminate preoperative thyroid cytopathology are followed by repeat biopsy and possibly molecular testing before undergoing definitive surgery. However, in children, the guidelines are considerably more aggressive and recommend definitive surgery after the first indeterminate thyroid biopsy. Here, the largest pediatric cohort to date with meta-analysis is presented, and the authors propose a re-evaluation of this up-front approach to pediatric thyroid care.

Tracheotomy in the preschool population: indications and outcomes.

OBJECTIVE: Although more tracheotomy procedures are performed within the first year of life than in any other age group, preschool-aged children requiring tracheotomy remain understudied. We characterize the indications and outcomes for patients between the ages of 3 and 6 years undergoing tracheotomy. METHODS: Out of 480 pediatric tracheotomy procedures performed at a tertiary-care hospital between 1988 and 2004, 15 patients underwent primary tracheotomy between 3 and 6 years of age. RESULTS: Most (60%) procedures were performed for pulmonary toilet. Upper-airway obstruction represented the second most common indication (40%), and trauma necessitated tracheotomy procedures more often than had been predicted (40%). The decannulation rate was 40%; 2 patients died. CONCLUSION: Trauma contributed to both upper-airway obstruction as well as requirements for pulmonary toilet. These procedures performed secondary to trauma will likely continue to increase. SIGNIFICANCE: Tracheotomy procedures in the preschool population remain uncommon; however, nearly half of those studied were performed as a direct result of otherwise preventable trauma.

Tracheotomy in the first year of life: outcomes in term infants, the Vanderbilt experience.

OBJECTIVE: In an era emphasizing critical care of preterm infants, we characterize the indications and outcomes of tracheotomies performed in the first year of life in term infants compared to preterm infants. METHODS: Retrospective study of 127 tracheotomies performed in the first year of life at a tertiary-care children's hospital between 1988-2004. RESULTS: Mean gestational ages of the term and preterm groups were 38.97 and 29.71 weeks, respectively (P < 0.001). Indications for tracheotomy were upper airway abnormalities in 53% for the term group. The number of subsequent airway procedures required was 1.39 in the term group, achieving decannulation in 36.3%, with a 20.5% mortality rate. CONCLUSION: Compared to preterm infants, the term decannulation rate was favorable, as chronic lung disease was uncommon. However, non-tracheotomy-related mortalities remained high. SIGNIFICANCE: Tracheotomies are often performed for relief of upper airway obstruction, and congenital and acquired comorbidities not related to tracheotomy are associated with adverse outcomes in term infants. EBM RATING: C-4.

Congenital diaphragmatic hernia repair on extracorporeal life support: a decade of lessons learned.

Congenital diaphragmatic hernia (CDH) is a vexing anomaly that manifests with variable pulmonary compromise in neonates. More than one-third of neonates with CDH require extracorporeal membrane oxygenation (ECMO) for refractory pulmonary hypertension (PHN). To assess the outcome of neonates having CDH repair on ECMO, we reviewed our experience for babies treated between 1992 and 2003. Of 97 neonates with CDH, 40 required ECMO, and 30 were repaired on bypass. Eighteen were supported by veno-venous bypass (VV) and 12 by veno-arterial bypass (VA). While on ECMO, transfusion requirements increased twofold postoperatively (15 to 33 cc x kg(-1) day(-1), P = 0.03) and then significantly decreased after decannulation (1.5 cc x kg(-1) x day(-1), P < 0.01). Non-intracranial hemorrhage occurred in 7 (23%) infants and intracranial hemorrhage in 3 (10%). Twelve (40%) infants died; one (3%) on ECMO secondary to refractory PHN. The mean length of stay for the 18 (60%) survivors was 48 days. Comparisons between survivors and nonsurvivors showed a significantly increased mortality for infants placed on VA bypass (P < 0.01). However, no other variable was predictive of survival. We conclude that CDH repair on ECMO is technically feasible, shows similar survival to the Extracorporeal Life Support Organization (ELSO) registry, and is associated with few bleeding complications.

Operative intervention for complete pancreatic transection in children sustaining blunt abdominal trauma: revisiting an organ salvage technique.

Complete pancreatic transection (CPT) in children is managed commonly with distal pancreatectomy (DP). Alternatively, Roux-en-Y distal pancreaticojejunostomy (RYPJ) may be performed to preserve pancreatic tissue. The purpose of this study was to review our experience using either procedure in the management of children sustaining CPT after blunt abdominal trauma. We retrospectively reviewed the records of all children admitted to our institution during the last 15 years who were confirmed at operation to have CPT after blunt mechanisms. Summary statistics of demographic data were performed to describe children receiving either RYPJ or DP. CPT occurred in 28 children: 15 had DP, 10 had RYPJ, and three had cystogastrostomy. RYPJ children, compared with DP, were younger (7.5 vs. 12.3 years, P = 0.039) and sustained more grade IV pancreatic injuries (70% vs. 14%, P = 0.01). DP patients were 5.63 times more likely to tolerate full enteral feeds (P = 0.009). Nevertheless, when controlling for age, injury severity score, and pancreatic injury grade, procedure type did not statistically affect total and postoperative lengths of stay and postoperative complications. In the operative management algorithm of children sustaining CPT, DP may afford an earlier return to full enteral feeds. RYPJ seems otherwise equivalent to DP and preserves significant pancreatic glandular tissue and the spleen.

Eight-year follow-up after palliative embolization of a neonatal intracranial dural arteriovenous fistula with high-output heart failure: management strategies for symptomatic fistula growth and bilateral femoral occlusions in pediatric patients.

The authors report the 8-year follow-up of a patient previously described in the literature who originally presented in high-output cardiac failure secondary to a complex neonatal intracranial dural arteriovenous fistula (DAVF). The earlier case report described palliative treatment with a combination of extracorporeal membrane oxygenation (ECMO) and endovascular embolization for life-threatening high-output cardiac failure secondary to a DAVF. Access was obtained using the ECMO cannula, and embolization was performed while the patient was connected to the ECMO machine. The patient made an excellent recovery following partial embolization of the fistula, but then presented again 7 years later with worsening headaches secondary to significant growth of the known residual portion of the fistula identified on CT angiography. The child also developed bilateral femoral artery (FA) occlusions secondary to multiple previous FA punctures. To achieve complete obliteration of the remaining fistula, the patient required a retroperitoneal approach to the iliac artery and percutaneous puncture of the internal jugular vein. Embolization was performed with a combination of platinum coils and ethylene vinyl alcohol copolymer liquid embolic agent. There were no complications, and the child remains neurologically normal, with no signs of permanent cardiovascular sequelae. In this case report, the authors discuss the long-term management of AVFs treated by endovascular strategies early in life. After neonatal access, sometimes the FAs occlude, requiring more invasive access strategies. The authors also discuss the follow-up method, intervals, and threshold for further treatment for these lesions, and present a review of the literature.

Defining hepatoblastoma responsiveness to induction therapy as measured by tumor volume and serum alpha-fetoprotein kinetics.

PURPOSE: Hepatoblastoma is commonly unresectable at presentation, necessitating induction chemotherapy before definitive resection. To refine the paradigm for timing of resection, we questioned whether a plateau in hepatoblastoma responsiveness to neoadjuvant therapy could be detected by calculating tumor volume (TV) and serum alpha-fetoprotein (sAFP) kinetics. METHODS: To calculate TV and sAFP as measures of treatment responsiveness over time, infants having initially unresectable epithelial-type hepatoblastomas were identified at a single institution (1996-2008). Effects of therapy type, therapy duration, and lobe of liver involvement on TV, sAFP, margin status, and toxicity were analyzed. RESULTS: Of 24 infants treated for epithelial-type hepatoblastoma during this interval, 5 were resected primarily, and 15 had complete digital films for kinetics analysis. Both TV and sAFP decreased dramatically over time (P < .0001). No statistically significant difference in mean TV or sAFP was detected after chemotherapy cycle 2. Left lobe tumors had greater presenting levels of and significantly slower decay in sAFP compared with right lobe tumors (P = .005), although no statistically significant differences in TV existed between liver lobes. Resection margins did not change with therapy duration. CONCLUSIONS: Measuring TV and sAFP kinetics accurately reflects hepatoblastoma responsiveness to induction therapy. Treatment toxicities may be reduced by earlier resection and tailoring of chemotherapeutic regimens.