Isolated aortic arch anomalies are associated with defect severity and outcome in patients with congenital diaphragmatic hernia.

PURPOSE: Congenital diaphragmatic hernia (CDH) patients often have suspected isolated aortic arch anomalies (IAAA) on imaging. The purpose of this work was to describe the incidence and outcomes of CDH + IAAA patients. METHODS: Cardiovascular data were collected for infants from the CDH Study Group born between 2007 and 2019. IAAA were defined as coarctation of aorta, hypoplastic aortic arch, interrupted aortic arch, and aortic aneurysmal disease on early, postnatal echocardiography. Patients with major cardiac malformations and/or chromosomal abnormalities were excluded. Primary outcomes included the rate of aortic intervention, rates of extracorporeal life support (ECLS) utilization, and mortality. RESULTS: Of 6357 CDH infants, 432 (7%) were diagnosed with a thoracic aortic anomaly. Of these, 165 were diagnosed with IAAA, most commonly coarctation of the aorta (n = 106; 64%) or hypoplastic aortic arch (n = 58; 35%). CDH + IAAA patients had lower birthweights (3 kg vs. 2.9 kg) and Apgar scores (7 vs. 6) than patients without IAAA (both χ2 p < 0.001). CDH + IAAA were less likely to undergo diaphragm repair (72 vs. 87%, p < 0.001), and overall mortality was higher for CDH + IAAA infants (58 vs. 24%, p < 0.001). When controlling for defect size, birth weight, and Apgar, IAAA were significantly associated with mortality (OR 3.3, 95% CI 2.2-5.0; p < 0.01) but not associated with ECLS (OR 0.98, 95% CI 0.65-1.50; p = 0.90). Only 17% (n = 28) of CDH + IAAA patients underwent aortic intervention. CONCLUSIONS: IAAA in CDH are associated with increased mortality. This often simply reflects severity of the defect and thoracic anatomic derangement, as opposed to unique aortic pathology, given few CDH + IAAA patients undergo aortic intervention.

Introduction of a novel ultrasound-guided extrathoracic sub-paraspinal block for control of perioperative pain in Nuss procedure patients.

BACKGROUND: A safe and effective method of multilevel thoracic pain control remains an elusive goal in patients undergoing the Nuss procedure. The aim of our study was to develop a nonopioid centered approach using a novel regional technique as part of a quality improvement initiative. METHODS: The proposed ultrasound-guided technique positions multi-perforated soaker catheter deep to the paraspinal muscles from T2 to T11. The project was conducted in two phases. First, a cadaveric dissection was performed to establish the pathway of spread of local anesthetic in vivo. Second, a pilot double blind randomized control project was conducted to evaluate effectiveness of the technique in ten patients and to derive parameters necessary for the definitive future study. Outcomes were evaluated based on the narcotic requirement, pain scores and functional measures. RESULTS: Placement of the catheters in two cadavers demonstrated reliable positioning in the subparaspinal tissue plane, and multilevel dye spread along the intercostal nerve path. In addition, a potential route of spread toward the paravertebral space along the canal accommodating dorsal ramus of the thoracic nerve was demonstrated. The pilot trial demonstrated a trend in decreased cumulative hydromorphone requirement in comparison to the control group at both 24h (0.19±0.09mg/kg vs. 0.13±0.08mg/kg p=0.72) and 48h (0.37±0.2mg/kg vs. 0.3±0.12mg/kg p=0.37). Functional performance ability was higher in the treatment group on both POD#1 (6.7±1.8 vs. 4.8±1 p=0.0495) and POD#2 (8.9±0.8 vs. 6.5±1.2 p=0.04). Pain scores were similar among the two groups (p=0.96). CONCLUSIONS: We describe a new technique to treat multilevel thoracic pain following the Nuss procedure that is reproducible, safe, allows diminished opioid use and enhances functional recovery.

Initial experience with thoracoscopic esophageal atresia and tracheoesophageal fistula repair: lessons learned and technical considerations to achieve success.

The minimally invasive surgical (MIS) repair of esophageal atresia/tracheoesophageal fistula (EA/TEF) is challenging and requires advanced endoscopic skills. The purpose of this study was to provide insight in successfully introducing the MIS repair based on the initial cases performed by a single pediatric surgeon and review of the experience of others. A retrospective review of all MIS TEF repairs performed by a single surgeon was conducted. Data gathered included patient demographics, technical details of repair including operative time, short- and long-term postoperative morbidity, length of stay, and follow-up. Eight cases (seven Type C, one Type D) were selected for MIS repair based on the judgment of the surgeon with consideration of adequate patient size, stability, type of associated anomalies, and expected length of esophageal gap. Operative time was an average of 207 minutes and there was one conversion to open for successful repair. There were no leaks and only one patient required a single anastomotic dilation at 19 months of age. There were two postoperative pneumothoraces of which one required bronchoscopic laser fistula ablation. Length of stay was an average of 16 days and length of follow-up is a median of 219 days. MIS repair of EA/TEF can be done successfully but requires careful patient selection, advanced MIS skills and meticulous attention to operative technique.

Differentiating biliary atresia from other causes of cholestatic jaundice.

Diagnosis of biliary atresia (BA) in infants presenting with cholestatic jaundice (CJ) requires exploratory surgery with cholangiography. However, the lack of a standardized approach to preoperative evaluation of infants with CJ can lead to a high number of negative surgical explorations. We reviewed our experience with CJ and BA to determine preoperative variables that might reliably identify BA. Infants explored for possible BA over a 5-year period were retrospectively reviewed. Preoperative clinical indices and liver biopsy results were reviewed. Statistical analysis was conducted by Student's t test and Fisher's exact test (P < 0.05). Twenty patients were identified, 10 with BA and 10 without (50% negative exploration rate). Nuclear cholescintigraphy (HIDA) excretion into the gastrointestinal tract was absent in all BA and in 8 of 10 without BA. Hepatomegaly was more common in the BA group (OR = 9.3, P = 0.07). BA had higher mean (± standard error) serum gamma-glutamyl transpeptidase levels (542 ± 130 vs 139 ± 25.8 U/L in non-BA, P = 0.03). There were insignificant differences in sex, type of feeding, TPN exposure and sepsis between the two groups. Although our small sample size limits conclusions, we suggest screening infants with CJ by measuring GGT levels, absence of hepatomegaly, presence of cholic stools and/or excretion on HIDA scan to undergo pecutaneous liver biopsy given the lower likelihood of BA necessary.

Thoracoscopic decortication for advanced pediatric empyema.

BACKGROUND: Although tube thoracostomy with fibrinolytic agents and video-assisted thoracoscopic (VATS) decortication are relatively equivalent in the treatment of empyema with regard to time to patient defervescence and hospital discharge, the potential for greater benefit with VATS decortication in the setting of advanced empyema has not been explored fully. This paper describes our transition from a policy of drainage and antibiotics to primary operative management with VATS. We wanted to assess the safety and efficacy of primary operative management as a first-line treatment for advanced disease. METHODS: A retrospective review was conducted of 25 patients treated for stage 3 or 4 empyema. The primary endpoints were morbidity and death. The secondary endpoints were conversion to an open procedure, time to defervescence, and length of hospital stay. RESULTS: The morbidity rate was 16% with no deaths. No patient required conversion to open decortication. The mean time to defervescence was 3.2 days, and on average, patients were discharged on postoperative day 9. Patients were discharged home earlier when managed primarily with VATS. CONCLUSIONS: Video-assisted thoracoscopic decortication is a safe and effective treatment for pediatric stage 3 or 4 empyema.

Line positions and line strengths for the 3<--0 electric quadrupole band of H2 1Sigmag +.

Several rotational lines in the S and Q branches [including the previously unobserved Q(2) and Q(3) lines] of the 3-0 electric quadrupole band of H2 have been detected by cavity ring-down spectroscopy. Line strengths were measured at densities between 2.7x10(18) and 7.5x10(19) molecules cm-3 at room temperature. The observed line strengths in the S branch are consistent with earlier measurements, and systematically below theoretical calculations [relative differences of approximately 10% for the S(1),S(2), and S(3) lines, and nearly 30% for the S(0) line]. Line strength measurements for the Q branch range from 25% to 33% below theoretical calculations.

Tumor size and extent of disease at diagnosis predict the response to initial therapy for papillary thyroid carcinoma in children and adolescents.

Treatment of papillary thyroid carcinoma (PTC) in children and adolescents is controversial. We previously showed that large tumor size, multifocal disease, and extensive disease at diagnosis predict recurrence. We examined 47 patients with PTC to determine whether these features predict response to treatment. Overall, 70% of the patients (33/47) remitted with initial treatment. 79% (15/19), of Class I, 86% (12/14) of Class II, and 100% (6/6) of Class III, but none of Class IV patients (n = 8) (p < 0.001) achieved remission. Tumor size for patients who entered remission (2.0 +/- 0.2 cm) was less than for patients with persistent disease (4.2 +/- 0.4) (p < 0.0005). Extent of disease at diagnosis correlated with the number of radioactive iodine (RAI) treatments (p = 0.022) and dose (p = 0.002) required to achieve first remission. We conclude that extensive disease at diagnosis and larger tumor size predict failure to remit after initial treatment of PTC in children and adolescents.

Unique management of stage 4S neuroblastoma complicated by massive hepatomegaly: case report and review of the literature.

Stage 4S neuroblastoma is an unusual malignancy that has an excellent prognosis, except in young infants. A 2-month-old with 4S neuroblastoma complicated by massive hepatomegaly, managed by abdominal decompression surgery and a negative-pressure dressing system is presented. Diffuse alveolar hemorrhage also developed, which was treated with high-dose corticosteroids.