Boston Children's Health Physicians

(1) Background: There has been an increase in the utilization of the minimally invasive vertical right axillary thoracotomy approach for repairing congenital heart defects in children recently. We aim, in the current study, to evaluate the outcomes of this approach in repairing anomalous pulmonary venous connections with or without an associated sinus venosus defect. (2) Methods: A total of 23 consecutive patients underwent surgical repair of anomalous pulmonary venous connections between April 2018 and February 2024. Perioperative and clinical follow-up data were obtained. (3) Results: The median age and weight were 36 months (1–277 months) and 14.4 kg (3.6–79.4 kg), respectively. More than half were females (13; 56.5%). There was no conversion to sternotomy. Partial anomalous pulmonary venous connections were the most frequent primary diagnoses (14; 60.9%), followed by scimitar syndrome (3; 13%), while two patients (8.7%) had total anomalous pulmonary venous connections. Repair techniques included single patch in 10 patients (43.5%), Warden in 6 (26.1%), and two-patch technique in 4 (17.4%). The median cardiopulmonary bypass and aortic cross-clamp times were 91 and 62 min, respectively. All patients were extubated in the operating room. The median length of hospital stay was 2 days. There were no mortalities or reoperations for pulmonary/systemic venous pathway obstruction. (4) Conclusions: Vertical right axillary thoracotomy is a valuable approach for repairing anomalous pulmonary venous connections with or without sinus venosus defects. All repair techniques, including Warden and scimitar, can be performed safely through this approach. The cosmetic superiority and short hospital stay make this approach worth considering.

To evaluate a novel stereoelectroencephalography (sEEG) headband designed to reduce artifacts during sEEG recordings in patients with drug-resistant epilepsy.

To describe four cases of Responsive Neurostimulation (RNS) in the bilateral pulvinar nuclei (PUL) in individuals with drug resistant epilepsy (DRE). This will show that due to widespread PUL connectivity, bilateral PUL RNS may be an option for some individuals with bilateral multifocal epilepsy. This study comprises two centers' experience with bilateral PUL RNS for DRE. Patients treated with bilateral PUL RNS at Westchester Medical Center (Valhalla, NY) and Corewell Health (Grand Rapids, MI) between the years 2019 and 2022 were analyzed and described. Presented here are methods for target selection, device programming, and clinical outcomes. Two patients with Lennox-Gastaut phenotype (aged 13 and 21 years) with posteriorly dominant discharges were implanted with bilateral PUL electrodes. Additionally, two patients (aged 20 and 31 years) with independent left and right occipital bilateral multifocal seizure onsets were implanted with bilateral RNS devices targeting the ipsilateral PUL and ipsilateral occipital cortex. Subclinical and clinical seizures were captured by RNS electrocorticography (ECoG) in all patients. RNS implantation and treatment was well-tolerated without adverse effects in all patients. Relative to baseline, two patients had 25% and 50% reduction in disabling seizures, and two patients had 71% and 100% reduction in disabling seizures. Stimulation paradigms utilized high frequency stimulation in both Lennox-Gastaut phenotype patients. Low frequency (individualized to the terminal ictal frequencies) stimulation was effective in the two bioccipital patients. RNS with electrode placement targeting bilateral PUL is safe, and no adverse effects have been attributable to the pulvinar electrode placement. PUL responsive neurostimulation is potentially effective for patients with bilateral multifocal, posteriorly dominant DRE. Both high and low frequency responsive stimulation are treatment options. Longer follow-up will shed light on the ultimate reduction of seizure burden. We describe four cases where stimulation devices were placed in the Pulvinar area of the thalamus (central sensory area in the brain). This is very unique and different location than where these devices are typically placed. These patients all had great outcomes with marked seizure reduction, demonstrating that this placement is both safe and effective.

Responsive neurostimulation (RNS) is often considered to be a palliative therapy for drug-resistant epilepsy (DRE) and is generally not considered to be a treatment for patients with tuberous sclerosis complex (TSC). Here, the authors present the case of a 24-year-old male with TSC who obtained seizure freedom following RNS device implantation. Prior to RNS device implantation, the patient underwent tuberectomies, subependymal giant cell astrocytoma resection, vagus nerve stimulator placement, and left frontal lobe resection but continued to have frequent seizures. An RNS device was implanted, which initially led to a decrease in seizures, but he continued to have 12 seizures per month. He then underwent lead revision for stimulation via a different electrode. After that lead change, he had no seizures for almost 3 years. In the following 3 years, he had two episodes of breakthrough seizures, both of which occurred with medication weans. Although the patient still requires antiseizure medication, he has had years of seizure freedom with RNS therapy. This study exhibits the potential effectiveness of RNS therapy for patients with TSC and DRE. RNS should be considered for patients with TSC when other therapies have not sufficiently treated their epilepsy. https://thejns.org/doi/10.3171/CASE23411.

Antibody-mediated rejection (AMR) is a major cause of kidney allograft loss. There is a paucity of large-scale pediatric-specific data regarding AMR treatment outcomes. Data were obtained from 14 centers within the Pediatric Nephrology Research Consortium. Kidney transplant recipients aged 1-18 years at transplant with biopsy-proven AMR between 2009 and 2019 and at least 12 months of follow-up were included. The primary outcome was graft failure or an eGFR <20 mL/min/1.73 m2 at 12 months following AMR treatment. AMR treatment choice, histopathology, and DSA class were also examined. We reviewed 123 AMR episodes. Median age at diagnosis was 15 years at a median 22 months post-transplant. The primary outcome developed in 27.6%. eGFR <30 m/min/1.73 m2 at AMR diagnosis was associated with a 5.6-fold higher risk of reaching the composite outcome. There were no significant differences in outcome by treatment modality. Histopathology scores and DSA class at time of AMR diagnosis were not significantly associated with the primary outcome. In this large cohort of pediatric kidney transplant recipients with AMR, nearly one-third of patients experienced graft failure or significant graft dysfunction within 12 months of diagnosis. Poor graft function at time of diagnosis was associated with higher odds of graft failure.

FEV /FVC normally decreases through childhood, increases briefly during early adolescence, and then declines throughout life. The physiology behind this temporary increase during early adolescence is not well understood. The objective of this study was to determine if this pattern also occurs in children with asthma. Design: Single-center, cross-sectional, retrospective analysis of pulmonary function tests (PFTs) obtained over a 5-year period in children 5 to 18 years of age with persistent asthma. Results: 1,793 patients satisfied all inclusion and exclusion criteria. Mean age (± SD) was 10.4 ± 3.8 years. 48% were female. Mean FEV /FVC was 0.83 ± .09. FEV /FVC in children with persistent asthma declined from age 5 to age 11 by 5.7 % compared to 7.3% in healthy girls, and 5.8% compared to 9.4% in healthy boys. FEV /FVC increased by 1.2% until age 16 in children with asthma, compared to 2.2% in healthy girls, and 2.5% compared to 2.3% in healthy boys. The ratio was lower in obese children with asthma at all ages but demonstrated the same curvilinear shape as in healthy children. In absolute terms, FEV grew proportionately more than FVC during early adolescence, so the ratio of FEV /FVC increased during that period. The curvilinear shape of the curve remained in postbronchodilator testing, though significantly blunted. Conclusions: The “Shepherd’s Hook” pattern in the FEV /FVC curve is preserved in children with persistent asthma. This was also true in obese patients with asthma, although their FEV /FVC ratios were lower throughout all stages of childhood and adolescence.