A right-sided aortic arch with an isolated left subclavian artery represents a rare anatomical variant, posing diagnostic challenges and clinical complexities. Here, we present a case of a 14-year-old male presenting with respiratory symptoms, unveiling a right-sided aortic arch with an isolated left subclavian artery. Through detailed clinical evaluation, radiographic imaging, and diagnostic modalities including chest radiography, computed tomography angiography, ultrasound, and time-of-flight magnetic resonance angiography, the anatomical features and associated complications were delineated. The discussion encompasses embryological underpinnings, clinical manifestations, and therapeutic considerations, shedding light on the rarity and clinical implications of this anomaly.

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BACKGROUND: Mediastinal tumors pose a challenging respiratory and circulatory management during anesthesia procedures, there is a risk of circulatory collapse or complete airway obstruction, which in severe cases can lead to cardiac arrest. We reported a case of anesthetic management using a bronchial blocker placed outside the tracheal tube. In this case report, the patient\'s trachea was so severely compressed that the airway was extremely narrow, only 4 mm at its narrowest point. By reporting the anesthetic management of this patient, we intend to provide an unusual approach for airway management.
METHODS: A 52-year-old male patient was admitted to the hospital due to cough and expectoration for one year. Additionally, the patient experienced chest tightness and asthma after physical activity. The enhanced computed tomography revealed there existed an irregular soft tissue mass in the right upper mediastinum, which significantly compressed the trachea and esophagus. The results of the mediastinal puncture pathology showed the presence of mesenchymal tumors. According to the results above, the patient was diagnosed with a mediastinal tumor and scheduled to undergo tumor resection under general anesthesia. We used a bronchial occluder outside the tracheal tube for general anesthesia. After surgery, the patient received thorough treatment and was subsequently discharged from the hospital.
CONCLUSIONS: In patients with severe airway compression from a mediastinal tumor airway compression, positioning a bronchial occluder externally to the tracheal tube is an effective method of airway management. However, we still need more clinical practice to help the process become more standardized.

UNASSIGNED: Lymphatic malformation (LM), most commonly present in the neck area, is benign vascular malformations of the lymphatic system. In an infant, however, LM poses a high risk of adverse outcomes.
UNASSIGNED: We present a case with a giant fetal LM. Through ultrasonography, at 23+ weeks of gestation, a septate cystic mass 7.2×6.5×6.3 cm in size was found on the right side of the fetus\'s neck. After extensive counseling by the multidisciplinary team, the parents chose to continue the pregnancy. Severe fetal tracheal compression was observed at 29 weeks by magnetic resonance imaging (MRI). At 31 weeks and 5 days, owing to suspected fetal distress, an emergency cesarean section was performed and a male baby weighing 1720 g was delivered. The mass was 10×16×8 cm in size and ex utero intrapartum treatment (EXIT) was implemented. Due to progressive growth of the mass secondary to intralesional bleeding, an intralesional injection of bleomycin was administered three days later. This injection was repeated at the age of 1 month and 8 days. The baby was followed up and, by a year after his birth, LM had disappeared. The baby has since been in good health.
UNASSIGNED: Accurate prenatal diagnosis and regular monitoring of a fetus with LM may improve prognosis. It is essential to have a trained multidisciplinary team to evaluate the condition of the fetus and the neonate and to provide treatment based on the evaluation. Our experience with intralesional bleomycin injection for the treatment of a giant fetal neck LM in a preterm infant had a favorable outcome. Long-term follow-up by a multidisciplinary team is needed in such cases.

We present the clinical course and echocardiographic and genetic findings of two fetuses with an unusual vascular ring, created by a left aortic arch with a right arterial duct and an aberrant right subclavian artery. One fetus was diagnosed with 22q11.2 microdeletion and the other became symptomatic in infancy. It is important to consider the position of the arterial ductal ligament in patients who present with tracheoesophageal compressive symptoms in the presence of a left aortic arch. These cases also highlight that a vascular ring formed from a left arch may have similar associations to a vascular ring formed by a right aortic arch.

BACKGROUND: Tracheal compression (TC) due to vascular anomalies is an uncommon, but potentially serious cause of chronic respiratory disease in childhood. Vascular slings are congenital malformations resulting from abnormal development of the great vessels; in this group of disorders the most prevalent entity is the aberrant innominate artery (AIA). Here we provide a report on diagnosis and treatment of AIA in nine children with unexplained chronic respiratory symptoms. We describe the cases, perform a literature review, and provide a discussion on the diagnostic workup and treatment that can help manage AIA.
METHODS: Clinical history, diagnostic procedures and treatment before and after the AIA diagnosis were retrospectively reviewed in nine children (5 boys and 4 girls), who were referred for recurrent-to-chronic respiratory manifestations over 10 years (2012-2022). We performed a comprehensive report on the ongoing clinical course and treatment as well as an electronic literature search on the topic.
RESULTS: Diagnoses at referral, before AIA was identified, were chronic dry barking cough associated with recurrent pneumonia (n = 8, 89%), lobar/segmental atelectasis (n = 3, 33%), atopic/non atopic asthma (n = 3, 33%); pneumomediastinum with subcutaneous emphysema complicated the clinical course in one case. When referred to our Unit, all patients had been previously treated with repeated antibiotic courses (n = 9, 100%), alone (n = 6, 67%) or combined with prolonged antiasthma medications (n = 3, 33%) and/or daily chest physiotherapy (n = 2, 22%), but reported only partial clinical benefit. Median ages at symptom onset and at AIA diagnosis were 1.5 [0.08-13] and 6 [4-14] years, respectively, with a relevant delay in the definitive diagnosis (4.5 years). Tracheal stenosis at computed tomography (CT) was ≥ 51% in 4/9 cases and ≤ 50% in the remaining 5 subjects. Airway endoscopy was performed in 4 cases with CT evidence of tracheal stenosis ≥ 51% and confirmed CT findings. In these 4 cases, the decision of surgery was made based on endoscopy and CT findings combined with persistence of clinical symptoms despite medical treatment. The remaining 5 children were managed conservatively.
CONCLUSIONS: TC caused by AIA may be responsible for unexplained chronic respiratory disease in childhood. Early diagnosis of AIA can decrease the use of expensive investigations or unsuccessful treatments, reduce disease morbidity, and accelerate the path toward a proper treatment.

OBJECTIVE: The aim of this study was to assess the surgical and follow-up outcomes in children who operated for aberrant innominate artery.
METHODS: A total of 15 consecutive patients (12 males, 3 females; mean age 16.3 ± 19.0 months; range 3 months to 6 years) who underwent aortopexy between February 2018 and December 2021 were evaluated. Demographic data, preoperative and postoperative clinical status and postoperative outcomes were retrospectively analyzed.
RESULTS: The mean age at operation was 16.3 ± 19.0 months. The median weight was 8.3 kg (range, 7-14.5 kg).There was no complications at intraoperative period. The mean percent degree of tracheal stenosis was 0.68 ± 0.12. The median (range) MV duration, PICU stay, and ward stay of the patients were 2 h (0-3 h), 2.5 days (1-4 days), and 5 days (3-8 days), respectively. The mean patients\' number of emergency service applications and hospitalization at the preoperative period was 6.2 ± 3.9/2.3 ± 1.6 and, at the postoperative period was 3.3 ± 2.2/0.9 ± 0.8. In comparison of the preoperative and postoperative service application number and hospitalization number, there was significant difference (p < 0.005 and 0.006, respectively). No reoperation was required. There was no mortality.
CONCLUSIONS: Aberrant innominate artery is rarely seen. These pathologies misdiagnosis with different reactive airways. Following the diagnosis, treatment can be achieved by surgery successfully.

Retrosternal extension of the goiter can cause compression of the trachea, esophagus and major blood vessels. Airway management is indeed a challenge in patients with airway obstructive signs and symptoms and it is based on the severity of the patient\'s clinical symptoms, availability of airway equipments, familiarity and expertise. We encountered a patient with retrosternal goiter with tracheal compression, presented for surgery required pediatric Fiber-Optic Bronchoscope (FOB) for securing the airway. A 45 year female patient presented with a swelling in front of the neck for 6 months. Recently, she developed intermittent stridor which was aggravated by lying supine. In computed tomography, there was a retrosternal extension of thyroid gland into superior mediastinum causing tracheal compression and narrowing (80%). Awake fiber-optic intubation with paediatric FOB was used to secure the airway before induction of anaesthesia. Paediatric FOB can be useful to secure airway in patients with tracheal compression and narrowing.

Double aortic arch is the most common form of complete vascular ring. The trachea and/or esophagus could be compressed by the complete vascular ring, which may lead to early respiratory and/or esophageal symptoms in children with double aortic arch. Accurate prenatal assessment of tracheal compression could provide relevant information for perinatal clinical management of double aortic arch and emergency treatment of infants with double aortic arch. The fetal trachea is filled with amniotic fluid and can be clearly visualized with prenatal ultrasound. Previous studies reported the use of prenatal ultrasound to measure the tracheal internal diameters in normal fetuses and showed a linear correlation between the fetal tracheal internal diameters and gestational age. However, to the best of our knowledge, few studies have quantitatively evaluated tracheal compression in fetuses with double aortic arch using ultrasound.
This study aimed to evaluate the tracheal compression caused by the vascular ring in fetuses with double aortic arch using prenatal ultrasound and to analyze the relationship between tracheal compression and postnatal clinical symptoms.
The data of fetuses with double aortic arch diagnosed with prenatal ultrasound at 2 institutions from January 2011 to April 2021 were retrospectively analyzed. Singleton pregnancies with normal fetuses as the control group were prospectively recruited. The tracheal compression-evaluated by comparing the tracheal internal diameter z scores against the gestational age-was assessed in fetuses with double aortic arch and in normal fetuses. The live-born infants with double aortic arch were divided into symptomatic and asymptomatic groups for the comparison of z scores. The receiver operating characteristic curve for the tracheal internal diameter z score cutoffs and prediction of symptomatic infants with double aortic arch was plotted. Intraobserver and interobserver agreements were investigated.
A total of 26 fetuses with double aortic arch were diagnosed, and 14 fetuses (53.8%) with double aortic arch were delivered alive. Among the 14 live-born infants, 7 (50.0%) were symptomatic, whereas 7 (50.0%) were asymptomatic. The tracheal internal diameter z scores were significantly lower in the double aortic arch group than in the normal groups (-0.62±1.36 vs 0.00±0.78; P<.001). The tracheal internal diameter z scores were significantly lower in the symptomatic group than in the asymptomatic group (-1.42±0.92 vs -0.49±0.96; P=.018). The area under the curve was 0.878 (95% confidence interval, 0.689-1.000). Using a tracheal internal diameter z scores cutoff of -1.21, the sensitivity was 71%, and the specificity was close to 100%. The intraclass correlation coefficients of interobserver and intraobserver agreements were 0.987 (95% confidence interval, 0.980-0.992) and 0.975 (95% confidence interval, 0.955-0.987), respectively.
The clinical symptoms in infants with double aortic arch were associated with prenatal tracheal compression, which can be prenatally evaluated using ultrasound. If fetuses are diagnosed with double aortic arch, prenatal surveillance of the tracheal internal diameters and comparison with z score reference ranges could provide pertinent information that would aid perinatal clinical management.

An 82-year-old male was referred to the emergency department for severe respiratory distress. Computed tomography angiography showed tracheal compression due to a large ruptured saccular aneurysm of the descending thoracic aorta. Emergency Thoracic Endovascular Aneurysm Repair (TEVAR) was performed. To reduce tracheal compression, an endotracheal stent was placed (silicone Dumon©). Following surgery, respiratory function improved. Two days after the surgery, the patient refused further invasive treatment, including mechanical mucus aspiration from the endotracheal stent, and palliative sedation was initiated. Conventional treatment to reduce tracheal compression by a saccular aortic aneurysm is open surgical aneurysm repair. If open repair is contraindicated because of patient age, comorbidity, or in case of severe hemodynamic instability following aneurysm rupture, TEVAR with endotracheal stent placement may serve as a bridge to definite surgery to reduce tracheal compression.