Scimitar Syndrome and H-type Tracheo-esophageal Fistula in a Newborn Infant

Lastinger, Allison; Yaman, Malek El; Gustafson, Robert A.; Yossuck, Panitan

doi:10.1016/j.pedneo.2013.06.010

Cited by 5 publications

(5 citation statements)

References 14 publications

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“…The first case was a case of SS with TEF as VACTERL association without a detailed description of imaging findings (2). The second case involved a SS with H-type TEF similar to the patient in the present case (8).…”

Section: Discussionmentioning

confidence: 62%

A Case of Scimitar Syndrome with H-Type Tracheoesophageal Fistula and Multiple Anomalies: Diagnosis Using Electrocardiography-Gated Chest CT

Lim

Kang

Lee

et al. 2014

J Korean Soc Radiol

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Section: Discussionmentioning

confidence: 62%

A Case of Scimitar Syndrome with H-Type Tracheoesophageal Fistula and Multiple Anomalies: Diagnosis Using Electrocardiography-Gated Chest CT

Lim

Kang

Lee

et al. 2014

J Korean Soc Radiol

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“…ere have been several reports in which Scimitar syndrome was diagnosed during the neonatal period [1,6]. In these reports, right pulmonary artery hypoplasia did not occur at birth.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with Scimitar syndrome have varied clinical presentations and are often diagnosed during adulthood because of the absence of symptoms. e exact developmental mechanism of right pulmonary artery hypoplasia and right lung hypoplasia in Scimitar syndrome is unclear [1]. Moreover, previous reports have not fully discussed the association between Scimitar syndrome and hemodynamic changes after birth.…”

Section: Introductionmentioning

confidence: 98%

Unusual Course of Scimitar Syndrome Preceded by Lung Hypoplasia

Hoshino

Arai

2019

Case Reports in Pediatrics

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In patients with Scimitar syndrome, right pulmonary artery hypoplasia is considered to lead to right lung hypoplasia because of decrease in blood flow. However, there are no reports wherein the change was actually detected. Thus, the exact developmental mechanism of right pulmonary artery hypoplasia and right lung hypoplasia in patients with Scimitar syndrome is unclear. We experienced a case of Scimitar syndrome preceding right lung hypoplasia, and right pulmonary artery hypoplasia gradually revealed with time. We hypothesized that, in our patient, the lung hypoplasia led to pulmonary artery hypoplasia due to decrease in blood flow. If there are no differences in the diameter of the left and right pulmonary artery in patients with Scimitar syndrome at birth, we propose the necessity of careful observation due to the possibility that pulmonary artery hypoplasia may develop in the future.

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“…Embolization of the aortopulmonary collaterals can also be done by detachable coils [11]. During our literature search, we came across other case reports of scimitar syndrome associated with other congenital defects such as VACTERL, H-type tracheoesophageal fistula [5,12], and imperforated anus [13]. We also came across case reports showing features of failure to thrive due to recurrent chest infections, leading to immediate repair of the defect.…”

Section: Discussionmentioning

confidence: 99%

Scimitar syndrome – Uncommon variant with common presentation

Dave¹,

Parikh²,

Vasoya³

2020

IJCR

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S cimitar syndrome is a complex association of cardiovascular and bronchopulmonary abnormalities, with the main feature of partial or total anomalous pulmonary venous drainage of the right lung to the systemic circulation [1]. It is characterized by partial anomalous pulmonary venous drainage of the right lung causing left-to-right shunt along with the right lung hypoplasia, dextraposition of the heart [2], and pulmonary hypertension due to aortopulmonary collaterals [3]. When visualized on imaging, it resembles a backsword with a curved blade, also known as scimitar in Middle East [4]. In infants, it can present as recurrent lung infection and can develop complications such as pulmonary arterial hypertension and congestive cardiac failure [5], while in adults, it usually remains asymptomatic [6]. It can be associated with other congenital heart defects such as single ventricular defect causing congestive cardiac failure [7] or atrial septal defects [8]. The surgical treatment includes rightsided pneumonectomy [9] or, a tunnel connecting the scimitar vein, and the left atrium can be made by Dacron graft through intra-atrial baffle technique [10]. CASE REPORT A 9-month-old male baby presented to the pediatric outpatient clinic with complaints of fever with cough, cold, and decreased oral intake for 5 days. His vitals demonstrated low-grade fever of 98.2°F with a pulse rate of 110/min, and respiratory rate of 36/ min. On physical examination, he looked pale and crepitations were heard over his chest. Therefore, he was admitted and commenced on IV antibiotics and nebulization. The laboratory findings showed anemia (6.70 g/dl), leukocytosis with counts of 13,200/mm 3 , and elevated C-reactive protein (CRP) levels of 23.40 mg/dl. The chest X-ray showed diffuse changes of bronchitis. His further laboratory investigations are mentioned in Table 1. His clinical condition improved over a period of time and there was a decrease in the count and CRP levels. His oral intake also improved gradually and thus was discharged after 3 days with the prescription of oral antibiotics. Following this, he had four bouts of respiratory tract infections with similar complaints and was treated on an outpatient basis. He underwent a 32 slice computed tomography (CT) scan with IV contrast which demonstrated non-visualization of the right main pulmonary artery and smaller right lung. The 2D echocardiography confirmed the hypoplastic right pulmonary artery with a small left-to-right patent ductus arteriosus shunt. The aortopulmonary collaterals were noted supplying to the right lung in the absence of the right pulmonary artery. These findings were in accordance with the scimitar syndrome variant with preferential blood flow to the left lung causing mild pulmonary artery hypertension.

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Scimitar Syndrome and H-type Tracheo-esophageal Fistula in a Newborn Infant

Cited by 5 publications

References 14 publications

A Case of Scimitar Syndrome with H-Type Tracheoesophageal Fistula and Multiple Anomalies: Diagnosis Using Electrocardiography-Gated Chest CT

A Case of Scimitar Syndrome with H-Type Tracheoesophageal Fistula and Multiple Anomalies: Diagnosis Using Electrocardiography-Gated Chest CT

Unusual Course of Scimitar Syndrome Preceded by Lung Hypoplasia

Scimitar syndrome – Uncommon variant with common presentation

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