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Inicio Revista Española de Anestesiología y Reanimación (English Edition) Scimitar syndrome: Anaesthetic management for pulmonary resection of the unaffec...
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Vol. 64. Issue 10.
Pages 594-599 (December 2017)
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Vol. 64. Issue 10.
Pages 594-599 (December 2017)
Case report
Scimitar syndrome: Anaesthetic management for pulmonary resection of the unaffected lung
Síndrome de la cimitarra: manejo anestésico para resección pulmonar del pulmón no afecto
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B. Fernández-Torresa,b,
Corresponding author
barfertor@hotmail.com

Corresponding author.
, A. Fernández-Lópeza, M. Congregadoa,b
a Hospital Universitario, Virgen Macarena, Sevilla, Spain
b Facultad de Medicina, Universidad de Sevilla, Sevilla, Spain
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Abstract

Scimitar syndrome is a rare congenital anomaly characterised by anomalous drainage of the right pulmonary veins in the inferior vena cava, frequently associated with right lung and pulmonary artery hypoplasia, dextrocardia and abnormal systemic arterial supply to the lower lobe. Pulmonary resection surgery on healthy lung is exceptional, and there are no published records of it, as far as we know.

A man with scimitar syndrome diagnosed with a lung nodule with malignant features in the contralateral lung. This situation implies huge anaesthetic complexity, mainly for intraoperative ventilation. Although spirometry and stress test did not contraindicate the planned lobectomy, scintigraphy showed a hypoplastic right lung with an uptake of 15%.

From an anaesthetic point of view we discarded selective ventilation of the right lung, since the shunt made it functionally non-existent. In consequence we proposed four anaesthetic possibilities. After the placement of an epidural catheter and left selective intubation, thoracoscopy with intermittent apnoeas was our first choice, and we could complete the extirpation and avoid excessive complexity.

Keywords:
Scimitar syndrome
Anomalous drainage of pulmonary veins
Anaesthesia
Unipulmonary ventilation
Unaffected lung
Resumen

El síndrome de la cimitarra es una rara anomalía congénita caracterizada por drenaje anómalo de las venas pulmonares derechas en cava inferior, asociado frecuentemente a hipoplasia del pulmón y arteria pulmonar derecha, dextrocardia y aporte arterial sistémico anómalo al lóbulo inferior. La resección sobre el pulmón sano resulta excepcional, y no conocemos antecedentes publicados.

Un varón con síndrome de la cimitarra es diagnosticado de nódulo pulmonar maligno del pulmón contralateral. Esta situación conlleva una enorme complejidad anestésica, fundamentalmente para la ventilación intraoperatoria. Aunque la espirometría y la prueba de esfuerzo no contraindicaban la lobectomía prevista, la gammagrafía objetivó un pulmón derecho hipoplásico con captación por debajo del 15%.

Descartamos la ventilación selectiva del pulmón derecho, ya que el shunt lo hacía funcionalmente inexistente, y planteamos 4 posibilidades anestésicas. Tras la colocación de un catéter epidural e intubación selectiva izquierda, la toracoscopia con apneas intermitentes fue nuestra primera elección.

Palabras clave:
Síndrome de la cimitarra
Drenaje venoso pulmonar anómalo
Anestesia
Ventilación unipulmonar
Pulmón no afecto

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