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Thoracoscopic excision of double-pulmonary sequestration in left haemithorax, without anomalous blood supply (supply from pulmonary artery) in a neonate: First case
Sunita Ojha1, Prabhar Srivastava2, Anil Poonia2, Rajiv Bansal1
1 Department of Neonatal and Pediatric Surgery, Santokba Durlabhji Memorial Hospital and Research Institute, Jaipur, Rajasthan, India
2 Department of Pediatric, Santokba Durlabhji Memorial Hospital and Research Institute, Jaipur, Rajasthan, India
|Date of Submission||11-Sep-2020|
|Date of Decision||30-Oct-2020|
|Date of Acceptance||24-Nov-2020|
|Date of Web Publication||06-May-2021|
Department of Neonatal and Pediatric Surgery, Santokba Durlabhji Memorial Hospital and Research Institute, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
Pulmonary sequestration (PS) is a rare anomaly. PS is a mass of abnormal pulmonary tissue that does not communicate with the tracheobronchial tree and is supplied by an anomalous systemic artery. Although aberrant systemic arterial supply is considered the key element to diagnose PS, rarely it can have arterial supply from the pulmonary artery as a spectrum of sequestration. Here, we present an unusual case of double (upper and lower) extralobar sequestration, present unilaterally (left haemithorax) in a neonate, without anomalous blood supply (supply from the left pulmonary artery and drainage into the superior and inferior pulmonary veins), not reported in literature earlier, to the best of our knowledge. In the present case, a child presented with respiratory discomfort at birth, requiring surgery due to inability to wean off respiratory support. It is important to be aware of this variant of sequestration spectrum. In a 18 days old child, both pulmonary sequestrations were resected thoracoscopically, making it a rare case, not described earlier in literature.
Keywords: Double-lung sequestration, pulmonary sequestration, pulmonary arterial supply
|How to cite this URL:|
Ojha S, Srivastava P, Poonia A, Bansal R. Thoracoscopic excision of double-pulmonary sequestration in left haemithorax, without anomalous blood supply (supply from pulmonary artery) in a neonate: First case. J Min Access Surg [Epub ahead of print] [cited 2021 Jun 14]. Available from: https://www.journalofmas.com/preprintarticle.asp?id=315533
| ¤ Introduction|| |
Pulmonary sequestration (PS) is a rare anomaly with 0.15%–1.8% incidence. By definition, PS is a mass of abnormal pulmonary tissue that does not communicate with the tracheobronchial tree and is supplied by anomalous systemic artery. Surgical interest in these lesions arose when operative injury to anomalous blood supply resulting in patient death was first reported in 1940. We describe an unusual case of double (upper and lower) extralobar sequestration in the left haemithorax of a neonate without anomalous blood supply (supply from the left pulmonary artery and drainage into the superior and inferior pulmonary veins), not reported in literature earlier.
| ¤ Case Report|| |
A full-term, 2.5-kg, male baby presented with respiratory distress at birth requiring resuscitation. Chest X-ray showed a large mass in the left haemithorax at the mid zone and at the basal zone. Computed tomography (CT) scan of the chest revealed a large mass in the left side of the mediastinum, extending from the anterosuperior mediastinum to the supra-diaphragmatic region, occupying most of the left haemithorax. The mass was compressing the left lung posteriorly, causing mediastinal shift to the right side. Arterial supply was from the left pulmonary artery and drainage was into the superior and inferior pulmonary veins. No bronchial communication into this mass was identified [Figure 1]. Bronchogram showed normal tracheobronchial tree. The child was managed on high-flow nasal cannula (HFNC) for few days. The large size of the mass, severe retractions and inability to wean off HFNC, led to the decision of surgical resection.
|Figure 1: Computed tomography scan – Two mediastinal masses extending into the upper and lower left haemithorax with branch from the pulmonary artery|
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The child was operated thoracoscopically on day 18 of life, with normal bilateral lung ventilation. He was placed in the supine position, with the left side slightly elevated. The first port (5 mm) was placed in the anterior axillary line at the fifth intercostal space, the second port (3 mm) was placed cephalad in the anterior axillary line, while the third port (5 mm) was placed anterior to the anterior axillary line in the sixth intercostal space. Pressure of 4–5 mmHg and flow of 4 l/min were used for thoracoscopy. Upper sequestration had arterial supply from the left pulmonary artery just before take-off of blood supply for the lung. Venous drainage was into the superior pulmonary vein. Lower sequestration was supplied by the pulmonary artery and was draining into the inferior pulmonary vein. The vascular pedicle was dissected, clipped and sequestrations resected, taking care of supply to the remaining lung [Figure 2]. No bronchial communication was seen in both extralobar sequestrations. The lung expanded well. Specimens (4 cm × 5 cm/4 cm × 4 cm) were retrieved through a 5-mm port site after extending it to 12 mm. One chest tube (12 Fr) was placed through the same site. No intraoperative anaesthetic complications were encountered as saturation was well maintained by high ventilatory rates (40–50/min) and low-ventilatory pressure (10/4 peak inspiratory pressure/positive end-expiratory pressure). Duration of the surgery was 170 min with negligible blood loss. Histopathology confirmed it as lung sequestration. The child was extubated post-operatively and weaned off HFNC in 48 h; the chest tube was removed on the 2nd post-operative day. The child recovered well and discharged on the 5th post-operative day.
|Figure 2: Intraoperative picture. Solid arrow (vascular pedicle of lower sequestration, branch from the pulmonary artery and drainage to the inferior pulmonary vein), arrow at the clips of vascular pedicle of upper resected sequestration|
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| ¤ Discussion|| |
PS, also known as accessory lung, is a non-functioning primitive lung that has aberrant systemic blood supply but no trachea-bronchial communication. PS can be extralobar or intralobar. About 15%–25% of sequestrations are extralobar. Treatment is surgical resection. Surgery in symptomatic congenital lung malformations is associated with higher perioperative complications, longer operative time, increased blood loss and post-operative morbidity. If untreated, outcome may be complicated; even fatal, because of pulmonary hypoplasia due to impaired lung development.
Maldevelopment involving pulmonary parenchyma or vessels or a combination of both is called 'sequestration spectrum'. At the one end of the spectrum is anomalous vascular supply to the non-sequestrated lung, whereas at the other end is abnormal pulmonary tissue without anomalous vascular supply. Between these two spectra lie variants of sequestrations. Case of bilateral sequestration with anamolous supply, communicating with stomach requiring laparotomy and thoracotomy has been reported by Joy et al. To the best of our knowledge double sequestration unilaterally with pulmonary arterial supply has not been described ever. We managed this case thoracoscopically, helping in quick recovery of the child.
Only one case of pulmonary-sequestrated segment with an aberrant pulmonary arterial supply in a 64-year-old male had been described, but the patient was not operated.
| ¤ Conclusion|| |
Surgeons and radiologists should be aware and careful about the variation in sequestration spectrum from anomalous vascular supply to a non-sequestrated lung, to abnormal pulmonary tissue without anomalous vascular supply.
Two sequestrations in one haemithorax, in a neonate, without anomalous arterial supply, resected thoracoscopically, have never been reported earlier in literature, to the best of our knowledge.
We acknowledge the contribution of Dr Gajendra Gupta and Dr Ashesh Shah for technical and material support and Arpita Chippa and Jophy Jose for technical assistance.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| ¤ References|| |
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[Figure 1], [Figure 2]