Rare Diseases Symptoms Automatic Extraction

Two-stage approach in the management of thoracic neuroenteric cyst with spinal extension: thoracoscopic excision following dorsal laminectomy.

[congenital diaphragmatic hernia]

Neuroenteric cysts (NC) are rare pathologies and localized generally in posterior mediastinum or abdomen where they may extend to spinal canal through a vertebral defect. Isolated spinal lesions require dorsal/ventral laminectomy and thoracic ones thoracotomy or thoracoscopy. Posterolateral approach via thoracotomy is generally performed for lesions with both thoracic and spinal components. Minimal invasive excision of a thoracic NC with spinal extension in an infant is presented herein.A term female newborn with an antenatal (26th week) diagnosis of congenital diaphragmatic hernia (CDH) was admitted. On physical examination, she was normal except mild dyspnea and CDH were excluded on radiogram. Left parenchymal opacity necessitated thorax tomography that revealed lobulated cystic lesion (6 × 3.5 × 4.5 cm) in posterior mediastinum. MRI showed intraspinal extension of the lesion through a hemivertebrae (6th). Two-stage procedure was planned for suspected neuroenteric cyst. First, intraspinal component was excised with dorsal laminectomy and the connection was closed. Then, the thoracic component was excised thoracoscopically. Histopathological evaluation confirmed the diagnosis. Total parenteral nutrition and high dose somatostatin analog was needed due to transient left chylothorax on postoperative course. She was well and symptom-free in postoperative period.Neuroenteric cysts may lead to misdiagnoses in antenatal period. MRI is critical to show spinal and vertebral pathologies in suspected cases. Thoracoscopy may safely be performed for thoracic lesions with spinal extension in two-stage approach following closure of the connection and excision of the spinal component.