Thoracoscopic Repair of a Symptomatic Congenital Cervical Lung Herniation

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Author: American Pediatric Surgical Association
Specialties: Pediatric Surgery
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Stephen J. Fenton, MD, Justin H. Lee, MD.
University of Utah School of Medicine, Salt Lake City, UT, USA.

Congenital cervical lung herniation is an extremely rare cause of stridor and dysphagia. It more often occurs on the right and results from the disruption of Sibson’s fascia that allows for apical lung parenchyma to herniate into the neck. There is a known
association with Vitamin E deficiency, cleft lip and palate, and Cri-du chat syndrome. Surgical intervention is rarely required for spontaneous pneumothorax, stridor, dysphagia, or cosmetic issues due to the incarcerated lung tissue.

We report the thoracoscopic treatment of an infant with symptomatic congenital cervical lung herniation.

A previously healthy 9 month-old girl was evaluated with a several week history of progressive stridor and dysphagia. The stridor was more pronounced with crying and especially noted with crawling. The parents stated that she could not crawl for
prolonged distances due to increased work of breathing. She was also noted to have dysphagia and would choke while feeding unless held upright. The child appeared healthy with normal vital signs and was noted to have stridor on exam. Plain films of
the neck demonstrated herniation of the right lung apex into the thoracic inlet with significant displacement of the trachea. The child underwent an elective thoracoscopic repair. An opening below the Azygous vein was identified that allowed for herniation
of an apical lobe into the neck. Inflation of this trapped lobe caused displacement of the esophagus and trachea to the contralateral side resulting in her symptoms. The hernia was opened by division of the Azygous vein and Sibson’s fascia. The apical lobe was resected and the area reinforced with placement of biologic mesh. She had an unremarkable post-operative course with resolution of her dysphagia and significant improvement in her stridor allowing for normal activity.

A thoracoscopic approach to repair symptomatic congenital cervical lung herniation is feasible.

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