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A patient in their mid-40s presented with a history of worsening dyspnoea. The patient had neurofibromatosis type 1 and had previous spinal surgery for kyphoscoliosis.
A chest x-ray showed a right sided ‘white out’, thought to be a pleural effusion (figure 1A). CT thorax showed a thoracic scoliosis with internal fixation and a large fluid collection within the right hemithorax. A chest drain was scheduled to be inserted.
However, further review of the CT images identified an unusual abnormality within the right hemithorax. Several of the thoracic spinal pedicles were absent and there was communication of fluid from a dilated spinal canal to the large fluid collection within the right hemithorax (figure 1B).
A thoracic MR image confirmed the appearance was due to a large cerebrospinal fluid collection within the right hemithorax, which directly communicated to the right side of the spinal canal (figure 1C). The appearances were due to a huge intrathoracic meningocoele rather than a pleural effusion.
An intrathoracic meningocoele is a rare abnormality due to herniation of spinal meninges through an intervertebral foramen or bony deformity, producing a cerebrospinal fluid filled sac.1 Despite being rare, it is often associated with neurofibromatosis type 1.2
Failure to recognise this rare presentation may have resulted in inappropriate chest drain insertion with potentially disastrous consequences.
This case illustrates a rare cause of ‘white out’ of the lung and highlights the importance of recognising an intrathoracic meningocoele in the context of neurofibromatosis type 1.
This extremely rare abnormality could easily have been misinterpreted as a large pleural effusion which if drained may have led to potentially fatal consequences.
Competing interests None.
Patient consent Detail has been removed from this case description. The editors and reviewers have seen the detailed information available and are satisfied that the information backs up the case the authors are making.
Provenance and peer review Not commissioned; internally peer reviewed.
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