Recurrence and Sphenoidal Prolapse of a Rathke Cleft Cyst following Endoscopic Transsphenoidal Resection Presenting with Delayed Spontaneous CSF Leak: A Case Report

Background and Importance Recurrence of Rathke cleft cysts following resection are rare in the neurosurgical literature, and there are no reports of patients presenting with cyst recurrence with concomitant prolapse into the sphenoid sinus and CSF rhinorrhea. Furthermore, sellar floor reconstruction is not recommended following resection if no CSF leak is seen intraoperatively to facilitate continued drainage. We present a patient who developed CSF rhinorrhea and sphenoidal prolapse of a recurrent Rathke cleft cyst 7 months after endoscopic transsphenoidal subtotal resection.

Clinical Presentation The patient initially presented with headaches and was found to have a cystic sellar lesion with suprasellar extension. Ophthalmologic examination revealed a mild bitemporal hemianopsia. At that time she underwent endoscopic transsphenoidal resection with both frozen section and final pathology consistent with a Rathke cleft cyst. At the time of closure, no CSF egress was seen with Valsalva maneuver, and bony reconstruction was not performed. Her post-operative course was uncomplicated until she returned 7 months after initial resection with a 2-week history of clear rhinorrhea which was positive for β-2-transferrin. A CT Head showed pneumocephalus within the sella, and an MRI brain showed a cystic lesion extending from the sella inferiorly into the sphenoid sinus concerning for a prolapsed Rathke cleft cyst. She was taken to the OR for endoscopic transsphenoidal exploration. Endoscopic visualization within the sphenoid sinus revealed a prolapsed cyst extending from the sella with a focal defect and spontaneous CSF egress. The distal portion of the cyst was resected followed by reduction with bipolar cautery. The cyst was not resected in its entirety due to concern for the presence of neural tissue within the cyst. Following adequate reduction, the mucosa surrounding the sella and the bony defect was removed. A fat graft harvested from the patient’s abdomen was then placed into the defect and buttressed with a septal cartilage graft. A nasal packing was used for several days to bolster the closure. No further CSF rhinorrhea was seen throughout the remainder of her hospital stay and the patient remains symptom free in clinical follow up.

Conclusion: Although recurrence of Rathke cleft cysts is reportedly rare following resection, we present a patient who developed prolapse of a recurrent cyst into the sphenoid sinus and who presented with frank CSF rhinorrhea 7 months after initial resection. Reconstruction of the bony defect was performed at the time of re-operation, and follow-up continues for this patient.

Fig. 1 Coronal CT Head showing pneumocephalus within the sella.

Fig. 2 Coronal T2 MRI brain showing cystic lesion extending into the sphenoid sinus.

Fig. 3 Sagittal T1 post-gadolinium MRI brain showing enhancing cystic lesion extending from the sella into the sphenoid sinus.