Intrasellar cysticercosis cyst treated with a transciliary supraorbital keyhole approach – A case report

Background: Neurocysticercosis is the most common parasitic disease affecting the central nervous system. Isolated sellar cysticercosis cysts are rare and can mimic other sellar lesion as cystic pituitary adenoma, arachnoid cyst, Rathke cleft cyst, or craniopharyngioma. The surgical resection is mandatory because the cysticidal drugs are ineffective, however, new microsurgical approaches are emerging to reduce complications and need to test in this condition. We present a patient with a sellar cysticercosis cyst treated by transciliar supraorbital keyhole approach. Case Description: A 45-year-old female with presented with chronic severe headaches, progressive deterioration of 6 months in visual acuity and bitemporal hemianopia. The pituitary hormonal levels were normal. Magnetic resonance findings showed a sellar and suprasellar cyst and underwent a microsurgical supraorbital transciliar keyhole approach for lesion resection. Pathologically, the lesion demonstrated a parasitic wall characterized by wavy, dense cuticle, and focal globular structure, surrounding inflammatory reaction with plasma cells. Postoperatively, the patient recovery fully neurologically. Conclusion: Intrasellar cysticercosis cyst causes significant neurological deficits due to its proximity to the chiasm, optic nerves, pituitary stalk, and the pituitary gland. Surgical section is an effective treatment. The supraorbital keyhole craniotomy offers satisfactory exposure, possibility of total resection with dissection of the supra and parasellar structures, short operative time, less blood loss, short hospital stay, and good overall surgical outcome.

Ten-Year Follow-Up of Collision Tumors Composed of Craniopharyngioma and Pituitary Adenoma: A Case Report and Literature Review

Although craniopharyngioma (CP) and pituitary adenoma (PA) are common tumors of the parasellar lesions, the coexistence of CP and PA is very rare. A 48-year-old male visited our hospital because of consciousness disturbance. The neuroimaging revealed a sellar tumor contact with a massive suprasellar cyst including calcification. Preoperative diagnosis was CP, and the patient underwent craniotomy to resolve the suprasellar mass effect. The histological examination disclosed adamantinomatous CP, and subsequently a transsphenoidal approach was chosen for the residual intrasellar tumor. Against expectations, the histological diagnosis was not CP but PA. The patient underwent gamma knife surgery for the residual tumor, and the postoperative course was good. After a 10-year follow-up, both lesions were still completely controlled. If we had suspected and diagnosed the tumor involved as not only CP but also PA at the first operation, the second operation could have been avoided because we would have chosen gamma knife surgery for the residual tumor. We should draw attention to this rare situation for differential diagnosis of parasellar tumor to avoid unnecessary surgery and to decide the best strategy for treatment. In addition, the biological behavior of collision tumors composed of CP and PA is probably the same as solitary CP or PA based on a long-term follow-up of our case.

Bobble-head doll syndrome: report of 2 cases and a review of the literature, with video documentation of the clinical phenomenon

Bobble-head doll syndrome (BHDS) is a rare pediatric movement disorder presenting with involuntary 2- to 3-Hz head movements. Common signs and symptoms also found on presentation include macrocephaly, ataxia, developmental delay, optic disc pallor or atrophy, hyperreflexia, tremor, obesity, endocrinopathy, visual disturbance or impairment, headache, and vomiting, among others. The syndrome is associated with suprasellar cysts, third ventricular cysts, or aqueductal obstruction, along with a few other less common conditions. The cause of involuntary head motions is not understood. Treatment is surgical. The authors present 2 cases of BHDS. The first is a 14-year-old boy with BHDS associated with aqueductal obstruction and triventricular hydrocephalus secondary to a tectal tumor. He was successfully treated by endoscopic third ventriculostomy, and all symptoms resolved immediately in the recovery room. This case is unusual in its late age of symptom onset, the primacy of lateral (“no-no”) involuntary head rotations, and the associated tectal tumor. The second case is a 7.5-year-old girl with BHDS associated with a suprasellar cyst. She was successfully treated with an endoscopic fenestration but preexisting endocrinopathy persisted, and the patient was diagnosed with autism spectrum disorder at age 12 years. This second case is more typical of BHDS. A comprehensive and up-to-date review of the literature of BHDS and video documentation of the phenomenon are presented.

Characteristics of Rathke's cleft cyst based on cyst location with a primary focus on recurrence after resection

OBJECT Rathke's cleft cysts (RCCs) are benign lesions with a location that is entirely intrasellar, intrasellar with suprasellar extension (intrasuprasellar), or purely suprasellar. The recurrence of RCC is relatively uncommon. The present study was conducted to report clinical characteristics, histological features, and outcomes based on location of the cyst with a primary focus on analyzing the predictors of squamous metaplasia and recurrence in these 3 types of RCCs. METHODS A retrospective review of the medical records of patients with symptomatic RCCs who had undergone resection at the authors' institution was conducted. Data points, including clinical presentation, preoperative endocrine status, operative details, imaging findings, pathology, and clinical outcomes, were reviewed. A multivariable regression model was used to identify predictors of recurrence. RESULTS The mean age of the 87 eligible patients, 64 females and 23 males, was 41 ± 14 years (range 10–73 years). Sixteen patients (18%) had an entirely intrasellar RCC, 21 (24%) had a purely suprasellar cyst, and 50 (58%) had an intrasuprasellar RCC. The mean cyst volume was 2.4 ± 0.9 cm3 (range 0.36–4.9 cm3). Headache was the most frequent initial symptom (76%) followed by visual disturbance (45%). The transsphenoidal approach was performed for all intrasellar RCCs (16 cysts) and 33 of 50 intrasuprasellar RCCs. The transcranial route was used for all suprasellar cysts (21 cysts) and 17 of 50 intrasuprasellar RCCs. Squamous metaplasia was present in 27 (31%) of 87 RCCs. The occurrence of squamous metaplasia was associated with cyst location (p = 0.027), T1 signal intensity (p = 0.004) and ring enhancement on Gd-enhanced MRI (p = 0.017), and cyst volume (p = 0.045). A suprasellar location (p = 0.048, OR 3.89, 95% CI 1.010–15.020), ring enhancement on Gd-enhanced MRI (p = 0.028, OR 3.922, 95% CI 1.158–13.288), hypointensity on T1-weighted MRI (p = 0.002, OR 6.86, 95% CI 1.972–23.909), and cyst volume (p = 0.01, OR 0.367, 95% CI 0.170–0.789) were independent predictors of squamous metaplasia. The mean time to reaccumulation (11 [12.6%] of 87 cases) and recurrence (7 [8%] of 87 cases) was 14 ± 6 months. Recurrence-free survival was 84.5% at a mean of 98.2 ± 4.6 months after treatment. A suprasellar cyst location (p = 0.007, OR 7.7, 95% CI 1.75–34.54), the occurrence of squamous metaplasia (p = 0.007, OR 19.3, 95% CI 2.25–165.18), and isointensity on T2-weighted MRI (p = 0.041, OR 10.29, 95% CI 1.094–96.872) were the independent predictors of RCC recurrence. CONCLUSIONS A suprasellar cyst location, the occurrence of squamous metaplasia, and isointensity on T2-weighted MRI were independent predictors of RCC recurrence. The extent of resection and type of surgical approach used were not associated with recurrence. A tailored extent of resection based on cyst location and predictive factors is recommended.

Spontaneous Rupture, Disappearance, and Reaccumulation of a Rathke's Cleft Cyst

Rathke's cleft cysts (RCCs) are benign epithelium-lined intrasellar cysts containing mucoid material and are believed to originate from the remnants of Rathke's pouch. Most are asymptomatic but may cause symptoms secondary to compression of adjacent structures such as visual disturbances and endocrinopathies, especially hypopituitary. Furthermore, inflammation such as an aseptic meningitis syndrome may be associated with these tumors, presumably resulting from leakage of cyst material into the subarachnoid space. We present a unique case of spontaneous rupture and complete disappearance of a known sella-suprasellar cyst associated with a severe headache syndrome, followed by cyst reaccumulation requiring surgery. Although this phenomenon is well accepted, to our knowledge, this is the first report of the complete disappearance of a Rathke's cyst presenting with the classic syndrome. Furthermore, it was remarkable how quickly it recurred and became symptomatic, providing evidence that an “empty sella syndrome” may indeed need clinical follow-up.