Pituitary lymphoma appearing 9 years after pituitary adenoma resection.

Background: Pituitary lymphomas (PLs) are very rare, accounting for <0.1% of all intracranial tumors. Of which, PL that is associated with PL is even rarer. Here, we describe a case of PL of a 51-year-old woman that appeared 9 years after pituitary adenoma resection. Case Description: A 51-year-old woman presented with visual disturbance. She had a history of pituitary adenoma resected through endoscopic trans-sphenoidal surgery (eTSS) 9 years before. Although her previous annual follow-up did not show any signs of recurrence, she noticed visual disturbance. One month later, her visual acuity rapidly worsened with headache and fatigue, being referred to our hospital. On examination, she had bilateral quadrantanopia. Her laboratory data showed slightly increased prolactin levels. Magnetic resonance images showed a mass in the sella with suprasellar extension, so she underwent eTSS. The tumor had a fibrous, hard part and a soft gray part, and it was mostly resected. Visual symptoms improved transiently, but ophthalmoplegia appeared 2 weeks after surgery, indicating intrathecal dissemination. Histological analysis confirmed the diagnosis of T-lymphoblastic lymphoma. Positron emission tomography showed tracer accumulation at the pancreas, confirmed as lymphoma through biopsy. However, we could not determine which site of lymphoma was the primary site. She underwent chemotherapy, including cyclophosphamide, vincristine sulfate, doxorubicin hydrochloride, dexamethasone, and methotrexate. The patient died despite several months of treatment. Conclusion: Recurrence of pituitary adenoma cannot be carelessly assumed from a pituitary growing mass after pituitary adenoma resection. PLs have poor prognosis due to their aggressive character. Immediate biopsy and confirmation of the diagnosis are necessary for the treatment of pituitary masses with aggressive features.

Repeated recurrent intracerebral hemorrhage may be involved in cerebral amyloid angiopathy-related inflammation: An autopsy case.

We report an autopsy case of repeated recurrent intracerebral hemorrhage (ICH) diagnosed as having cerebral amyloid angiopathy-related inflammation (CAA-ri) in a 65-year-old woman. She had no history of hypertension or other risk factors for stroke. She had a history of lobar ICH in the right parietal lobe 30 months prior. Follow-up magnetic resonance imaging (MRI) performed 29 months after the initial ICH revealed superficial siderosis in the left frontal lobe. She initially presented with severe headache and dysarthria. An initial computed tomography (CT) revealed a subarachnoid hemorrhage on the left frontal lobe. One hour later, an epileptic seizure occurred, and another CT performed at the time revealed lobar ICH in the frontal lobe. Emergency surgical hematoma evacuation was performed. The cortical artery was removed from the surgical specimen. Hematoxylin and eosin (HE) staining revealed lymphocytic perivascular cuffs, and immunohistochemical staining revealed abundant amyloid-ß (Aß) deposits. The patient was diagnosed as having CAA-ri. On the next day, and 19, 46, 55, 78, 79, and 85 days after admission, ICH recurred. We did not administer immunosuppressive drugs, such as high-dose corticosteroids or cyclophosphamide, because of the patient's condition. Following the last ICH, the patient died on the 90th day after symptom onset. An autopsy was performed. Histologically, abundant Aß deposits were observed within the leptomeningeal and cortical arteries. Further dense-cored amyloid plaques were observed in the cortical samples. Our findings that ICH occurred initially three times in the left hemispheres and later five times in the right hemispheres were considered immune-mediated effects on vascular function. Further, superficial hemosiderosis in the left frontal lobe occurred before the first three ICH events, and hyperintensity of the white matter on MRI was identified in the right parietal lobe, a typical finding of CAA-ri. We hypothesize that the inflammatory process in CAA-ri may be related to recurrent ICH.

Negative Remodeling of Carotid Canal during Spontaneous Disease Progression in Moyamoya Disease.

OBJECTIVE: This study was designed to observe the changes in the carotid canal over time by measuring the carotid canal diameter longitudinally in adult patients with moyamoya disease in whom disease stage progressed spontaneously. METHODS: Of 70 adult patients with moyamoya disease, 10 adult patients on 15 sides with spontaneous progression of the disease stage during conservative follow-up were included in this study. Another 10 moyamoya cases on 15 sides were selected as the controls without disease progression. All patients were followed up for at least 60 months after the progression of disease stage was confirmed. In addition, 5 patients who underwent microvascular decompression were included as healthy controls. The carotid canal diameter was measured with bone window computed tomography (CT) and source images of time-of-flight magnetic resonance angiography at initial presentation, and then were serially monitored with the latter. RESULTS: There was a significant correlation between the values obtained from CT and magnetic resonance imaging (R2 = 0.992, P < 0.001). The carotid canal diameter in moyamoya disease at initial presentation was 4.29 ± 0.61 mm, being smaller than 5.20 ± 0.51 mm in healthy controls (P < 0.01). In response to disease progression, the carotid canal diameter started to decrease at 6 months after disease progression was confirmed, and reduced to about 85% of the original level during 60 months (P < 0.01). The phenomenon was not observed in patients without disease progression. CONCLUSIONS: The carotid canal diameter can decrease in response to disease progression even in adult moyamoya disease. "Negative" bone remodeling may play a key role in this unique phenomenon.

A case of intestinal perforation with a residual shunt tube placed during childhood: should we remove the non-functioning tube?

We report a 22-year-old man who had abdominal shunt tube migration into colon. He was diagnosed with pilocytic astrocytoma at optic-chiasm to hypothalamus at age of 7, and treated by resection, chemotherapy, and irradiation. He developed hydrocephalus and had multiple ventriculo-peritoneal shunt surgery. At age of 19, he fell in coma due to the subarachnoid and intra-ventricular hemorrhage due to the aneurysm rupture. The ventricle tube was removed, leaving the shunt valve and abdominal tube. The new shunt system was reconstructed at the contralateral side. He was at bed rest after this episode. At age of 20, he had high fever unable to control with antibiotics. The abdominal computed tomogram showed the shunt tube migration in the descending colon. The tube was removed under laparoscopy, and the inflammation was cured. The abandoned peritoneal shunt tube should be removed in patients with high tube migration risks.

Long-term Outcomes of Non-syndromic and Syndromic Craniosynostosis: Analysis of Demographic, Morphologic, and Surgical Factors.

In this study, we analyzed the outcomes of patients (followed for 5-38 years, average 17.3 years) with craniosynostosis and evaluated their long-term prognosis. In all, 51 patients who underwent surgery for craniosynostosis between 1982 and 2015, including 12 syndromic and 39 non-syndromic cases, were included. The average age at the initial surgery was significantly lower in the syndromic group than that in the non-syndromic group (9.8 months old vs. 19.9 months, respectively). The surgical procedures did not significantly differ between the two groups, but repeat surgery was significantly more common in the syndromic group than in the non-syndromic group (4 children [30.8%] and 3 children [7.7%], respectively). The children requiring repeat surgery tended to be younger at the initial surgery than those who did not. Those patients who required repeat surgery did not have significantly different surgical procedures initially. The incidence of developmental retardation was 49.0% (43.5% in the non-syndromic group and 66.7% in the syndromic group), and only two children in the non-syndromic group displayed recovery. This study is the first to analyze the prognosis for patients who were followed for at least 5 years after cranioplasty. Repeat surgery was common, especially in syndromic patients. Severity of skull deformity and early initial surgery may be important factors determining the need for repeat surgery. Developmental retardation was also common, and improvement was rare even after surgery.

[Three-Dimensional Skull Model with Vascular Structures for Occipital Expansion in Patients with Developed Occipital Emissary Veins].

We report the application of a three-dimensional skull model with vascular structures for occipital expansion in a patient who had developed occipital emissary veins. A 15-year-old boy visited a dentist for orthodontic treatment and was diagnosed with midface hypoplasia. He had undergone a ventriculo-peritoneal shunt at the age of 10 months. On visiting our institution, he was diagnosed with exophthalmos, low visual acuity, reversed bite, and developmental retardation. Moreover, he had sleep apnea and was supported with continuous positive airway pressure at night. Computed tomography and magnetic resonance imaging demonstrated brachycephalus, chronic tonsillar herniation, midface hypoplasia, and well-developed occipital emissary veins. We chose occipital expansion for lowering intracranial pressure and created a three-dimensional skull model with vascular structures and shunt tube. On this model, we wrote scalp incision and craniotomy lines avoiding injury to the veins and shunt tube. The operation was completed using this plan. Patients with syndromic craniosynostosis frequently have developed emissary veins and previous shunt operation. The three-dimensional skull model with vascular structures will be expected to reduce the risks of surgery.