Rapidly enlarging orbital tumor in a facial fibrous dysplasia lesion

Malignant change in fibrous dysplasia (FD) is a well-described phenomenon in the literature. When rapid enlargement is observed in an FD lesion, malignant change should be considered first. We present a case with McCune-Albright syndrome in which a rapidly enlarging orbital tumor developed in a facial FD lesion, which was subsequently proven to be a metastasis from an incidental esophageal carcinoma. Although metastasis of a malignant tumor to an FD lesion is quite rare and has been neglected as a differential diagnosis in the literature, it should be included in the important differential diagnosis because hematogenous metastasis could readily occur in FD due to its abundant blood flow. The importance of systemic examinations, such as positron emission tomography, is also emphasized.     

A case of combined glossopharyngeal and trigeminal neuralgia

It is well-known that idiopathic neuralgias of the trigeminal and glossopharyngeal nerves are caused by vascular compression at the root entry zone of the cranial nerves. Because they are functional diseases, initial treatment is medical, especially with carbamazepine. However, if medical therapy fails to adequately manage the pain, microvascular decompression (MVD) is prescribed. Glossopharyngeal neuralgia is rare, and combined trigeminal and glossopharyngeal neuralgia is an extremely rare disorder. A 70-year-old woman presented herself to Hokkaido Neurosurgical Memorial Hospital because of paroxysms of lancinating pain in her left pharynx and another lancinating pain in her left cheek. Carbamazepine, which was prescribed at another hospital, favorably relieved the pain; however, drug eruption compelled her to discontinue the medication. The multi-volume method revealed that a root entry zone of the left glossopharyngeal nerve was compressed by the left posterior inferior cerebellar artery, and the left trigeminal artery was compressed by the left superior cerebellar artery. MVD for both nerves was performed employing a left lateral suboccipital craniotomy. She experienced complete relief of pain immediately after MVD. Combined trigeminal and glossopharyngeal neuralgia is extremely rare, but some groups noted a relatively high incidence of concurrent trigeminal neuralgia in patients with glossopharyngeal neuralgia up until the 1970's. Glossopharyngeal neuralgia includes pain near the gonion; therefore, there is an overlap of symptoms between glossopharyngeal and trigeminal neuralgias. By virtue of recent progress in imaging technology, minute preoperative evaluations of microvascular compression are possible. Until the 1970's, there might have been some misunderstanding regarding the overlap of symptoms because of lack of the concept of microvascular compression as a cause of neuralgia and rudimentary imaging technology. Minute evaluations of both symptoms and imaging are very important.     

Hemangiopericytoma on the intradural thoracic spinal cord: a case report

Hemangiopericytoma develops from many organs. In the central nervous system, most tumors arise in the intracranial portion, and tumors originating from the spinal cord are rare. Its clinical course and neurological characteristics have not been disclosed. We present a case of a 51-year-old woman with gradually progressing paraparesis. Magnetic resonance (MR) images of the thoracic spine demonstrated an intradural tumor at the 6 and 7 thoracic vertebral body level. The patient underwent total excision of the tumor. The histological diagnosis was hemangiopericytoma. MR images after the operation showed no residual tumor and the patient was followed up without adjuvant therapy. However, 5 years later, the patient complained of back pain and gait disturbance again, and MR images showed a recurrence of the tumor. We resected the tumor under motor evoked potential (MEP) monitoring and removed the extradural part of the tumor, but the part of the tumor which had infiltrated the spinal cord was left due to the lowering of MEP amplitude. The operation resulted in partial resection. Spinal intradural hemangiopericytoma is very rare, and only 15 cases including the present case have been reported. This paper will discuss the clinical characteristics and treatment for this tumor.     

Left pulmonary artery banding to repair ipsilateral diffuse pulmonary arteriovenous fistula

ABSTRACT: Congenital pulmonary arteriovenous fistula (PAVF) is a rare disease which causes hypoxemia by shunting deoxygenated blood from the pulmonary artery into pulmonary venous return. Lung transplantation is the most effective therapy to treat severe, diffuse PAVF. However, the availability of lungs for transplantation is limited in most parts in the world. For patients with diffuse PAVF affecting only one side of the lungs, ipsilateral pulmonary artery banding (PAB) is an effective treatment, but not yet standard of care. We report successful treatment of a patient with diffuse left-sided PAVF with PAB. We believe that PAB is an effective therapy for severe unilateral PAVF and may serve as a bridge to lung transplantation.     

Aorto-pseudoaneurysm-ventricular regurgitation after aortic root replacement

We describe a 48-year-old man with aorto-pseudoaneurysm-ventricular regurgitation. It was due to disruption of both proximal and distal anastomoses after aortic root replacement for detachment of a prosthetic valve implanted for a sinus of Valsalva aneurysm with aortic valve regurgitation. The chest was opened during cardiopulmonary bypass using a deep hypothermic circulatory arrest to avoid rupture of the aneurysm because of a close relation between the pseudoaneurysm and the sternum. Aortic root re-replacement was performed successfully.     

Utility of the Dermatology Life Quality Index at initiation or switching of biologics in real-life Japanese patients with plaque psoriasis: Results from the ProLOGUE study

BackgroundPlaque psoriasis significantly affects patients’ health-related quality of life. To aid treatment decisions, not only objective assessment by physicians but also subjective assessment by patients is important.ObjectiveTo assess the significance of Dermatology Life Quality Index (DLQI) evaluation at the time of biologics introduction in clinical practice in Japanese patients with plaque psoriasis.MethodsThis was a single-arm, open-label, multicenter study. At baseline, Psoriasis Area and Severity Index (PASI) and DLQI scores were measured and stratified based on DLQI scores ≥6/≤5 and PASI scores ≤10/>10. Other patient-reported outcomes assessed included EQ-5D-5L, itch numerical rating scale (NRS), skin pain NRS, Generalized Anxiety Disorder-7 (GAD-7), Patient Health Questionnaire-8 (PHQ-8), Sleep Problem Index-II (SPI-II), and Treatment Satisfaction Questionnaire for Medication-9 (TSQM-9).ResultsOf the 73 enrolled patients, 23 had PASI scores ≤10. Those with PASI/DLQI scores >10/≥6 had a significantly higher median PASI score than those with PASI/DLQI scores >10/≤5 (p = 0.0125). Regardless of PASI scores (>10/≤10), median itch NRS and GAD-7 scores were significantly higher in patients with DLQI scores ≥6 than in those with DLQI scores ≤5 (itch NRS, p = 0.0361 and p = 0.0086, respectively; GAD-7, p = 0.0167 and p = 0.0273, respectively). Patients with PASI/DLQI scores ≤10/≥6 had significantly higher skin pain NRS (p = 0.0292) and PHQ-8 (p = 0.0255) scores and significantly lower median SPI-II scores (p = 0.0137) and TSQM-9 Effectiveness domain scores (p = 0.0178) than those with PASI/DLQI scores ≤10/≤5.ConclusionDLQI may be useful for assessing patients’ concerns that cannot be identified by PASI alone while initiating biologics or switching from other biologics in clinical practice.