[A Case of IgG4-related Hypertrophic Pachymeningitis].

Hypertrophic pachymeningitis is a chronic inflammatory disease, manifesting as a fibrous thickening of the dura mater. Recently, it was reported that IgG4 is related to the etiology of some diseases, such as pancreatitis. Here, we report a case of hypertrophic pachymeningitis related to infiltration of IgG4 positive plasma cells. A 68-year-old man presented at a nearby hospital with a headache and a low-grade fever. A blood test revealed inflammation, as well as elevation of IgG4 level. Magnetic resonance imaging(MRI)revealed diffuse thickening of the dura mater, dominantly in the posterior fossa and cerebellar tentorium. The lesion was enhanced significantly with gadolinium(Gd). An open biopsy was performed to determine pathological diagnosis. Hematoxylin and eosin staining showed infiltration of inflammatory cells, including plasma cells. The infiltrating cells were positive for the IgG4. Post-operatively, the patient was treated with glucocorticoid, and both the inflammation and patient symptoms were improved. In conclusion, IgG4 is related to the etiology of hypertrophic pachymeningitis and glucocorticoid therapy is effective for this disease.

[A Rare Case of Ruptured Internal Carotid-Posterior Communicating Artery Aneurysm Associated with Contralateral Delayed Oculomotor Nerve Palsy].

A 55-year-old man presented with difficulty breathing, chest pain, and disturbance of consciousness, and was transferred to our hospital. Initial whole body CT revealed a diffuse subarachnoid hemorrhage(SAH)with severe pulmonary edema that was considered neurogenic in origin. He received controlled ventilation under sedation and conservative care for the SAH. One day after the onset of the SAH, his left pupil suddenly became dilated to 6mm, with no reaction to light. Head CT showed no new bleeding. Subsequent CT angiogram revealed a right internal carotid-posterior communicating artery aneurysm(IC-PC AN)with a posterior-lateral projection; however, no vessel abnormality was observed in the left anterior or posterior circulations. The aneurysm was successfully treated with coil embolization. We reviewed reported cases of isolated oculomotor nerve palsy(ONP), without direct compression by ruptured aneurysms, and found that only 11 cases exist, including our case. Interestingly, six of them were associated with anterior communicating artery aneurysms. A mass effect, chemical stimulation, and a jet stream of blood were proposed as the mechanisms of this rare type of ONP, in addition to the specific neurovascular relationships between the oculomotor nerve and the posterior cerebral/posterior communicating/superior cerebellar arteries, with abnormal nerve contact or compression. Though rare, ruptured IC-PC ANs could cause contralateral ONP; other types of ruptured aneurysms may also lead to indirect effects on nervous structures without direct compression. This type of rare presentation of ruptured aneurysms should be considered, especially in cases of multiple aneurysms, such as IC-PC ANs.

[A Rare Case of Ruptured/Unruptured Miniature Aneurysms Originating from the Infundibular Dilatation of the Callosomarginal Artery, Branching from the Infracallosal(A2)Segment of the Azygos Anterior Cerebral Artery].

We report the first case of two de novo miniature aneurysms(ruptured/unruptured)emerging from the infundibular dilatation(ID)of the callosomarginal artery, which branches from the infracallosal(A2)segment of the azygos anterior cerebral artery(AACA), in a 36-year-old woman. The patient had previously been diagnosed with a miniature, unruptured aneurysm, occurring in the A2 segment of the AACA, detected by CT angiography(CTA)at another hospital two years ago, and had been followed up with MR angiography(MRA)every 6 months. Three months after the final check-up with MRA, which did not indicate a significant change in the aneurysm, the patient presented with subarachnoid hemorrhage. Subsequent CTA and digital subtraction angiograms revealed that the right callosomarginal artery, originating from the apex of the aneurysmal bulge, had a maximal diameter of 3mm, indicating an ID of the artery. Additionally, two miniature bleb-like aneurysms emerged from the ID, projecting in opposite directions:one projecting to the right-posterior/superior direction and the other to the left-anterior/inferior direction. Both aneurysms were successfully clipped via a right pterional approach with partial resection of the gyrus rectus, and the right-projecting aneurysm was confirmed to be ruptured during surgery. In the current report, we review previously reported cases of AACA with aneurysms, and discuss their clinical characteristics, and the possible mechanisms underlying the formation of the ID and de novo aneurysms in this extremely rare case.

[A Case of Ruptured Internal Carotid-Posterior Communicating Artery Aneurysm Associated with Acute Subdural Hematoma, Extending from the Interhemispheric Space to the Posterior Fossa].

A 69-year-old woman was admitted to our hospital because of a sudden severe headache without a history of head trauma. CT and MRI revealed an acute subdural hematoma (ASDH) extending from the right interhemispheric space to the posterior fossa bilaterally, with a small amount of subarachnoid hemorrhage that was predominantly localized to the left side of the basal cistern. CT angiogram demonstrated a long protruding ruptured aneurysm at the junction of the right internal carotid and posterior communicating arteries (IC/PC AN) with a posteroinferior projection, associated with a small bleb located near the tentorial edge close to the ipsilateral posterior clinoid process, for which she received clipping surgery. Though rare, IC/PC AN could cause pure or nearly pure ASDH in the above-mentioned distribution. Therefore, in patients with such ASDH, especially without a history of head injury or precise information regarding the situation at the time of onset, urgent imaging evaluation and early intervention are essential to prevent devastating re-rupture events.

CT findings, prognosis, and follow-up of pulmonary artery periadventitial hematoma with aortic dissection: a retrospective single-center study.

Background: Pulmonary artery periadventitial hematoma (PAPH) with aortic dissection (AD) is a rare condition but has been reported to correlate with prognosis. However, there are few cases of PAPH, and the relationship with computed tomography (CT) findings of AD are unknown. This study aimed to evaluate CT findings and early prognosis in patients with PAPH in AD. Methods: This was a retrospective analysis of data from patients with Stanford type A AD diagnosed with contrast-enhanced CT in our institution from April 2008 to February 2023; 316 patients were included in the analyses. Patients comprised a PAPH group (n=78) and a non-PAPH group (n=238). The PAPH group was further divided into a group that died within 1 week of onset (death group; n=15) and a group that survived (alive group; n=63). PAPH was classified into three grades on the basis of the CT findings, as follows: Grade 1: PAPH only in the mediastinum; Grade 2: PAPH that extended into the lung field, with/without interlobular septa; and Grade 3: PAPH with pulmonary hemorrhage. Results: Compared with the non-PAPH group, the PAPH group had higher rates of early death (P=0.001), pericardial (P<0.001) and mediastinal hemorrhage (P<0.001). When comparing the death and alive groups, there was a significant difference in the rates of inoperable case (P<0.001), Grade 3 PAPH (PAPH with pulmonary hemorrhage) (P<0.001), and hemothorax (P=0.02). Multivariable analysis showed a significant association between Grade 3 PAPH (PAPH with pulmonary hemorrhage) and early death (P=0.004). Conclusions: Standard type A AD with PAPH is not rare. Mortality was higher in the PAPH group vs. the non-PAPH group, and Grade 3 PAPH (PAPH with pulmonary hemorrhage) was a significant risk factor for early death.

A case of chylous ascites caused by reflux from the cisterna chyli to the disrupted common iliac lymphatic vessel: fluoroscopic records during intranodal lymphangiography.

A 40-year-old woman underwent laparoscopic common iliac lymphadenectomy for metastasis from rectal cancer. Two weeks after the surgery, she was found to have massive chylous ascites. After failure of conservative treatment, bilateral inguinal intranodal lymphangiography was performed. No definite extravasation was observed while lipiodol injected through the left inguinal node was ascending. When we punctured the right inguinal lymph nodes and started the injection of lipiodol, extravasation of diluted lipiodol was noted at the level of the first sacrum. Careful observation revealed that the ascending lipiodol became diluted in the cisterna chyli, refluxed through the median paraaortic route, leaked from the excised left common iliac lymph vessel, and flowed into the abdominal cavity. Lipiodol used in lymphangiography did not reduce chylous ascites at all. Twenty-seven days after lymphangiography, laparoscopic lymphatic ligation was performed, and the chylous ascites disappeared completely. CT obtained 40 days after surgical repair revealed disappearance of ascites and enlargement of the thoracic duct, which had not been observed on preoperative lymphangiography. Notably, lymphatic reflux from the cisterna chili can occur without obstruction of the thoracic duct and may result in chylous ascites.

"Pigtail through snare" technique: an easy and fast way to retrieve a catheter fragment with inaccessible ends.

BACKGROUND: A catheter fragment with inaccessible ends can be retrieved using the well-known two-step method: making a free end with a pigtail catheter and seizing it with a snare catheter. Here we propose an easier and faster modification, named the "pigtail through snare" technique. CASE PRESENTATION: A 61-year-old female patient underwent removal of a central venous catheter fragment migrating to the right atrium. Both ends located in the right atrial appendage and left hepatic vein were inaccessible. Initially, a snare loop was opened in the inferior vena cava and a pigtail catheter was advanced through the snare loop to hook the catheter fragment. The free end was created by pulling the pigtail catheter, dragged automatically into the snare loop, grasped, and retrieved immediately. CONCLUSIONS: By passing the pigtail catheter through the snare loop in advance, the snaring maneuver becomes easy and fast in retrieving the catheter fragment with inaccessible ends.

Surgical Venous Drainage Disconnection from Cavernous Sinus Dural Arteriovenous Fistula and Ruptured Varix.

BACKGROUND: Cavernous sinus dural arteriovenous fistulas (CS dAVFs) occasionally behave aggressively (e.g., intracranial hemorrhage, venous infarction, seizures) depending on the drainage flow and presence of a collateral route of cortical or basal cerebral venous drainage. When a CS dAVF with aggressive behavior is encountered, a radical cure is required to avoid catastrophic deficits. However, conventional transvenous cavernous sinus (CS) embolization via the inferior petrosal sinus does not always achieve shunt obliteration. We herein report a case of surgical venous drainage disconnection in an 83-year-old woman with a CS dAVF. CASE DESCRIPTION: The patient presented with coma and anisocoria due to intracranial hemorrhage. Because of the patient's critical condition, we had no choice but to perform emergency decompressive craniectomy and hematoma evacuation without detailed preoperative hemodynamic information obtained by digital subtraction angiography. Postoperative digital subtraction angiography showed a CS dAVF with retrograde venous drainage of the deep middle cerebral vein (DMCV) and varix formation in the affected DMCV, causing hemorrhagic episodes. Five days after admission, the patient's neurologic state worsened because of rebleeding from the varix, which had increased in size. The percutaneous transvenous approach failed because of compartmentalization within the CS. Open surgery was performed; the deep vasculature was exposed by the transsylvian approach, and the arterialized DMCV was permanently clipped at its proximal segment with disconnection from the venous varix and fistulous point. Shunt obliteration was successfully achieved. CONCLUSIONS: Surgical venous drainage disconnection from the fistulous point may be an alternative radical therapy for CS dAVFs with aggressive behaviors.

Frequent vomiting attacks in a patient with Lhermitte-Duclos disease: a rare pathophysiology of cerebellar lesions?

Lhermitte-Duclos disease (LDD) is a neurological disease caused by a hamartomatous lesion in the cerebellum. Clinically, LDD is commonly associated with progressive space-occupying lesion effects in the posterior fossa, increasing intracranial pressure, occlusive hydrocephalus, and focal neurological deficits of adjacent structures. The authors report the case of a 10-year-old boy with LDD who had been suffering from vomiting attacks (VAs). These VAs had been brief in duration but extremely frequent, and they had been resistant to antiemetic drugs since the early postnatal period. Magnetic resonance imaging at 8 months of age revealed a right cerebellar lesion with very little space-occupying lesion effect, but the causal relationship with VAs was not evident at that point, because no clinical symptoms or signs other than vomiting were suggestive of increased intracranial pressure. The VAs were initially diagnosed as autonomic ataxia and had been treated with antiemetic drugs for approximately 10 years, but the patient's symptoms were not improved at all in frequency or duration. He developed convulsive seizures at 9 years of age and was referred to the authors' epilepsy center. The VAs were initially speculated to represent an aspect of seizures, but antiepileptic agents proved ineffective against this symptom despite remission of convulsive seizures. Video-electroencephalography monitoring did not show any evolving ictal patterns associated with the vomiting. Careful reevaluation of MRI studies revealed that the cerebellar lesion was fused with the cerebellum, middle and inferior cerebellar peduncles, and dorsolateral medulla oblongata with some distortion. FDG-PET identified hypermetabolism in the cerebellar lesion. After establishing the diagnosis of LDD, the authors performed subtotal resection of the lesion based on the likelihood of a causal relationship between the cerebellar lesion and the vomiting center of the medulla oblongata. Postoperatively and for 2 years, VAs have remained completely suppressed. The authors hypothesize that the pathophysiology of VAs in LDD includes a tumor-like space-occupying effect on the vomiting center of the medulla oblongata, and even partial resection of the lesion may prove effective.