Carotid cavernous dural arteriovenous fistula (DAVF) usually presents with conjunctival injection, proptosis, loss of visual acuity and ophthalmoplegia. There have been some carotid cavernous DAVF case reports presenting with isolated oculomotor, abducens and trochlear nerve palsy. We experienced a patient presenting with bilateral abducens nerve palsy and no other ocular signs who was diagnosed as carotid cavernous DAVF after conventional angiography. According to this case, carotid cavernous DAVF should be considered in the differential diagnosis of isolated bilateral abducens nerve palsy, in which case conventional angiography may be helpful in diagnosis.
Abducens Nerve/physiopathology* ; Arteriovenous Fistula/complications* ; Carotid Artery Diseases/complications* ; Case Report ; Cavernous Sinus*/radiography ; Cranial Nerve Diseases/physiopathology ; Cranial Nerve Diseases/etiology ; Dura Mater/blood supply* ; Female ; Human ; Middle Age ; Paralysis/physiopathology ; Paralysis/etiology*

OBJECTIVE: To heighten the awareness of the facial nerve tumors. METHOD: The clinical data of twenty-three patients complaining of facial paralysis who were diagnosed postoperatively as facial nerve tumors were analyzed. The hearing assessment of all patients was based on pure tone audiometry at the frequency of 0. 5, 1, 2, 4 kHz. Temporal bone high resolution CT scan and temporal bone MRI with gadolinium enhancement were conducted on all patients. Facial nerve function was assessed with the House-Brackmann (HB) grading system. Facial electroneurography (ENoG) was conducted on 20 patients to quantify the degree of nerve degeneration preoperatively. The pathological types of tumor were determined by postoperative pathological reports. RESULT: Nineteen out of 23 cases presented hearing loss (82.6%), 10 cases suffered from tinnitus (43.5%), otalgia (17.4%) affected 4 cases, 3 cases manifested otorrhea (13.0%), and 2 cases presented vertigo (8.7%). Geniculate ganglion was the most commonly involved site (20 cases, 87.0%), followed by tympanic segments (18 cases, 78.3%), pyramid segment (16 cases, 69.6%), mastoid segment (10 cases, 43.5%), labyrinthine segment (9 cases, 39.1%), internal auditory canal segment and parotid gland segment (5 cases, 21.7%, respectively). Twenty-one cases (91.3%) of schwannomas, 1 case (4.3%) of neurofibroma and 1 case (4.3%) of hemangiomas were identified with histopathology postoperatively. The tumors were all completely excised, and the facial nerve function could recovered to HB III at the best after facial nerve repairment. CONCLUSION Facial nerve tumor is a rare and often misdiagnosed disease which was commonly manifested as facial nerve paralysis. Temporal bone CT and MRI can help to clarify the diagnosis preoperatively. Pure tone audiometry and electroneurography also plays a some certain roles in the diagnosis of facial nerve tumors. The tumors should be completely resected and the surgical approaches were determined based on tumor size, facial nerve function and preoperative auditory function.
Cranial Nerve Neoplasms ; complications ; Facial Nerve ; pathology ; Facial Nerve Diseases ; complications ; Facial Paralysis ; etiology ; Hearing Loss ; Hemangioma ; Humans ; Neurilemmoma ; Temporal Bone

OBJECTIVE: To study the anatomic characteristics of recurrent laryngeal nerve during thyroid surgery. METHOD: A retrospective review of surgical data of 307 patients undertook thyroid surgery was conducted. RESULT: Total 342 recurrent laryngeal nerves were identified during the surgery(184 on the right side, left 158). 215 (62.9%) nerves were deep to the inferior thyroid artery, 106(31.0%)were superficial to the artery, 21(7.5%) were between the arterial branches. A nerve bifurcation was found in 203(59.4%). None of nerve bifurcation was found in 136(39.8%). 3(0.9%)were confirmed to hold non-recurrent laryngeal nerves during operations. No patient showed permanent laryngeal recurrent nerve paralysis postoperatively. CONCLUSION The careful dissection and protection of the recurrent laryngeal nerve was an effective method to prevent its injury during thyroid surgery.
Arteries ; Cranial Nerve Diseases ; etiology ; prevention & control ; Dissection ; Humans ; Postoperative Period ; Recurrent Laryngeal Nerve ; Retrospective Studies ; Thyroid Gland ; surgery ; Thyroidectomy ; Vocal Cord Paralysis

Adult ; Anti-Inflammatory Agents ; therapeutic use ; Atrioventricular Block ; diagnosis ; etiology ; pathology ; Cranial Nerve Diseases ; diagnosis ; etiology ; pathology ; Humans ; Male ; Prednisolone ; therapeutic use ; Sarcoidosis ; complications ; diagnosis ; pathology

OBJECTIVE: Aimed to analyse the clinical features of the patients with sphenoid sinus mucocele, achieve earlier diagnosis and more timely intervention and decrease the occurrence of misdiagnoses. METHOD: A retrospective study was first conducted in patients with sphenoid sinus mucoele treated in Xiangya hospital from Jan 2000 to Jan 2015. Then literature reports on this disease were collected and analyzed from China National Knowledge Infrastructure (CNKI) and Wan Fang database. RESULT: We collected 82 patients with sphenoid sinus mucocele treated in Xiangya hospital. There were 52 patients presented with headache, 31 patients presented with visual impairment, 10 patients presented with cranial nerve palsy, 2 patients presented with exophthalmos, 15 patients presented with nasal symptoms, and 5 patients with no obvious symptoms. There was no significant difference for symptoms distribution between male and female patients (P > 0.05). Among 45 patients with headache as first symptom and 10 patients with ethmoid sinus mucocele, there were 18 patients and 8 patients subsequently suffering from visual impairment, respectively. We also collected 161 patients in literature except for enrolling, the 82 patients treated in Xiangya hospital, and found that headache was the most common symptom, followed by visual impairment, in the two independent cohorts. CONCLUSION To the best of our knowledge, this is the study of maximum sample for sphenoid sinus mucocele in China. Headache and visual impairment are the most common symptoms for sphenoid sinus mucocele. Surgical treatment should be early performed when the desease accompanied with headache or ethmoid sinus mucocele, to avoid other complications such as visual impairment and even blindness.
China ; Cranial Nerve Diseases ; etiology ; Databases, Factual ; Diagnostic Errors ; Ethmoid Sinus ; Exophthalmos ; etiology ; Female ; Headache ; etiology ; Humans ; Male ; Mucocele ; complications ; diagnosis ; pathology ; Paranasal Sinus Diseases ; Retrospective Studies ; Sphenoid Sinus ; pathology ; Vision Disorders ; etiology

A 51-year-old woman with breast cancer presented with progressive diplopia. Neuro-ophthalmologic examination revealed right gaze palsy and peripheral facial nerve palsy. Brain magnetic resonance imaging (MRI) was normal. However, two months later a repeat brain MRI revealed an enhancing round nodular mass at the right facial colliculus of the lower pons, at the location of the abducens nucleus. Localized metastasis to the abducens nucleus can cause gaze palsy in a patient with breast cancer.
*Abducens Nerve Diseases ; Breast Neoplasms/*pathology ; Cranial Nerve Neoplasms/*complications/*secondary ; Facial Paralysis/complications ; Female ; Fixation, Ocular ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Ocular Motility Disorders/*etiology/physiopathology ; Pons/pathology

BACKGROUND AND OBJECTIVEDuring the resection of jugular foramen tumors via the basic far lateral approach, the jugular foramen tumor area as well as its adjacent structures, especially the intracranial part, can be better exposed, which avoids stripping of the petrous part of temporal bone and displacement of facial nerve, and protects the patient's hearing from damage. However, when applied in tumors developed from ventral to the brain stem and middle fossa, with extracranial tumors, this surgical approach seems to be inadequate and limited. This study was to explore the microsurgical technique and clinical value for treating giant dumbbell-shaped tumors at jugular foramen (JF) via a modified far lateral approach.

METHODSA retrospective analysis was performed in 16 patients with huge dumbbell-shaped tumors at JF which were removed through the modified far lateral approach (suboccipital transjugular-jugular tubercle-jugular process) between January 2001 and December 2008. The process of operation, and pre-and postoperative clinical data were included in the analysis.

RESULTSGross total tumor removal was achieved in 14 cases, subtotal removal in 1 case, and partial removal in 1 case. Follow-up examinations in most patients demonstrated that the patient with an obvious preoperative deficit had a good recovery. During the follow-up from three months to seven years, 10 (76.9%) cases with lower cranial nerve involvement showed obvious improvement of symptom after operation, 8 (80.0%) cases with facial palsy obtained various degrees of alleviation, and 7 (77.8%) cases with hearing impairment at different levels restored hearing. Two patients developed new lower cranial nerve palsies after operation, and underwent functional rehabilitation in the three-month follow-up.

CONCLUSIONSModified far lateral approach is helpful for removing the huge tumors at JF, especially for tumors extending to the petroclival region ventral part of pontomedullary junction. It has a higher rate of total resection, preoperative cranial nerve function impairment is expected to restore, and also has the advantage of protecting the facial nerve, labyrinth and vertebral artery structure from unnecessary damage.

Adult ; Cranial Nerve Diseases ; etiology ; Female ; Follow-Up Studies ; Glomus Jugulare Tumor ; complications ; diagnosis ; diagnostic imaging ; surgery ; Hearing Loss ; etiology ; Humans ; Magnetic Resonance Imaging ; Male ; Meningioma ; surgery ; Microsurgery ; methods ; Middle Aged ; Neurilemmoma ; surgery ; Radiography ; Recovery of Function ; Retrospective Studies

OBJECTIVEBlau syndrome (BS), an autosomal dominant inherited autoinflammatory disease, is caused by NOD2 mutations. This study aimed to analyze NOD2 gene of suspected BS patients to make definite diagnosis, find NOD2 mutation types and clinical features of Chinese BS cases, and find some clinical indications to identify BS by comparing BS and non-BS cases.

METHODEighteen suspected BS children (7 boys and 11 girls, age of first visit was from 1 y 8 m to 9 y 6 m) who visited Peking Union Medical College Hospital from 2006 to 2014 and their parents's DNA were extracted from 4 ml blood specimens. PCR was performed for exon 4 of NOD2 and PCR products were purified by 2% gel electrophoresis and sequenced directly. Role of novel missense mutations in pathogenicity was analyzed by SIFT and sequencing NOD 2 of fifty normal controls. Clinical data of BS children diagnosed by NOD2 analysis were summarized and compared with the data of non-BS group.

RESULT(1) Twelve of eighteen suspected BS children were diagnosed as BS by NOD2 analysis, and the remaining 6 were excluded. Seven missense mutations were detected, 4 were reported before: c.1000C>T, p. Arg 334Trp; c.1001G>A, p. Arg334Gln; c.1538T>C, p. Met513Thr; c.1759C>T, p. Arg587Cys. Three novel mutations were found: c. 1147 G>C, p.Glu383Gln; c.1471A>T, p. Met491Leu; c.2006A>G, p.His669Arg. (2) Chronic symmetric arthritis and multi-joints periarticular hydatoncus, which were painless with fluctuation, were found in all 12 BS children with NOD2 mutations. Skin rash, chronic symmetric arthritis, and recurrent uveitis were identified in 7 patients. Three patients had no skin rash, while 1 had no uveitis, 1 only had symmetric arthritis and multi-joints periarticular hydatoncus. Four children inherited the disease from father. (3) Compared with other 6 non-BS children, BS children had such different clinical characteristic (P < 0.05): All the BS cases had multiple periarticular hydatoncus, which always had no persistent fever, most had no elevated CRP, while non-BS group always had no hydatoncus, most had persistent fever, all had elevated CRP.

CONCLUSIONThe 12 BS children were diagnosed by NOD2 analysis; 7 missense mutations were detected, 3 were novel mutations, adding new findings to human NOD2 mutations. Although classic BS was characterized by skin rash, arthritis, and eye involvement, some presented with less than 3 of the classic features. Chronic symmetric arthritis and multi-joints periarticular hydatoncus were the most comment fetures. Comparing with non-BS group, all BS cases had multi hydatoncus surrounding multi-joints, always had no persistent fever, most had no elevated CRP. Those features may distinguish BS in clinical settings.

Arthritis ; etiology ; Asian Continental Ancestry Group ; genetics ; Base Sequence ; Case-Control Studies ; Child ; Child, Preschool ; Cranial Nerve Diseases ; complications ; genetics ; Exanthema ; etiology ; Exons ; genetics ; Female ; Humans ; Infant ; Male ; Mutation ; genetics ; Mutation, Missense ; Nod2 Signaling Adaptor Protein ; genetics ; Synovitis ; complications ; genetics ; Uveitis ; complications ; etiology ; genetics