Successful surgical treatment of oculomotor palsy due to schwannoma of the cavernous sinus in a 7-year-old girl: a case report.

Oculomotor nerve schwannoma in children not associated with neurofibromatosis is a rare disease, with 26 pediatric cases reported so far. There is no established treatment plan. A 7-year-old girl presented with oculomotor nerve palsy. Surgical reduction of the tumor combined with postoperative gamma knife surgery preserved the oculomotor nerve, improved oculomotor nerve function, and achieved tumor control during the observation period of 20 months. The combination of partial surgical resection and gamma knife surgery as a treatment strategy for oculomotor nerve schwannoma resulted in a good outcome.

Congenital fibrosis of the extraocular muscles: review of recent literature.

PURPOSE OF REVIEW: Congenital fibrosis of the extraocular muscles (CFEOM) is caused by abnormal development of the innervation of extraocular muscles. We update the recent literature regarding the clinical, anatomic, genetic, and molecular characteristics of CFEOM. Surgical considerations are addressed. RECENT FINDINGS: CFEOM is broken down into three main subtypes, CFEOM1, CFEOM2, and CFEOM3. Several recent reports of individuals, as well as family pedigrees, highlight the phenotypic heterogeneity of CFEOM. Intracranial and intraorbital radiologic findings have enhanced our understanding of the disease pathophysiology. Molecular genetics research has increased our understanding of the development of extraocular muscles and their innervation as well as pathophysiology of CFEOM. SUMMARY: Our understanding of the pathophysiology of CFEOM has increased with the recent contributions from neuroimaging, molecular genetics, and pedigree analysis. Surgical management of patients with CFEOM continues to be challenging.

[Isolated Unilateral Strabismus Sursoadductorius (Congenital Trochlear Palsy) with and without Hypotrophia of the Superior Oblique Muscle-Orthoptic Differences in Adulthood]. / Unilateraler dekompensierender Strabismus sursoadductorius mit und ohne Hypotrophie des M. obliquus superior ­ orthoptische Unterscheidungsmerkmale im Erwachsenenalter.

PURPOSE: The clinical feature of unilateral decompensating strabismus sursoadductorius (dSSA; often called congenital superior oblique palsy, CSOP) is not an etiologically uniform entity. Hypotrophy of the superior oblique muscle (HMOS) is a frequent and immediate cause of dSSA/CSOP. In this study, clinical characteristics of dSSA/CSOP with and without HMOS are compared. PATIENTS AND METHODS: Twenty-five consecutive patients (age 14 - 69 years; median 43 years) were included in this study, 14 with 3T MRI-proven HMOS (group 1) and 11 without HMOS (group 2). HMOS was defined as a reduction of the medio-lateral (ML) as well as based on ML and cranio-caudal (CC) diameter calculated area = (ML · CC/4) · π of the affected superior oblique muscle (SOM) < 80% in comparison to the contralateral SOM (measured on the single coronal image on which the muscle has its greatest extent). The two groups were compared in terms of head tilt, cyclo- and vertical deviation and the Bielschowsky head tilt test. Patients were classified according the Knapp's classification. RESULTS: Both the incidence of head tilt with 14/14 vs. 5/11 (χ2 = 0.003) and its degree: 11.1 ± 4.5° vs. 3.2 ± 4.1° (p < 0.001) was higher in group 1 than in group 2, as well as the Bielschowsky head tilt test: 9.3 ± 4.3° vs. 3.8 ± 4.9° (p = 0.008). The average amount of hypertropia was larger in group 1 than in group 2 during adduction: 16.7 ± 5.3° vs. 9.3 ± 3.4° (p < 0.001) as well as during adduction and downgaze of the affected eye: 14.6 ± 7.1 vs. 7.2 ± 3.7° (p = 0.03). In the sagittal plane, the increase of vertical deviation was larger in group 1 than in group 2: 2.8 ± 7.8 vs. - 2.4 ± 4.2 (p = 0.04); the excyclodeviation was larger in group 1 in all three planes (sagittal plane, adduction and abduction) in comparison to group 2: 1.3 ± 4.1 vs. - 2.9 ± 2.8 (p = 0.006), 2.4 ± 5.2 vs. - 2.2 ± 2.9 (p = 0.01), 0.5 ± 3.8 vs. - 2.7 ± 3.9 (p = 0.05). Knapp's class II was found in 6 of 15 patients in group 1 and only in this patient group (χ2 = 0.03). CONCLUSIONS: In both groups, the vertical deviation showed a great dispersion. In patients without HMOS (group 2), vertical deviation in adduction did not exceed 15°. Patients with HMOS (group 1) do not show the typical features of a later acquired trochlear palsy due to an early developed compensating innervation. A vertical deviation in adduction of more than 15°, increasing excyclodeviation towards downgaze in all three planes (sagittal plane, adduction and abduction) and Knapp's class II are relatively reliable predictors of a hypoplasia of the SOM. An internationally uniform term for this group of patients, such as superior oblique weakness or superior oblique hypotrophy, would be desirable.

Paradigms for single-patient multimodality treatment for cerebral aneurysms: single-center eleven-year experience.

Endovascular and surgical techniques are conventional options for treating intracranial aneurysms, but criteria for selecting an optimal approach for individual patients remain variable across practitioners and institutions. While endovascular and surgical approaches are generally used alone, both modalities combined in single patients can produce efficacious outcomes. The aim of this study was to evaluate outcomes of combined, concomitant endovascular and surgical modalities in the treatment of multiple and/or complex aneurysms in single patients. Indications, sequencing rationale, and categorization for multimodality treatments are reviewed. All intracranial aneurysms treated at our institution from 2004 to 2014 were reviewed. Single patients who had undergone concomitant endovascular and surgical treatments were eligible for participation in our study. Demographic data and clinical presentation parameters, including location, size, and morphological features of lesions, treatment sequencing, and outcomes were recorded. Our cohort consisted of 27 patients with 57 aneurysms who received concomitant endovascular and surgical treatment of their aneurysm(s). One patient arrived to us after he had an aneurysm clipped at an outside institution and then required treatment for a contralateral ruptured aneurysm. 66.7% of patients were diagnosed with subarachnoid hemorrhage. These were subdivided according to therapeutic approach: clipping and coiling (CL+CO), clipping and stenting (CL+ST), bypass and endovascular parent vessel occlusion (PVO) (BY+PVO), attempted clipping then stenting, and bypass followed by stenting. Glasgow Outcome Scale was as follows: CL-CO-Multiple, 4.17 (five in unruptured patients, 3.75 in ruptured); CO-CL-Multiple, five (all patients had a ruptured aneurysm); CL-CO-Single, three (all patients had a ruptured aneurysm); CO-CL-Single, five (all patients had a ruptured aneurysm). No patients suffered a new neurological deficit as a result of treatment. A total of two mortalities were documented. Concomitant, mutimodality endovascular and surgical therapy may offer a safe and potentially more effective paradigm than single modality approaches for the management of multiple, complex, or "failed" aneurysm treatments in selected patients.

Surgical Treatment of Posttraumatic Ophthalmoplegia Through the Reconstruction of the Lateral Orbital Wall.

Orbital fractures are injuries frequently related to traumas of the midface. These fractures can be associated with ocular lesions, ranging from small abrasions on the cornea to serious complications such as hyphema and ocular globe rupture. Diplopia and ophthalmoplegia are common findings in orbital fractures. They can be caused by mechanical factors as bone fragments or muscle imprisonment. The aim of this study was to report a case of a 40-year patient, male showing diplopia and ophthalmoplegia due to the orbital fracture. The patient was treated by general anesthesia. It was performed a supra orbital approach and the fragments were removed. A titanium mesh to restore the orbital anatomy was installed. After 40 days of follow-up, the patient has no aesthetic or functional complaints. In orbital traumas, the ophthalmological evaluation should be performed carefully aiming to avoid ocular sequelaes. In surgical patients, the surgery should be done as early as the clinical conditions permit, to restore the ocular function.

Frontotemporal epi- and subdural approach to a cavernous sinus tumor for sphenobasal vein preservation--technical case report.

In cavernous sinus (CS) surgery, venous complication may occur in some types of venous drainage. The sphenobasal vein (SBV) drains from the superficial middle cerebral vein (SMCV) to the pterygoid venous plexus at the temporal skull base. A frontotemporal epi- and interdural approach (Dolenc approach), which is one of the CS approaches, may damage the SBV's route. We report a case of intracavernous trigeminal schwannoma that contained the SBV and discuss our modified surgical procedure that combined epi- and subdural approaches to preserve the SBV. A 64-year-old man complained of right progressive oculomotor palsy and was referred to our hospital for surgery. MR images revealed a hemorrhagic tumor in the right CS. Three-dimensional venography revealed that the SMCV drained into the pterygoid venous plexus via the SBV. After identifying the first branch of the trigeminal nerve epidurally, we incised the dura linearly along the sylvian fissure and entered the subdural space to visualize the SBV. The incision was continued to the meningeal dura of the lateral wall of the CS along the superior margin of the first branch of the trigeminal nerve, and the Parkinson's triangle was opened from the subdural side. The tumor was grossly totally removed, and the SBV was preserved. In conclusion, a frontotemporal epi- and subdural approach to the intracavernous trigeminal schwannoma can effectively preserve the SBV.

[The effect of superior oblique tucking on the Bielschowsky head tilt test].

OBJECTIVE: To investigate the effect of superior oblique tucking on the Bielschowsky head tilt test. METHODS: A retrospective analysis of 22 patients with congenital unilateral superior oblique palsy, who underwent tucking of the superior oblique tendon in Tianjin Eye Hospital, depending on vertical deviation in the primary position, ocular motility, objective cyclotorsion and abnormal head posture. Bielschowsky head tilt test was evaluated quantitatively by the prism cover test. Α positive Bielschowsky head tilt test was defined as one in which the vertical deviation when the head tilt to the side of the paresis was at least 5. 0(△) greater than that on tilt to the uninvolved side. The follow-up period ranged from 3 to 12 months (mean 4.6 months). Kolmogorov-Smirnov (K-S), Rank sum test and Spearman correlation analysis statistical methods were used in this study. RESULTS: All patients had vertical deviation and significant abnormal head posture before operation. After the procedure of superior oblique tucking,vertical deviation and symptom of unacceptable abnormal head posture were ameliorated or disappeared. OBJECTIVE torsion was 15.62°±7.36° before the surgery, 9.91°±10.09°1d after the surgery and 11.25°±9.17°at the last follow-up visit, respectively (P<0.05). We found that there were positive correlations between the objective cyclotorsion and the vertical deviation difference value between the paralyzed side and uninvolved side at 1 d after the surgery and last follow-up visit(P<0.05). The vertical deviation of the paresised eye when the head tilted to the side of paresis side and uninvolved side difference were 5.00(△)-17.00(△), average 8.68(△)±3.23(△), 1.00(△)-8.00(△),average 3.36(△)±2.01(△) and 0.00(△)-14.00(△),average 3.77(△)±3.01(△), preoperatively, 1d after the surgery and at the last follow-up visit respectively. Using Rank sum test, the pre-and postoperative difference was statistically significant (Z=-4.64, P<0.01). A positive Bielschowsky head tilt test was found in all cases preoperatively. The results of Bielschowsky tilt test was still positive in 5 cases and negative in 17 cases (77.3%). CONCLUSIONS: In most cases with unilateral superior oblique paresis, the results of Bielschowsky tilt test became negative after superior oblique tucking. The long-standing results is worthy of observation. (Chin J Ophthalmol, 2016, 52: 589-595).

Paralytic lagophthalmos syndrome in the plastic and maxillofacial surgeon practice. / Sindrom paraliticheskogo lagoftal'ma v praktike plasticheskogo i chelyustno-litsevogo khirurga.

OBJECTIVE: to identify and give an objective assessment of all changes in the periorbital region in paralysis orbicularis oculi muscle. METHODS: With physical, laboratory and instrumental methods of research, we analyzed the survey of 33 patients with paralytic lagophthalmos. This survey has allowed us to identify the symptoms of periorbital changes characteristic of paralysis orbicularis oculi involved in lagophthalmos development. RESULTS: After analyzing the results of a survey of 33 patients with paralytic lagophthalmos, we identified 15 symptoms of changes in the periorbital region. CONCLUSION: In our opinion, lagophthalmos should be considered as a syndrome, including certain symptom in a particular case. For adequate closure of the optic fissure and obtaining a good aesthetic result is necessary to simultaneously remove more of the symptoms.

Successful Sequential Liver and Isolated Intestine Transplantation for Mitochondrial Neurogastrointestinal Encephalopathy Syndrome: A Case Report.

BACKGROUND Mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE) is an autosomal recessive disease caused by thymidine phosphorylase deficiency leading to progressive gastrointestinal dysmotility, cachexia, ptosis, ophthalmoparesis, peripheral neuropathy and leukoencephalopathy. Although liver transplantation corrects thymidine phosphorylase deficiency, intestinal deficiency of the enzyme persists. Retrospective chart review was carried out to obtain clinical, biochemical, and pathological details. CASE REPORT We present a case of liver and subsequent intestine transplant in a 28-year-old man with MNGIE syndrome with gastrointestinal dysmotility, inability to walk, leukoencephalopathy, ptosis, cachexia, and elevated serum thymidine. To halt progression of neurologic deficit, he first received a left-lobe partial liver transplantation. Although his motor deficit improved, gastrointestinal dysmotility persisted, requiring total parenteral nutrition. After exhaustive intestinal rehabilitation, he was listed for intestine transplantation. Two-and-half years after liver transplantation, he received an intestine transplant. At 4 years after LT and 20 months after the intestine transplant, he remains off parenteral nutrition and is slowly gaining weight. CONCLUSIONS This is the first reported case of mitochondrial neurogastrointestinal encephalomyopathy to undergo successful sequential liver and intestine transplantation.

Surgery for longer duration supranuclear ophthalmoplegia secondary to brain stem cavernoma: A case report and literature review.

BACKGROUND: Previous reports revealed that patients with acquired paralytic strabismus caused by central nervous system diseases are primarily affected by the etiology and treatment of the condition. Strabismus correction for these acquired paralytic strabismus should be performed as soon as the primary disease has been stabilized for 6 months in order to archive a favorable surgical outcome. CASE: We followed an infrequent case of longer-lasting supranuclear ophthalmoplegia secondary to brain stem cavernoma. OBSERVATION: A 25-year-old Chinese Han female developed aberrant head posture and ipsilateral conjugate gaze palsies 8 years after the first brainstem hemorrhage caused by pontine cavernoma. The patient was diagnosed with supranuclear ophthalmic palsy and brain stem cavernoma after surgery. A resection-recession procedure along with a rectus muscle transposition was performed. The patient's abnormal head position disappeared, with a normal primary position. CONCLUSION: Resection-recession procedures combined with rectus muscle transposition works very well for longer duration large-angle strabismus caused by brain stem cavernoma.

The proposal of subgroups for grade V on World Federation of Neurologic Surgeons grading for subarachnoid hemorrhage.

AIM: Some of cases suffering from subarachnoid hemorrhages (SAHs) in grade V on World Federation of Neurologic Surgeons (WFNS) grading can gain a good prognosis. The outcome of patients of SAH in grade V on WFNS grading in their institute was here investigated. METHODS: Between April 2007 and July 2012, consecutive 37 patients had SAH diagnosed on CT scan and were classified in grade V on WFNS grading in Kosei General Hospital. There were seventeen male and twenty female patients. We were assigned to patients with spontaneous respiration and without oculomotor palsy (N group, N.=11), and patients with oculomotor palsy (O group, N.=26). Patients were evaluated by mRS. RESULTS: The prognosis in N group was significantly better than in O group (P<0.001). CONCLUSION: Surgical treatments should be considered for SAH patients without oculomotor palsy. It is necessary to make subgroups in grade V on WFNS grading in order to decide operative indication and evaluate the treatment results of SAH in grade V.

[A case of cavernous sinus aspergillosis].

We reported a case of cavernous sinus aspergillosis. A 62-year-old man complained of trigeminal neuralgia in the right V1 region. Neurological examination on admission showed ptosis, loss of light reflex and ophthalmoplegia externa in the right side. MRI enhanced with gadolinium demonstrated sphenoid sinusitis and mass lesion in the right cavernous sinus. MRA revealed right internal carotid artery occlusion. An open biopsy using the extradural temporopolar approach was performed. Pus discharge was observed from the cavernous sinus and histological examination showed hypha of Aspergillus. With early voriconazole treatment, the patient had improvement in headache, ptosis and ophthalmoplegia externa. Cavernous sinus aspergillosis is often found after sphenoiditis. It results in invasion to an internal carotid artery and worsens the patient's prognosis by cerebral infarction, so early diagnosis and treatment are important. We should consider aspergillosis as one of the differential diagnoses of a mass in the cavernous sinus. The epidural approach to this lesion was available to obviate aspergillus dissemination into the medullary cavity.

[Partial rectus muscle transposition for strabismus with lateral rectus muscles paralysis].

OBJECTIVE: To investigate surgical approach for paralytic strabismus with lateral rectus muscles paralysis and evaluate its clinical effects. METHODS: Design retrospective, observational case series. 28 patients (31 eyes) who underwent augmented partial rectus muscle transposition (APRMT) in the treatment of paralytic strabismus with lateral rectus muscles paralysis from May 2009 to May 2011 at Eye & ENT Hospital of Fudan University were included in this study. The following data were analyzed: (1) The procedure that was performed, (2) The angle of deviation of primary position, (3) The presence of compensatory head posture, (4) The presence of diploma in the primary position, (5) The binocular single visual fields, (6) The motility of the affected eye. RESULTS: According to the results of rectus muscle forced traction test and examination before and after operation, among 31 eyes in 28 patients, 8 eyes were treated with APRMT, 10 eyes were treated with recession of antagonistic muscle of paralytic rectus muscle combined with APRMT, 7 eyes were treated with APRMT and recession of yoke muscle, 6 eyes were treated with APRMT and recession of yoke muscle combined with recession of antagonistic muscle. 17 among 28 patients were orthotopic in the primary position while others were in mild under correction with deviation no more than 10 prism degrees during 5 months (range: 3-8 months) follow-up in average. The diploma and abnormal head posture were eliminated in 26 patients except one patient who was appeared with 10 prism degrees under correction postoperatively and was resolved by wearing prisms thereafter. Binocular single visual fields were extended from 27.0° ± 6.4 ° preoperatively to 40.5° ± 4.8 ° postoperatively (t = -34.692, P < 0.001). Ocular movement was improved from -4.9 ± 0.4 to -2.9 ± 0.9 postoperatively (t = -13.418, P < 0.001). CONCLUSIONS: APRMT can safely and effectively correct the primary position in paralytic strabismus with lateral rectus muscles paralysis, eliminate the presence of diploma in primary position and abnormal head posture, and improve the ability of the ocular movement, furthermore, significantly expand the scope of binocular single visual fields. The results also demonstrate that rectus muscle forced traction test is a key measurement to determine the surgical procedure.

Congenital Fibrosis of Extraocular Muscles: A Systematic Review and Meta-Analysis.

PURPOSE: To quantify typical disease characteristics for patients with congenital fibrosis of the extraocular muscles (CFEOM) including pre-and post-surgical ocular alignment and head positioning as well as average improvement with surgical intervention. PATIENTS AND METHODS: This investigation is a systematic review and meta-analysis. All studies detailing surgical intervention in cohorts of individuals with CFEOM were included. Ocular alignment and head positioning before and after surgery was recorded. Meta-analyses were performed to quantify these findings along with other patient characteristics across all included studies. RESULTS: The estimated average improvement in vertical alignment for patients with unilateral and bilateral CFEOM was 34.8 pd (prism diopters) and 21.27 pd, respectively. For horizontal alignment, patients with unilateral and bilateral CFEOM improved an average of 25.39 pd and 10.99 pd after surgery, respectively. There was no significant difference between the two most common surgeries used to address hypotropia and blepharoptosis in this patient population. The estimated proportion of patients with post-surgical head alignment within 5-9° was 60.64% and was 28.25% for patients with<5° of alignment. CONCLUSION: This study was able to illuminate many characteristic features of patients with CFEOM. Surgical intervention for the ocular alignment and head positioning of these patients demonstrates promising utility.

[Children orbital floor fracture: retrospective study, about 34 cases]. / Fracture du plancher de l'orbite chez l'enfant, étude rétrospective à propos de 34 cas.

INTRODUCTION: Orbital fractures represent 30 % of children facial fractures. Nausea and vomiting are more predictive of entrapment than local trauma stigmatisms. Entrapment and diplopia are more frequent in adults. Delay for surgery is unclear in literature varying from 6 hours to days. The aim of this study is to summarise the aspects of orbital floor fractures in children with regard to clinical and radiological presentation, management, and outcomes. PATIENTS AND METHODS: We conducted a retrospective study including 34 children presenting isolated orbital floor fracture. Clinical, radiological, ophthalmological, surgical data and outcomes were analyzed. RESULTS: Mean age was 9.4 years. In 15% of cases, no local stigmatism of trauma was present. Entrapment fracture was the most frequent, with 81% of fat or muscles entrapment. In all, 27% of the patient had residual diplopia. Residual diplopia developed after trap-door fracture with muscle entrapment and a more than 24 hours delay for surgery. CONCLUSION: Trap-door fracture is frequent in childhood population. Clinical diagnosis can be difficult. However, surgical treatment should be considered before 24 hours to avoid complication as residual diplopia.