Odontogenic Lemierre's syndrome with septic superior ophthalmic vein and cavernous sinus thrombophlebitis complicated by blindness and ophthalmoplegia.

Lemierre's syndrome is a triad consisting of oropharyngeal infection, internal jugular vein thrombophlebitis, and systemic embolisation typically involving lung and brain. Orbital involvement in this life-threatening condition is rare but potentially blinding and may be an indicator of intracranial involvement. We describe a case of odontogenic Lemierre's syndrome complicated by extensive orbital and intracranial septic venous thrombosis, with optic and cranial neuropathy resulting in monocular blindness and ophthalmoplegia. A multidisciplinary approach with abscess drainage, antibiotic and antithrombotic therapy, and close radiological monitoring was critical for preserving contralateral vision and neurological function.

Orbital apex syndrome secondary to an orbital sarcoma in a dog.

A 12-year-old castrated male cocker spaniel dog was presented with a 4-week history of left episcleral injection and pawing at the face. Clinical examination findings included left internal and external ophthalmoplegia, left dorsal strabismus, pain opening the mouth, and intermittent amaurosis. Imaging studies revealed a left orbital apex mass with adjacent sphenoid bone lysis and extension into the cranial cavity. A left exenteration was performed and histopathology confirmed an orbital soft tissue sarcoma. Key clinical message: This report describes an orbital tumor causing orbital apex syndrome. This condition should be differentiated from cavernous sinus syndrome as the latter does not course with optic neuropathy.

Syndrome de l'apex orbitaire secondaire à un sarcome orbitaire chez un chien. Un épagneul mâle castré âgé de 12 ans fut présenté avec une histoire d'injection épisclérale gauche et frottage de la face d'une durée de 4 semaines. Les trouvailles de l'examen clinique incluaient une ophtalmoplégie interne et externe à gauche, un strabisme dorsal à gauche, de la douleur lors de l'ouverture de la gueule et une amaurose intermittente. Les examens par imagerie ont révélé une masse dans l'apex orbitaire gauche avec lyse de l'os sphénoïde adjacent et extension dans la cavité crânienne. Une exentération gauche fut effectuée et l'histopathologie confirma un sarcome des tissus mous orbitaires.Message clinique clé:Ce rapport décrit une tumeur orbitaire causant un syndrome de l'apex orbitaire. Cette condition devrait être distinguée du syndrome du sinus caverneux étant donné que ce dernier n'évolue pas avec une neuropathie optique.(Traduit par Dr Serge Messier).

Orbital AL amyloid.

Amyloidosis and lymphoma localized to the ocular adnexa are rare, and their presentation may resemble more common inflammatory conditions such as autoimmune disease or infection, which can protract diagnostic evaluation and delay eventual therapy. In a patient with recalcitrant facial and tooth pain and ophthalmoplegia, evaluation should include careful histopathologic analysis of biopsy specimens. We report a case of orbital AL amyloidosis associated with localized lymphoma that presented with intractable dental pain and progressed to bilateral complete ophthalmoplegia.

Malposition of Teeth and Jaws in Patients with Congenital Superior Oblique Palsy.

BACKGROUND: Patients with congenital superior oblique palsy tend to adopt a head tilt to the contralateral side to maintain binocular single vision. It has long been recognised that facial asymmetries may be caused by a head tilt. The aim of this study was to describe the effect of habitual head tilt due to congenital superior oblique palsy on dental occlusion. PATIENTS AND METHODS: The study was designed as a descriptive cohort study. Ten patients with congenital superior oblique palsy (3 female, 7 male; mean age 51.7 (y) ± 15.8 SD, ranging from 19 to 69 (y)) underwent orthodontic examination. Orthodontic findings and values for vertical, torsional and horizontal deviation measured with the Harms tangent screen and stereopsis using a random dot test were compared. RESULTS: Three orthodontic parameters were found to correlate significantly or at least as trend with orthoptic parameters. Midline deviation of the upper jaw to the face (rho = 0.623; p = 0.054) and anterior positioning of upper first molar in the sagittal plane (rho = 0.594; p = 0.07) correlate with the vertical deviation; overbite correlates with horizontal deviation measured in the primary position (rho = 0.768; p = 0.016). CONCLUSIONS: In this small study, three orthodontic parameters correlated with orthoptic findings in patients with congenital superior oblique palsy. Further studies are needed to establish whether congenital superior oblique palsy is more frequent in patients exhibiting abnormal values of these orthodontic parameters.

Advanced cocaine-related necrotising sinusitis presenting with restrictive ophthalmolplegia.

We report a case of bilateral infero-medial orbital wall destruction, associated with loss of sinonasal architecture. The patient presented with intermittent horizontal diplopia following an acute on chronic infective sinusitis. Eight months previously the patient had developed a midline hard palate fistula for which a palatine prosthesis had been fitted. The broad differential diagnosis is discussed, though in this patient chronic cocaine abuse was identified as the underlying aetiology. Eye movement restriction worsened progressively with bilateral inflammation around the medial and inferior rectus muscles. Attempts to resolve the recurring cycle of sinus infection and inflammation by palatal fistula closure failed despite augmented techniques mobilising flaps from both nasal and palatal sides.

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.

Oculomasticatory rhythmic movements, insomnia and stroke-like episodes in a patient with POLG mutation.

The POLG mutation, a leading cause of mitochondrial diseases, exhibits a wide-ranging age of onset and a complex clinical presentation. We encountered an atypical clinical profile in an elderly man with a POLG mutation, characterised by a stroke-like episode, chronic insomnia and transient oculomasticatory rhythmic movement. History revealed chronic constipation since his 50s and progressive bilateral ophthalmoplegia since his early 60s. Subsequently, he had experienced acute encephalopathy and later developed chronic insomnia. The present neurological examination showed bilateral complete ophthalmoplegia, ptosis, and rhythmic ocular and jaw movements. Imaging indicated findings suggestive of a stroke-like episode and eventual genetic analysis revealed a homozygous missense mutation in the POLG gene. This case expands the clinical spectrum of POLG mutations in individuals over 60 years, showcasing the rare combination of a stroke-like episode, chronic insomnia and oculomasticatory rhythmic movement.

Severe visual loss and orbital infarction following periorbital aesthetic poly-(L)-lactic acid (PLLA) injection.

Visual loss following esthetic treatment of the face is a rare but devastating iatrogenic complication. The authors report a case of a 43-year-old man with blindness and ophthalmoplegia of the left eye following a treatment of the left periorbital region with the subcutaneous filler poly-(L)-lactic acid. The patient's symptoms began immediately following one of the tunneled injections. On presentation, the patient had clear signs of ocular and orbital ischemia. Angiography and further history suggested an embolic orbital infarction as the mechanism of injury. The increased usage of subcutaneous fillers for facial rejuvenation had introduced a small but proven risk of embolization of these viscous materials to the eye and orbit.

A 7-year old female with arthrogryposis multiplex congenita, Duane retraction syndrome, and Marcus Gunn phenomenon due to a ZC4H2 gene mutation: a clinical presentation of the Wieacker-Wolff syndrome.

Background: Duane retraction syndrome and arthrogryposis multiplex congenita have an incidence of approximately 1:1500-1:3000 live births. However, the association of these two entities with a Marcus-Gunn might be a rare and, until now, under-recognized clinical presentation of the Wieacker-Wolff Syndrome.Patient and methods: We report a 7-year-old female with dysmorphic features, global developmental delay, arthrogryposis multiplex congenita (AMC), Duane retraction syndrome (DRS), and unilateral Marcus Gunn jaw winking.Results: Whole Exome Sequencing showed a de novo premature stop codon in ZC4H2. Extensive genetic and metabolic work was negative otherwise and Brain MRI showed delayed non-specific myelination abnormalities. She continues to have significant delays but does not have regression, seizures or other neurological complications. She has required a multidisciplinary approach for the management of her multiple contractures.Conclusion: This case confirms ZC4H2 as a cause of syndromic DRS and extends the ZC4H2 phenotype to include Marcus Gunn jaw winking.

An unusual case of total ophthalmoplegia.

An eight-year-old male child presented with drooping of the left eyelid with a history of penetrating injury of hard palate by an iron spoon seven days ago, which had already been removed by the neurosurgeon as the computed tomography scan revealed a spoon in the left posterior ethmoid and sphenoid bone penetrating into the middle cranial fossa. On examination, visual acuity was 20/20 in each eye and left eye showed total ophthalmoplegia. Oral cavity revealed a hole in the left lateral part of the hard palate. We managed the case with tapering dose of systemic prednisolone. The total ophthalmoplegia was markedly improved in one month. Cases of foreign bodies in the orbit with intracranial extension are not unusual, but the path this foreign body traveled through the hard palate without affecting the optic nerve, internal carotid artery or cavernous sinus makes an interesting variation.

A lot of clot.

A 37-year-old man presented with fever and a red, painful right eye. He had proptosis, conjunctival chemosis, and ophthalmoplegia OD. The patient had extremely poor dentition and had self decompressed a dental abscess prior to admission. Magnetic resonance imaging of the brain and orbital revealed extraocular muscle engorgement and a dilated superior ophthalmic vein OD. Orbital echography revealed a lack of flow in the right superior ophthalmic vein. An extensive hematologic evaluation for infection and inflammation was negative. A chest radiograph showed a lung abscess for which he received intravenous antibiotics. Over time, the periorbital erythema, ophthalmoplegia, proptosis, and pain resolved. Repeat MRI showed resolution of the orbital findings and repeat chest x-ray showed resolution of the left upper lobe abscess.

Lacrimal-auricular-dental-digital (LADD) syndrome with diffuse ophthalmoplegia--a new finding.

Lacrimal-auricular-dental-digital (LADD) syndrome comprises multiple anomalies. It can be inherited as autosomal dominant with variable expressivity or can be sporadic in nature. The clinical features of LADD syndrome include variably, lacrimal system hypoplasia, ear anomalies (with or without hearing impairment), salivary system hypoplasia, epiblepharon, dry eyes, corneal limbal stem cells deficiency, hypodontia, microdontia, xerostomia, and clinodactyly. We would like to report a unique case series of LADD syndrome patients presenting with diffuse ophthalmoplegia and facial muscle dysfunction, which may be a distinct subset of LADD syndrome or a new syndrome itself. We believe this to be the first such report. We suggest careful examination of ocular movements in all newly diagnosed LADD syndrome patients.

Unilateral congenital fibrosis of the extraocular muscles with lid retraction: surgical treatment with a silicon plate on the orbital floor.

Fibrosis of the extraocular muscles can be an acquired or congenital disorder (CFEOM). The congenital disorder(1) is a complex strabismus with congenital restrictive ophthalmoplegia with or without ptosis. The surgery is challenging because the eye muscles are replaced by fibrous tissue or fibrous bands and in most cases the results are not satisfactory. We present the first case report of unilateral CFEOM with palpebral adherence and hypotropia, which was managed with our technique of a silicon plate implant on the orbital floor. The purpose of the implantation of the silicon plate in the orbital floor is to improve the hypotropia caused by CFEOM.

Orbital apex syndrome secondary to mucormycosis after a tooth extraction in an immunocompetent patient.

Mucormycosis is rare in immunocompetent patients. We describe an unusual case of orbital apex syndrome secondary to mucormycosis that occurred in an immunocompetent 64-year-old man following multiple tooth extractions. We found no frank involvement of the maxillary sinus, which is the usual pathway of spread for this fungal infection. Therefore, we suggest two possible alternate pathways from the oral mucosa to the orbital apex: one via the anterosuperior and posterosuperior alveolar vessels and one via the infraorbital branch of the maxillary artery.

Unilateral mydriasis without ophthalmoplegia--a sign of neurovascular compression? Case report.

OBJECTIVE: We aimed to demonstrate the use of neuroimaging studies in vascular compression of the oculomotor nerve. CLINICAL PRESENTATION: A 24-year-old woman was noted by her dentist to have anisocoria, with the left pupil being larger than the right. After detailed ophthalmologic and neurological examination, we proceeded to perform neuroimaging. T2-weighted images (2 mm) and constructive interference in steady-state (0.3 mm) images were utilized. INTERVENTION: Magnetic resonance imaging showed that duplicated left superior cerebellar artery, a prominent posterior communicating artery, and a posterior cerebral artery combined to compress the superomedial portion of the left oculomotor nerve. CONCLUSION: With continued improvements in neuroimaging, we think that more cases of isolated cranial neuropathies previously labeled as "idiopathic" will be shown to result from vascular compression.

[Botulism--observations on its course with emphasis on ophthalmologic symptoms]. / Botulismus--Verlaufsbeobachtungen mit Schwerpunkt auf der ophthalmologischen Symptomatik.

The natural history of a moderate intoxication with botulin (probably type B) in six patients is presented and discussed. All patients complained of a persistent and almost complete failure of accommodation, and marked dryness of the mouth. Reduced lacrimation was also noticeable. A disturbance of efferent pupillary reaction was noted only in four patients, and resolved relatively early. On testing with highly diluted pilocarpine solution, a denervation hypersensitivity reaction of the sphincter pupillae was seen. An optic nerve lesion could not be demonstrated in any of the cases. Manifest involvement of the striated musculature, such as a bilateral lateral rectus palsy and ptosis, was found in only one patient. In four patients the presenting symptom was gastroenteritis. Other systemic symptoms were dysphagia, persistent constipation, problems with micturition, general malaise and postural symptoms. All of the patients made a full recovery at the latest after 10 weeks.

Charcot-Marie-Tooth disease mimicking ocular myasthenia gravis.

The purpose of this report is to alert the ophthalmologist and neurologist to the fact that intermittent ocular symptoms may herald the onset of chronic progressive external ophthalmoplegia and therefore resemble the weakness produced by myasthenia gravis. In addition, chronic progressive external ophthalmoplegia may complicate the course of Charcot-Marie-Tooth disease.

Congenital fibrosis syndrome associated with central nervous system abnormalities.

BACKGROUND: Congenital fibrosis of extraocular muscles (CFEOM) is a complex strabismus syndrome that typically occurs in isolation and results from dysfunction of all or part of cranial nerves III (CNIII) and IV (CNIV) and/or the muscles that these nerves innervate. Only a few patients with CFEOM and additional central nervous system malformations have been reported. We describe four additional patients with CFEOM associated with central nervous system (CNS) abnormalities. METHODS: Four patients who presented with congenital restriction of eye movements in association with neurological abnormalities underwent complete ophthalmological examination including electroretinography (ERG) and eye movement recordings. Neurological examinations, neuroradiological studies, muscle histology, chromosomal and genetic linkage analysis were performed. RESULTS: Clinical examination and forced duction testing confirmed that all four patients met criteria for CFEOM; all had congenital restrictive ophthalmoplegia primarily affecting extraocular muscles innervated by the oculomotor nerve. Two brothers had CFEOM and Marcus Gunn jaw winking. In each of the four cases, CFEOM occurred in association with one or several neuroradiological findings, including agenesis of the corpus callosum, colpocephaly, hypoplasia of the cerebellar vermis, expansion of the ventricular system, pachygyria, encephalocele and/or hydrancephaly. CONCLUSIONS: We present four cases of CFEOM in association with CNS malformations that confirm that CFEOM can be part of a more complex neurological dysfunction and provide further support to a neurogenic aetiology for this disorder. We also describe for the first time the coexistence of CFEOM and Marcus Gunn jaw winking in two siblings. This suggests a genetic mechanism. Aberrant innervation supports primary developmental abnormality of cranial nerves in CFEOM.