Intraorbital surgery for trigeminal neuralgia

PURPOSE: First-division trigeminal neuralgia, or tic douloureux refractory to medications, presents problems to the surgeon because of the desirability of preserving corneal sensation. A new operation is described that may provide longer duration of pain relief than conventional supraorbital neurectomy, with preservation of the corneal reflex. METHODS: Four patients underwent resection of the supratrochlear and supraorbital nerves within the orbit accessed from an upper eyelid skin crease incision. RESULTS: Three patients with typical idiopathic trigeminal neuralgia involving branches of the frontal nerve are without pain 22 to 25 months after surgery. The final patient with atypical pain had no improvement after the procedure. Frontal nerve distribution anesthesia is present in all patients. Postoperative ptosis resolved in all patients within 4 months of surgery. CONCLUSIONS: This procedure should be added to the treatment options for patients with first-division trigeminal neuralgia. By avoiding injury to the trigeminal root and ganglion, this surgery carries no risk of facial motor dysfunction, dysthesia, and/or anesthesia in the other trigeminal branches including corneal anesthesia.     

Congenital corneal anesthesia: a series of four case reports

PURPOSE: Discussion of four cases of congenital corneal anesthesia with varied systemic associations and modes of presentation. Classification and systemic associations of congenital corneal anesthesia along with the importance of multi-specialty evaluation of such patients. METHODS: Clinical examination of all four cases and corneal sensation assessment with Cochet-Bonnet esthesiometer. Thorough systemic examination and investigations including serum bio-chemical analysis, roentgenography, ultrasonography, nerve conduction studies, and CT scan of the patients to identify systemic associations. RESULTS: All four patients proved to have bilateral congenital corneal anesthesia with Cochet-Bonnet esthesiometry confirming corneal anesthesia. Systemic examination revealed associated congenital mesenchymal anomalies in the first case. The second case had generalized reduction in pain sensitivity confirmed by nerve conduction studies. The third case was an isolated bilateral congenital corneal anesthesia, while the fourth was a case of congenital trigeminal nerve anesthesia involving all three divisions of both trigeminal nerves. Other causes of corneal anesthesia and self-inflicted corneal injuries were ruled out by clinical multi-specialty evaluation and appropriate investigations. Correct diagnosis and symptomatic treatment aided speedy relief of symptoms of all the patients. CONCLUSION: Congenital corneal anesthesia is a rare condition and is often a diagnostic dilemma. Although more common conditions that cause corneal anesthesia can mislead the clinician, the congenital variant should be kept in mind in any case of intractable corneal ulceration in children. Early diagnosis, symptomatic treatment and measures to prevent further corneal damage will immensely help in retaining useful vision for the affected children.     

三叉神经痛患者眼表表现及角膜神经的共焦显微镜观察

背景三叉神经眼支是角膜的主要感觉神经和营养神经,三叉神经痛是否会影响患侧眼角膜的功能和形态尚未见报道。共焦显微镜是角膜无创性活体检测的主要手段。目的观察和分析三叉神经痛患者角膜的共焦显微镜下表现及角膜神经在共焦显微镜下形态和密度的改变。方法收集就诊于河南省人民医院疼痛科的33例三叉神经痛患者,对所有受检眼应用角膜知觉敏感度测量计测定角膜知觉,并进行泪液分泌功能测定、泪膜破裂时间（BUT）测定和共焦显微镜观察,对侧眼作为对照组。结果三叉神经痛组角膜知觉测试的纤维长度为（54．348±6．793）mm,正常对照组的角膜知觉平均值为（55．217±6．480）him,差异无统计学意义（t=0．641,P=0．528）;三叉神经痛组SchiemerI试验的滤纸浸湿长度平均值为（9．390±6．583）mm,正常对照组为（9．300±5．295）mm,差异无统计学意义（t=0．070,P=0．945）;2组的BUT平均值分别为（6．09±4．177）S和（6．13±4．799）s,差异无统计学意义（t=-0．085,P=0．933）。角膜鼻侧、颞侧、上方、下方和中央区上皮下神经丛密度与对照组比较,差异均无统计学意义（P=0．840、0．459、0．268、0．120、0．607）。共焦显微镜下三叉神经痛组角膜上皮下神经丛神经纤维数量减少、扭曲;角膜基质中神经丛纤细、盘旋、弯曲;正常对照组上皮下神经纤维分布较密集,平行分布;角膜基质中神经纤维笔直走行,较上皮下神经纤维粗大且分支较多。结论共焦显微镜结果显示三叉神经痛患者患侧眼较正常眼角膜神经扭曲,但眼表功能和角膜神经密度计数无明显改变。     

Acute unilateral total visual loss after retrogasserian phenol injection for the treatment of trigeminal neuralgia: a case report

Phenol is a neurolytic agent that is widely used by percutaneous retrogasserian injection in the treatment of trigeminal neuralgia. We report a 78-year-old man who had acute unilateral total visual loss after retrogasserian phenol injection for the treatment of trigeminal neuralgia. The patient's visual acuity, eye movements and the pupillary defect did not improve in the affected eye even after mechanical decompression within 30 minutes and medical treatment, neither in the early period nor during the following two months. The visual loss in this patient seems to result from phenol neurotoxicity rather than mechanical compression of the intraorbital optic nerve. This case demonstrates that although percutaneous retrogasserian phenol injection is a relatively safe and noninvasive method of treatment for trigeminal neuralgia, severe complications may occur.     

Neuro-ophthalmic considerations in trigeminal neuralgia and its surgical treatment

PURPOSE OF REVIEW: In this review, we will briefly discuss the clinical manifestations and etiology of trigeminal neuralgia, outline the neuro-ophthalmic features of trigeminal neuralgia, and detail the neuro-ophthalmic side effects and complications of the surgical treatment of trigeminal neuralgia. RECENT FINDINGS: There is a variety of surgical treatment modalities available for patients with trigeminal neuralgia intolerable or resistant to medical therapy. Significant ocular and visual morbidity can result from the surgical treatment of trigeminal neuralgia. SUMMARY: Percutaneous or open surgical procedures for trigeminal neuralgia can result in corneal anesthesia, neurotrophic keratitis, exposure keratitis, herpetic keratitis, ocular motor cranial neuropathies, and optic neuropathy. Ophthalmologists should be aware of these potential problems because they may need to evaluate and provide care to patients with ocular or visual complaints following surgery for trigeminal neuralgia.     

Trigeminal schwannoma as a cause of chronic, isolated sixth nerve palsy

We treated two patients with trigeminal schwannomas, each of whom had an isolated, ipsilateral, abducens nerve palsy. Both patients initially had normal computed tomographic scan results and no neurologic signs of trigeminal nerve dysfunction. Trigeminal dysfunction did not develop until several years after the initial sixth nerve palsy, at which time a repeat examination disclosed the schwannoma. Accurate detection of a trigeminal schwannoma requires a careful clinical examination and appropriate neuroimaging studies. When such studies give normal results, they should be repeated if symptoms worsen or if new symptoms develop.     

Quantitative determination of corneal sensitivity in idiopathic trigeminal neuralgia and after neurosurgical interventions on the trigeminal nerve

The purpose of our study was to determine, by use of the electronic Draeger esthesiometer in quantitative, reproductive measurements, the corneal sensitivity in 55 patients with medically treated idiopathic trigeminal neuralgia before and after surgical treatment of the trigeminal nerve and root. 37 patients were included in a prospective study with measurements before and after glycerol rhizotomy, radiofrequency rhizotomy and microvascular decompression (MVD) of the trigeminal root. Moreover, 18 patients were examined retrospectively 7 to 17 years after retrogasserian rhizotomy according to Spiller/Frazier. Corneal sensitivity was determined by the mean value of 3 measurements in each of 5 positions on both eyes. Evaluation of data leads to the following conclusions: In case of neuralgia in the V1 and V2 divisions, corneal sensitivity may decrease without any clinical manifestation. The hypothesis that V2 contributes to corneal innervation got further evidence. Microvascular decompression may lead to a severe decrease of corneal sensitivity. In terms of complications concerning sensory loss of cornea, radiofrequency rhizotomy was the less risky treatment, followed by glycerol rhizotomy, MVD and the retrogasserian rhizotomy according to Spiller/Frazier. Sensitivity affected by prior medical or surgical treatment does not necessarily decrease by further surgical therapy. Postoperatively up to six weeks, corneal sensitivity remained almost the same as shortly after surgical procedure. Keratitis was observed in only one case retrospectively.     

Acute Unilateral Total Visual Loss after Retrogasserian Phenol Injection for the Treatment of Trigeminal Neuralgia: A Case Report

Phenol is a neurolytic agent that is widely used by percutaneous retrogasserian injection in the treatment of trigeminal neuralgia. We report a 78-year-old man who had acute unilateral total visual loss after retrogasserian phenol injection for the treatment of trigeminal neuralgia. The patient's visual acuity, eye movements and the pupillary defect did not improve in the affected eye even after mechanical decompression within 30 minutes and medical treatment, neither in the early period nor during the following two months. The visual loss in this patient seems to result from phenol neurotoxicity rather than mechanical compression of the intraorbital optic nerve. This case demonstrates that although percutaneous retrogasserian phenol injection is a relatively safe and noninvasive method of treatment for trigeminal neuralgia, severe complications may occur.     

Congenital corneal anesthesia

Congenital corneal anesthesia is a rare clinical entity that poses a diagnostic dilemma, particularly in the pediatric age group. The sensory deficit may be confined to the cornea, or extend to other divisions of the trigeminal nerve. The sensory deficit may occur as an isolated abnormality, as part of a complex neurological syndrome, or it may occur in association with multiple somatic abnormalities and congenital insensitivity to pain. This condition usually presents between the ages of 8 to 12 months. Poor vision, photophobia, conjunctival injection, and corneal ulceration in the absence of pain and distress in a child should alert the clinician to the possibility of anesthetic cornea. In the early stages of presentation, punctuate keratopathy is the main feature, which may progress to non-healing persistent corneal epithelial defects. This stage may progress to acute corneal lysis and perforation. In most patients, conservative approaches such as copious lubrication, prevention of self-harm and cautious use of bandage contact lenses are effective in preventing progressive corneal damage. Tarsorrhapy is effective in promoting epithelial healing and permanent lateral tarsorraphy may prevent further development of epithelial defects. Amniotic membrane graft may be considered in order to improve epithelial healing. Corneal grafts carry a poor prognosis. Accurate initial diagnosis, evaluation, and proper management are paramount to prevent visual loss due to long-term complications of corneal anesthesia. This review of the literature outlines the problems and approaches in diagnosis, evaluation, and management of this rare condition.     

Postherpetic ophthalmic neuralgia

Postherpetic ophthalmic neuralgia is the final stage of a varicella zoster infection. Many years after chickenpox infection, patients can develop herpes zoster in one or more specific dermatomal regions. The ophthalmic branch of the trigeminal nerve and the thoracic nerves are most commonly affected. Younger patients are less prone to postherpetic neuralgia than the older. Patients with a depression in cell-mediated immunity are more susceptible to develop postherpetic pain. Postherpetic ophthalmic neuralgia is a neuropathic pain and can be treated by anticonvulsants and tricyclic antidepressants. Neurodestructive procedures are not recommended as they enhance destruction and neuropathic pain. Sympathetic nerve blocks can be helpful. Neurostimulation is the last therapeutic resort.     

Vestibular Paroxysmia: (Disabling Positional Vertigo)

Evidence is presented for neurovascular cross-compression of the eighth nerve as the probable cause for vestibular paroxysmia (also termed disabling positional vertigo), a condition that can be treated effectively by carbamazepine. In analogy to trigeminal neuralgia, the diagnosis is based on five characteristic features: (1) short attacks of rotational or to-and-fro vertigo lasting from seconds to minutes, (2) attacks frequently dependent on particular head positions and whose duration is modified by changing head position, (3) hypacusis or tinnitus permanently or during the attack, (4) measurable auditory or vestibular deficits by neurophysiological methods, and (5) efficacy of carbamazepine. However, a pathognomonic sign is still lacking and current imaging techniques for identification of causative nerve-vessel contacts still have to be improved.     

Diagnostic yield for neuroimaging in patients with unilateral eye or facial pain

BACKGROUND: The neuroimaging evaluation of isolated pain in or around the eye has not been studied previously. We report the low diagnostic yield of neuroimaging in patients who have a normal ocular examination and unilateral eye pain or facial pain predominantly affecting the eye. METHODS: Retrospective review of patients referred to 3 neuro-ophthalmology practices for unexplained pain in or around the eye. Inclusion criteria were adults with isolated unilateral eye/facial pain, neuroimaging, and a normal eye exam. Ex-clusion criteria were symptoms typical of a defined pain syndrome (e.g., trigeminal neuralgia or giant cell arteritis), and exam findings that would account for the pain. RESULTS: One hundred and twenty-seven (127) of the 760 reviewed patients met study criteria, and underwent MRI (75) or CT (34) scans, or both (18). Imaging was normal in 106 (83%). Abnormalities (n = 21) on imaging (17%) included nonspecific T2-weighted hyperintensities (10), sinusitis (5), superior ophthalmic vein enlargement (1), pontine lacunar infarct (1), Chiari malformation (1), thalamic mass (1), old occipital stroke (1), and focal enlargement of the third cranial nerve (1). INTERPRETATION: Although imaging showed abnormalities in 17% of cases of isolated pain in or around the eye, only 2 abnormalities were believed to be possibly related to the pain and only 1 case was probably related. The diagnostic yield of neuroimaging in patients with a normal examination and isolated, unilateral eye/facial pain referred to a neuro-ophthalmologist is low.     

Manifestations of possible trophic changes in the corneal epithelium due to decreased trigeminal function

Neurotrophic corneal changes are described on the basis of six cases: 1) diabetes mellitus; 2) after surgery for trigeminal neuralgia; 3) intracerebellar hemorrhage; 4) postinfectious trigeminal neuropathy; 5) Wallenberg's syndrome; 6) encircling procedure for retinal detachment. The symptoms in common suggest a decrease in the trophic influence of the trigeminal nerve.     

Congenital corneal anesthesia related to trigeminal anesthesia: case report

Corneal anesthesia is a rare condition, therefore its diagnosis is frequently impaired or it is not noticed during the anterior segment examination. Case report of a 18-year-old patient referred to our Corneal and External Disease Department who complained of dry eye symptoms and with a suspicion of Sj?gren's syndrome. She had amblyopia of the right eye, consequence of corneal leucoma over the visual axis secondary to a fingernail traumatism inflicted by herself in childhood. On the ophthalmologic examination corneal sensitivity was absent in both eyes. Severe dry eye and breakup time less than four seconds. Diagnosis of congenital corneal anesthesia was established, secondary to trigeminal anesthesia found on neurological evaluation of facial sensitivity. She also showed sudden movements of the chin which evidenced sensorial pathology of the trigeminal nerve. The general ophthalmologist and specially anterior segment specialists must perform tests for corneal sensitivity during the routine eye examination.     

Periocular manifestations of trigeminal trophic syndrome: A case series and literature review

Trigeminal trophic syndrome (TTS) is a condition whereby persistent facial ulceration presents consequent to central or peripheral insult to the trigeminal nerve. Lesions are created by repetitive self-inflicted manipulation and trauma of dysaesthetic skin within the trigeminal dermatome.

We discuss four cases with aetiologies varied from presumed microvascular compromise to resection of cerebral meningioma, cerebrovascular accident, and herpes zoster ophthalmicus.

We discuss the management of the under-recognised associated periocular skin ulcerations that result from physical manipulation of dysesthic skin and prove to be persistent and challenging to treat. Patient education and counselling are crucial in understanding and preventing the detrimental effect of physical manipulation of the skin. Occlusive dressings can reduce recurrent trauma. Topical lubricants, antibiotics, or autologous serum may be needed in cases with corneal involvement or exposure. Surgical interventions may be used, but frequently fail if the underlying neurological pathology and skin manipulation has not been adequately addressed.

TTS should be suspected in persistent or recurrent facial ulceration with concomitant anaesthesia and paraesthesia in the trigeminal distribution, with alar nasi involvement being a key feature.     

Monocular blindness as a complication of trigeminal radiofrequency rhizotomy

PURPOSE: To raise the awareness of the rare complication of monocular blindness resulting from radiofrequency rhizotomy for trigeminal neuralgia. METHODS: Case series from tertiary referral centers. Patients were referred after complaint of loss of vision. RESULTS: We report three patients who had trigeminal neuralgia and underwent treatment by radiofrequency rhizotomy. Each developed complete loss of vision in one eye immediately after the procedure. One patient died of an unrelated cause, and pathologic analysis revealed changes compatible with acute trauma to the right optic nerve. CONCLUSION: Our third case confirms the hypothesis that traumatic optic neuropathy after trigeminal radiofrequency rhizotomy results from malpositioning of the rhizotomy needle through the inferior orbital fissure into the orbital apex rather than the foramen ovale.     

Painful ophthalmoplegia--a clinical case

We present the case of the patient S.M., female, 32 years old, who came in our eye clinic for left painful ophthalmoplegia. The onset of the clinical manifestations was a year and half ago, with the decrease of visual acuity at left eye, left partial ophthalmoplegia (partial lesion of the left oculomotor nerve) and secondary left trigeminal neuralgia. The paraclinical investigations excluded the systemic and locoregional diseases. CT exams, the left internal carotid angiography and the surgical intervention at the Neurosurgery Clinic in Timi?oara revealed a left juxtasella tumor at the base skull, located extradural, which capsule invade the left nerve oculomotor. We conclusion that the painful ophthalmoplegia are complex clinical syndromes, with a different etiopathogenesis (inflammatory, tumoral, vascular malformations: aneurysm etc.) and their diagnosis and treatment need a good interdisciplinary collaboration: ophthalmologist-neurologist-neurosurgeon-endocrinologist-paraclinical exams.     

SUNCT syndrome: Short-lasting Unilateral Neuralgiform headache with Conjunctival injection and Tearing

The SUNCT syndrome refers to Short-lasting Unilateral Neuralgiform headache with Conjunctival injection and Tearing. It is characterized by brief attacks of severe unilateral pain in the orbitotemporal region, associated with ipsilateral cranial autonomic disturbances. All SUNCT patients experience ipsilateral conjunctival injection and lacrimation. Mean age of onset is 50 years with a male predominance. The syndrome is often misdiagnosed as trigeminal neuralgia or cluster headache. Primary and secondary forms exist, the secondary form is most commonly associated with lesions of the posterior fossa or pituitary adenoma. The SUNCT syndrome is refractory to most commonly employed therapies. Lamotrigine has recently been reported as an effective first line therapy.     

SUNCT Syndrome: Short-Lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing

The SUNCT syndrome refers to Short-lasting Unilateral Neuralgiform headache with Conjunctival injection and Tearing. It is characterized by brief attacks of severe unilateral pain in the orbitotemporal region, associated with ipsilateral cranial autonomic disturbances. All SUNCT patients experience ipsilateral conjunctival injection and lacrimation. Mean age of onset is 50 years with a male predominance. The syndrome is often misdiagnosed as trigeminal neuralgia or cluster headache. Primary and secondary forms exist, the secondary form is most commonly associated with lesions of the posterior fossa or pituitary adenoma. The SUNCT syndrome is refractory to most commonly employed therapies. Lamotrigine has recently been reported as an effective first line therapy.     

Implicación ocular en el tratamiento con radiofrecuencia pulsada del ganglio esfenopalatino

Pulsed radiofrequency treatment of the sphenopalatine ganglion is an important interventional treatment in refractory cases of trigeminal neuralgia or atypical facial pain, given the easy access to its location. Despite the fact that complications from this technique are rare and it is a fairly safe procedure, ophthalmologists should know about it due to the anatomical relations of this ganglion.