Unilateral non-healing ulcers in zosteriform pattern.

Trigeminal trophic syndrome (TTS) is a rare disease that occurs after injury to the trigeminal nerve. Though this condition has been reported in the early 20th century, it is still a rare entity, with only around 200 cases reported so far. It characteristically presents with persistent facial ulceration with loss of sensation and paraesthesia along the distribution of the trigeminal nerve. We here report a case of TTS developing as a complication of herpes zoster, which possibly occurred due to the nerve damage caused by varicella-zoster virus.

Concurrent facial and trigeminal nerve palsies in a child following COVID-19 vaccination with the Pfizer vaccine.

We present the case of a teenaged boy who attended our Ear, Nose and Throat Emergency clinic with a left-sided lower motor neuron (LMN) facial nerve paralysis associated with sensory loss in the distribution of the ophthalmic (V1) and maxillary (V2) divisions of the trigeminal nerve. This happened 3 days following a first dose of the Pfizer-BioNTech BNT162b2 vaccine. He had a House-Brackmann grade V facial palsy, with marked inability to close the left eye. He was treated with a 10-day course of oral steroids and referred to ophthalmology for eye care. He had an MRI scan of the head, which revealed no space occupying lesions or other abnormalities. Over the 6-week period of follow-up, the patient's V1 and V2 sensation gradually resolved, along with improvement of his LMN facial nerve palsy to House-Brackmann grade 3. Despite the potential temporal relationship, it is not possible to establish a causal relationship between the patient's symptoms and the Pfizer-BioNTech BNT162b2 vaccine, thus further research is required.

Concurrent Bell's Palsy and Facial Pain Improving with Multimodal Chiropractic Therapy: A Case Report and Literature Review.

BACKGROUND Bell's palsy, also called facial nerve palsy, occasionally co-occurs with trigeminal neuropathy, which presents as additional facial sensory symptoms and/or neck pain. Bell's palsy has a proposed viral etiology, in particular when occurring after dental manipulation. CASE REPORT A 52-year-old Asian woman presented to a chiropractor with a 3-year history of constant neck pain and left-sided maxillary, eyebrow, and temporomandibular facial pain, paresis, and paresthesia, which began after using a toothpick, causing possible gum trauma. She had previously been treated with antiviral medication and prednisone, Chinese herbal medicine, and acupuncture, but her recovery plateaued at 60% after 1 year. The chiropractor ordered cervical spine magnetic resonance imaging, which demonstrated cervical spondylosis, with no evidence of myelopathy or major pathology. Treatment involved cervical and thoracic spinal manipulation, cervical traction, soft-tissue therapy, and neck exercises. The patient responded positively. At 1-month follow-up, face and neck pain and facial paresis were resolved aside from residual eyelid synkinesis. A literature review identified 12 additional cases in which chiropractic spinal manipulation with multimodal therapies was reported to improve Bell's palsy. Including the current case, 85% of these patients also had pain in the face or neck. CONCLUSIONS This case illustrates improvement of Bell's palsy and concurrent trigeminal neuropathy with multimodal chiropractic care including spinal manipulation. Limited evidence from other similar cases suggests a role of the trigeminal pathway in these positive treatment responses of Bell's palsy with concurrent face/neck pain. These findings should be explored with research designs accounting for the natural history of Bell's palsy.

Etiologies and Utility of Diagnostic Tests in Trigeminal Neuropathy.

OBJECTIVE: To evaluate the utility of diagnostic studies in identifying treatable etiologies of trigeminal neuropathy (TNP). PATIENTS AND METHODS: We performed a review of consecutive patients with nontraumatic, noniatrogenic TNP seen at Mayo Clinic between January 1, 2000, and August 31, 2019. Patients were excluded if they had trigeminal neuralgia without neuropathy or if their diagnostic work-up had been completed elsewhere. Data were analyzed to determine which diagnostic studies were most useful in identifying treatable etiologies. RESULTS: In total, 439 patients were included. The mean ± SD age was 56.3±13.6 years and 285 (64.9%) were female. Among the 180 cases in which an etiology was identified (41.0%), neoplasms were causative in 76 (42.2%), while specific connective tissue diseases were implicated in 71 (39.4%). Bilateral TNP (n=83) was associated with the presence of underlying connective tissue disease (P<.01). Identification of etiology was made by magnetic resonance imaging in 88 cases (48.8%), by abnormal connective tissue disease cascades combined with rheumatology consultation in 42 (23.3%), by a previously known connective tissue disorder in 30 (16.7%), and by abnormal connective tissue disease cascades alone in 8 (4.4%). Among the 439 study patients, electromyography was performed in 211 (48.1%) and lumbar puncture in 139 (31.7%), but their diagnostic utility was low. CONCLUSION: Underlying causes of nontraumatic, noniatrogenic TNP can be identified in approximately 40% of cases. Bilateral TNP is strongly associated with underlying connective tissue disease. Careful history taking, dedicated magnetic resonance imaging, and connective tissue panels have the greatest diagnostic utility. Electromyography and cerebrospinal fluid analysis are unlikely to elucidate treatable etiologies of TNP.

Blink Reflex in Neurotrophic Keratopathy: An Electrophysiological Evaluation.

PURPOSE: Neurotrophic keratitis (NK) is a rare condition which may result in visual loss. This case review investigates if there may be an association between NK and the blink reflex in the absence of facial nerve palsy and lagophthalmos. METHODS: This is a retrospective case review of 5 patients with trigeminal nerve damage referred to the oculoplastic department with suspected anesthetic corneae. Information on etiology, symptoms, duration, associated medical conditions, medications, examination findings including Mackie stage of keratopathy, management of keratopathy, and blink electrophysiology results was obtained. RESULTS: All 5 patients demonstrated absence of corneal sensation. All patients had preserved facial nerve function with no evidence of lagophthalmos. Keratopathy ranged from Mackie stage 0-2. Management ranged from ocular lubricants to Botulinum-toxin-induced ptosis. Blink studies demonstrated reduction in amplitude as well as increased latency in 2 patients, conferring reduced blink strength. Two patients demonstrated an absent blink reflex on the affected side. One patient had blink latency within the normative range; this patient recovered corneal sensation and was discharged. CONCLUSIONS: Our finding of reduced amplitude in blink studies offers both a factor in pathogenesis of NK and a potential therapeutic target. Additionally, blink studies may provide prognostic information for recovery and therefore guide management. We suggest performing blink electrophysiology in patients with trigeminal nerve damage to assess nerve function.

Bilateral corneal perforation caused by neurotrophic keratopathy associated with leprosy: a case report.

BACKGROUND: Neurotrophic keratopathy (NK) is a rare degenerative corneal disease caused by damage to the trigeminal nerve. We hereby describe a severe case with bilateral corneal perforation due to leprosy (Hansen's disease)-associated NK. CASE PRESENTATION: An 89-year-old man with a history of leprosy treated 40 years previously in our sanatorium developed bilateral corneal perforation due to NK. He had a history of bilateral persistent epithelial defects and bacterial keratitis. Although epithelialization was obtained with the use of autologous serum eye drops, progressive corneal thinning concomitant with stromalysis led to bilateral perforation. Over one month treatment with topical antibiotics, anti-inflammatory and lubricants resulted in healing of the epithelial defects and corneal perforations. A Cochet-Bonnet esthesiometer demonstrated a total absence of corneal sensation in both eyes. CONCLUSIONS: The present case indicated the irreversible nerve damage due to leprosy that had been cured 23 years ago, which can progress over the years and cause bilateral corneal perforations.

Neurotrophic Keratitis in a Pediatric Patient With Goldenhar Syndrome and Trigeminal Aplasia Successfully Treated by Corneal Neurotization.

Herein, the authors report an unusual case of a 6-year-old boy with right-sided Goldenhar syndrome and trigeminal nerve aplasia who developed neurotrophic keratopathy (NK). Despite the use of therapeutic contact lenses and multiple temporary tarsorrhaphy, NK worsened showing a central corneal scar, neovascularization, and significant stromal thinning, with risk of corneal perforation. Cochet-Bonnet esthesiometry revealed complete corneal anesthesia. To minimize additional corneal complications, the patient underwent indirect corneal neurotization by a sural nerve autograft anastomosed to the contralateral supratrochlear nerve. At 24-month follow up, no epithelial defects, complications, or recurrence were observed. Significant improvements in corneal sensitivity with esthesiometry score of 20 mm and reflex blinking were achieved. This case highlights corneal anesthesia should be suspected among Goldenhar syndrome ophthalmologic abnormalities and monitored before corneal changes become irreversible. Since corneal neurotization can successfully improve corneal sensation, it could be considered as an early therapeutic option to avoid refractory NK.

Microsurgical Resection of Trigeminal Schwannoma via Anterior Petrosal Approach: 2-Dimensional Operative Video.

Trigeminal schwannomas are complex lesions that may be related to many critical neurovascular structures. We present the case of a 59-year-old male presenting a history of left-sided trigeminal neuralgia. Preoperative imaging demonstrated a mass highly suggestive of a trigeminal schwannoma, and microsurgical resection was indicated considering the progressive symptomatology and important mass effect (Video 1). A middle fossa route including an anterior petrosectomy was chosen. The patient was placed supine with the head rotated to the contralateral side, and an arcuate incision was performed. A V-shaped zygomatic osteotomy was done to mobilize the temporalis muscle more inferiorly and better expose the middle fossa floor. Following craniotomy, peeling of the dura propria from the lateral wall of cavernous sinus was carried out starting by coagulation of middle meningeal artery. Some tumor was already identified and removed, and then the anterior petrosectomy was performed until we exposed the posterior fossa dura. The middle fossa dural incision was connected with the other one at the posterior fossa dura, by coagulation of the superior petrosal sinus. The tentorium was completely cut toward the incisura. After lesion debulking, the tumor was progressively removed by peeling the arachnoid from the lesion to maintain arachnoid planes and preserve the nerves and their blood supply. Postoperative imaging demonstrated complete tumor resection. The patient's symptoms improved, and there were no neurologic deficits on follow-up. Extensive laboratory training is fundamental to be familiarized with the normal anatomic nuances and prepared to face the anatomy distorted by lesion. Informed consent was obtained from the patient for the procedure and publication of this operative video.

Associated Neurotrophic Keratopathy in Complex Regional Pain Syndrome.

PURPOSE: To report a case of neurotrophic keratopathy (NK) in a patient with complex regional pain syndrome (CRPS) with ipsilateral facial involvement. METHODS: Case report. RESULTS: An 18-year old woman with a 5-year history of CRPS type I, a systemic disorder with a neuropathic component with associated limb and right facial involvement, presented with an insidious onset of blurred vision and pain in the right eye. Ocular examination revealed decreased corneal sensation, as measured by Cochet-Bonnet testing, associated with recurrent epithelial defects and whorl-like superficial corneal epitheliopathy. NK was suspected and confirmed by in vivo confocal microscopy (IVCM), which revealed rarefaction of the subbasal nerve plexus in the affected eye. To enhance corneal nerve health, plasma rich in growth factors drops were used. Persistence of NK prompted a superficial keratectomy with placement of an amniotic membrane graft and a course of cenegermin 0.002% (Oxervate; Dompé Farmaceutici SpA, Italy) in the postoperative period. This combination therapy resulted in successful epithelial closure and vision improvement after 8 weeks of therapy with no recurrence of disease for 11 months. Importantly, at that final visit, IVCM demonstrated growth of corneal nerves for the first time in this patient. CONCLUSIONS: This is the first case report of NK occurring in the context of CRPS, a neuropathy with ipsilateral facial pain involvement. IVCM was important in the diagnosis of NK, which responded successfully to ocular surface treatments focused on nerve health stimulation that ultimately resulted in corneal nerve growth.

CORNEAL NEUROTIZATION IN A PATIENT WITH SEVERE NEUROTROPHIC KERATOPATHY. CASE REPORT.

PURPOSE: Neurotrophic keratopathy (NK) is a degenerative corneal disease caused by damage to the trigeminal innervation due to a decrease in corneal sensitivity or complete anaesthesia. Impaired corneal innervation leads to morphological and metabolic disorders of the epithelium. In addition, it also leads to the development of recurrent or persistent epithelial defects in corneal ulcers, which may progress to stromal lysis and corneal perforation. One possible solution for severe NK is reinnervation of the anaesthetic cornea (corneal neurotization) using the supraorbital nerve and an autologous sensory nerve graft (indirect neurotization). This article presents the results of corneal neurotization in a young male patient with persistent epithelial defects and corneal ulcers due to corneal denervation. RESULTS: A 22-year-old man with a history of neurosurgery for astrocytoma of the cerebellum and trunk on the right side at the age of 2 years, was observed for postoperative paresis of the right facial nerve with lagophthalmos in his childhood. The presence of asymptomatic dysfunction of the right trigeminal nerve was also noted. At the age of 22 years, after right eyeball contusion, the vision of the right eye decreased and a persistent epithelial defect developed, followed by corneal ulceration. Due to the exhaustion of therapeutic options in a young patient with corneal anaesthesia, the cornea was reinnervated via the contralateral supraorbital nerve using an autologous sural nerve graft. Five months after the surgery, the sensitivity of the cornea of the right eye began to recover. After amniotic membrane transplantation, the extensive epithelial defect healed, and the opaque corneal stroma gradually cleared up. CONCLUSION: The reinnervation of the anaesthetic cornea (corneal neurotization) using the supraorbital nerve and the autologous sensory nerve graft represents a new solution for severe NK treatment. The severe corneal condition in our patient healed after the surgery.

[Ocular manifestations of viral diseases]. / Atteintes ophtalmologiques des infections virales.

Viral infections may involve all ocular tissues and may have short and long-term sight-threatening consequences. Among them, ocular infections caused by herpesviruses are the most frequent. HSV-1 keratitis and kerato-uveitis affect approximately are the leading cause of infectious blindness in the Western world, mainly because of corneal opacification caused by recurrences. For this reason, they may warrant long-term antiviral prophylaxis. Herpes zoster ophthalmicus, accounts for 10 to 20% of all shingles locations and can be associated with severe ocular involvement (keratitis, kerato-uveitis) of which a quarter becomes chronic/recurrent. Post herpetic neuralgias in the trigeminal territory can be particularly debilitating. Necrotizing retinitis caused by herpesviruses (HSV, VZV, CMV) are seldom, but must be considered as absolute visual emergencies, requiring urgent intravenous and intravitreal antiviral treatment. Clinical pictures depend on the immune status of the host. Adenovirus are the most frequent cause of infectious conjunctivitis. These most often benign infections are highly contagious and may be complicated by visually disabling corneal lesions that may last over months or years. Some arboviruses may be associated with inflammatory ocular manifestations. Among them, congenital Zika infections may cause macular or optic atrophy. Conjunctivitis is frequent during the acute phase of Ebola virus disease. Up to 15% of survivors present with severe chronic inflammatory ocular conditions caused by viral persistence in uveal tissues. Finally, COVID-19-associated conjunctivitis can precede systemic disease, or even be the unique manifestation of the disease. Utmost caution must be taken because of viral shedding in tears.

Trigeminal trophic syndrome: an important simulator of discoid cutaneous lupus erythematosus - a case series.

Trigeminal trophic syndrome occurs secondary to trigeminal nerve injury, leading to anaesthesia and paraesthesia, with consequent vigorous facial skin manipulation and lesion production, simulating other facial diseases such as ulcerative discoid lupus erythematosus, tumours and other artificially produced lesions. Ulceration and destruction of the ala nasi is a typical feature besides scratching end excoriations in the cutaneous segment affected. In this series, we present the features of five patients with trigeminal trophic syndrome, highlighting possible confusion with cutaneous lupus. Differential diagnoses, including discoid lupus erythematosus, are discussed, as well as possible treatment modalities.

Hypertrophic Interstitial Neuropathy of the Trigeminal Nerve: Case Report and Literature Review.

BACKGROUND AND IMPORTANCE: Hypertrophic interstitial neuropathy (HIN) is an uncommon, non-neoplastic lesion typically affecting peripheral nerves. Cranial nerve (CN) involvement is exceedingly rare. We present a case of isolated trigeminal nerve HIN manifesting with V3 distribution neuralgia. CLINICAL PRESENTATION: A 50-yr-old male presented with left sided trigeminal neuralgia refractory to medical management. The patient underwent retromastoid craniectomy for possible microvascular decompression. Intra-operatively, the trigeminal nerve appeared to be focally enlarged with a sausage-like configuration. We selectively resected 1 fascicle which was predominantly involved. Histopathological examination revealed onion bulb formations composed of Schwann cells around centrally placed axons. A diagnosis of HIN was made. Postoperatively, the patient experienced complete resolution of symptoms. CONCLUSION: This is the third case of isolated trigeminal nerve HIN in the literature. We performed a selective resection in a patient presenting with trigeminal neuralgia, resulting in complete resolution of symptoms. It is reported here with intraoperative microscope images, along with a review and analysis of this topic as it related to CN.