Síndrome de Horner: revision de la literatura / Horner´s Syndrome: literature review

Resumen El Síndrome de Horner (SH) es una condición provocada por un grupo heterogéneo de patologías. Estas tienen en común el daño de la vía simpática. La localización más común de la lesión es a nivel preganglionar, por trauma o cirugías en región cervical, torácica o ápex pulmonar. El diagnóstico es primordialmente clínico mediante la identificación de la triada de miosis, ptosis y anhidrosis. Se confirma con las pruebas para cocaína e hidroximetanfetamina. El tratamiento se lleva a cabo primero con agentes farmacológicos. Los que tienen disminución del campo visual por la ptosis o por razones cosméticas se llevan a tratamiento quirúrgico. En ese caso los abordajes operatorios recomendados para ptosis leve son el procedimiento de Fasanella-Servat, el avance de aponeurosis del elevador y en casos severos el cabestrillo frontal. El objetivo es hacer una revisión de los algoritmos diagnósticos y terapéuticos del SH para lograr un abordaje sistemático debido a las múltiples etiologías que posee.

Abstract Horner Syndrome is a condition caused by a heterogeneous group of pathologies. These have in common the damage of the sympathetic pathway. The most common location of the lesion is at the preganglional level, due to trauma or surgeries in the cervical, thoracic or pulmonary apex region. The diagnosis is primarily clinical by identifying the triad of myosis, ptosis and anhidrosis. It is confirmed with the cocaine and hydroxymethamphetamine tests. Treatment is first carried out with pharmacological agents. Those with diminished visual field due to ptosis or cosmetic reasons are undergoing surgical treatment. In this case, the recommended operative approaches for mild ptosis are the Fasanella-Servat procedure, the elevator aponeurosis advance and, in severe cases, the frontalis sling. The objective is to review the diagnostic and therapeutic algorithms of SH in order to achieve a systematic approach due to the multiple aetiologies it possesses.

Unilateral mydriasis: a complication of spine surgery in prone position / Midríase unilateral: uma complicação da cirurgia de coluna em decúbito ventral

Abstract Prone position though is commonly used for better access to surgical site, but may be associated with a variety of complications. Perioperative Visual Disturbances or loss is rare but a devastating complication that is primarily associated with spine surgeries in prone position. In this case we report a 42 year old ASA-II patient who developed anisocoria with left pupillary dilatation following lumbar microdiscectomy in prone position. Following further evaluation of the patient, segmental pupillary palsy of the left pupillary muscles was found to be the possible cause of anisocoria. Anisocoria partially improved but persisted till follow up.

Resumo O posicionamento em decúbito ventral, embora comumente usado para melhorar o acesso ao local cirúrgico, pode estar associado a uma variedade de complicações. Distúrbios ou perda visual no Perioperatório é uma complicação rara, mas devastadora, que está principalmente associada à cirurgia de coluna vertebral em decúbito ventral. Relatamos aqui o caso de um paciente de 42 anos de idade, ASA - II, que desenvolveu anisocoria com dilatação pupilar esquerda após microdiscetomia lombar em decúbito ventral. Após uma avaliação adicional do paciente, observamos que a paralisia segmentar dos músculos pupilares esquerdos seria a possível causa de anisocoria. A anisocoria melhorou parcialmente, mas persistiu até o acompanhamento.

Delayed Onset Abducens Nerve Palsy and Horner Syndrome after Treatment of a Traumatic Carotid-cavernous Fistula

PURPOSE: We report a patient with delayed-onset abducens nerve palsy and Horner syndrome after endovascular treatment of traumatic carotid-cavernous fistula (CCF). CASE SUMMARY: A 68-year-female visited our ophthalmic department complaining of gradual-onset ptosis of the left eye and horizontal diplopia. She had undergone endovascular treatment to treat left-sided traumatic CCF after a car accident 10 years before; she had been told at that time that the treatment outcome was favorable. The left-sided ptosis gradually developed 6 years after the procedure, accompanied by diplopia. The left eye exhibited miosis and the extent of anisocoria increased in dim light. An extraocular examination revealed 30 prism diopters of left esotropia in the primary gaze and a −4 abduction limitation of the left eye. CCF recurrence was suspected; however, magnetic resonance imaging with magnetic resonance angiography of brain did not support this. The esotropia did not improve during the 6-month follow-up and strabismus surgery was performed. CONCLUSIONS: Delayed-onset abducens nerve palsy and Horner syndrome can develop even after successful endovascular treatment of CCF. Strabismus surgery should be considered in patients whose diplopia does not spontaneously improve.

Oculomotor nerve palsy associated with internal carotid artery: case reports

Oculomotor nerve palsy limits the specific direction eyeball movement, and represents diplopia, mydriasis, and ptosis. The vascular-associated etiologies of oculomotor nerve palsy are the microvascular ischemia due to hypertension or diabetes, or compression of the nerve by the aneurysm. For the aneurysm, if not treated properly, it may result in mortality or severe neurological impairment. Thorough history taking, physical examinations, and proper imaging modality are needed to make an accurate diagnosis. A 76-year-old female with decreased mentality and anisocoria presented at our emergency department. An 83-year-old female presented with right ptosis and lateral-side deviated of the right eyeball. No definite lesion was noted on the initial non-contrast brain computed tomography (CT) and magnetic resonance imaging diffusion. An aneurysm was detected on CT angiography taken several hours later in the former patient. For the latter patient, a giant aneurysm was detected on magnetic resonance angiography that had been performed at another hospital 4 days earlier. These two patients underwent transfemoral cerebral angiography with coiling. They were discharged with no neurological sequelae.

Pupillometry in Chagas disease / Pupilometria na doença de Chagas

ABSTRACT Purpose: We investigated parasympathetic innervation abnormalities of the iris sphincter and ciliary muscles in chronic Chagas disease by measuring pupillary diameter and intraocular pressure. Methods: A group of 80 patients with Chagas disease was compared with 76 healthy individuals without chagasic infection. The following procedures were performed: pupillometry, hypersensitivity test to pilocarpine 0.125%, intraocular pressure measurement (IOP), basal pupil diameter (BPD), absolute pupillary constriction amplitude (ACA), relative pupillary constriction amplitude (RCA) and the presence of anisocoria. Results: The prevalence of anisocoria was higher in chagasic patients (p<0.01). These patients had mean basal pupillary diameter, mean photopic pupillary diameter and mean value of absolute pupillary constriction amplitude significantly lower than non-chagasic ones (p<0.01, mean difference -0.50mm), (p=0.02, mean difference -0.20mm), (p<0.01, mean difference -0.29mm), respectively. The relative pupillary constriction amplitude did not differ between the two groups (p=0.39, mean difference -1.15%). There was hypersensitivity to dilute pilocarpine in 8 (10%) of the chagasic patients in the right eye and in 2 (2.5%) in the left eye and in 1 (1.25%) in both eyes. The mean value of intraocular pressure had a marginal statistical significance between the two groups (p=0.06, mean difference -0.91mmHg). Conclusions: Patients with chagasic infection may exhibit ocular parasympathetic dysfunction, demonstrable by pupillometry and the dilute pilocarpine hypersensitivity test.

RESUMO Introdução: Investigaram-se anormalidades da inervação parassimpática dos músculos esfíncter da íris e ciliar na doença de Chagas crônica, através de medidas pupilares e da pressão intraocular. Métodos: Foram estudados dois grupos, um com 80 chagásicos e outro com 76 indivíduos saudáveis sem infecção chagásica. Foram realizados os seguintes procedimentos: pupilometria, teste de hipersensibilidade à pilocarpina a 0,125%, medida da pressão intraocular (PIO), diâmetro basal da pupila (DBP), amplitude de constrição pupilar absoluta (ACA), amplitude de constrição pupilar relativa (ACR), e presença de anisocoria. Resultados: A prevalência de anisocoria foi maior nos chagásicos (p<0,01). Estes pacientes apresentaram diâmetro basal pupilar médio, diâmetro fotópico médio e valor médio da amplitude de constrição pupilar absoluta, significativamente menores que os não chagásicos, (p<0,01, diferença de média -0,50mm), (p=0.02, diferença de média -0,20mm), (p<0,01, diferença de média -0,29mm), respectivamente. A amplitude de constrição pupilar relativa não diferiu entre os dois grupos (p=0,39, diferença de média -1,15%). Houve hipersensibilidade à pilocarpina diluída em 8 (10%) chagásicos no olho direito em 2 (2,5%) no olho esquerdo e em 1 (1,25%) em ambos os olhos. O valor médio da pressão intraocular teve significância marginal entre os dois grupos (p=0,06, diferença de média -0,91mmHg). Conclusões: Pacientes com infecção chagásica podem apresentar disfunção parassimpática ocular, demonstrável pela pupilometria e pelo teste de hipersensibilidade à pilocarpina diluída.

Approach to pupillary abnormalities via anatomical pathways / 영남의대학술지

The pupillary size and movement are controlled dynamically by the autonomic nervous system; the parasympathetic system constricts the iris, while the sympathetic system dilates the iris. Under normal conditions, these constrictions and dilations occur identically in both eyes. Asymmetry in the pupillomotor neural input or output leads to impaired pupillary movement on one side and an unequal pupil size between both eyes. Anisocoria is one of the most common signs in neuro-ophthalmology, and the neurological disorders that frequently cause anisocoria include serious diseases, such as vascular dissection, fistula, and aneurysm. A careful history and examination can identify and localize pupillary disorders and provide a guide for appropriate evaluations.

Approach to pupillary abnormalities via anatomical pathways / 영남의대학술지

The pupillary size and movement are controlled dynamically by the autonomic nervous system; the parasympathetic system constricts the iris, while the sympathetic system dilates the iris. Under normal conditions, these constrictions and dilations occur identically in both eyes. Asymmetry in the pupillomotor neural input or output leads to impaired pupillary movement on one side and an unequal pupil size between both eyes. Anisocoria is one of the most common signs in neuro-ophthalmology, and the neurological disorders that frequently cause anisocoria include serious diseases, such as vascular dissection, fistula, and aneurysm. A careful history and examination can identify and localize pupillary disorders and provide a guide for appropriate evaluations.

Transient Anisocoria during Medial Blowout Fracture Surgery

Transient anisocoria is rare during blowout fracture reconstruction. We report a case of transient anisocoria occurring during medial blowout fracture reconstruction and review the relevant literature. A 54-year-old woman was struck in the face and was admitted for a medial blowout fracture of the left eye. During the operation, persistent bleeding occurred. To control this bleeding, a 1% lidocaine solution with 1:200,000 epinephrine was applied to the orbital wall with cotton pledgets. In total, 40 mL of local anesthetic was used for the duration of the operation. After approximately three hours of the surgery, the ipsilateral pupil was observed to be dilated, with sluggish response to light. By 3 hours after the operation, the mydriasis had resolved with normal light reflex. In conclusion, neurological and ophthalmologic evaluation must be performed prior to blowout fracture surgery. Preoperative ophthalmic evaluation is simple and essential in ruling out any preexisting neurologic condition. Moreover, surgeons must be aware of the fact that excessive injection of lidocaine with epinephrine for hemostasis during orbital wall surgery can result in intraoperative anisocoria. Anisocoria-related situations must be addressed in a proficient manner through sufficient understanding of the mechanism controlling the pupillary response to various stimuli.

Ross Syndrome with Segmental Anhidrosis and Anisocoria: Application of Finger Winkle Test

Ross syndrome is characterized by a triad of segmental anhidrosis, tonic pupil, and generalized areflexia. Selective postganglionic autonomic denervation could be the differential diagnostic point for other diseases of the autonomic nervous system. Here we report a patient with regional anhidrosis in his left hand and sole, and anisocoria. An evaluation of sweating and the pupillary response together with generalized areflexia confirmed the diagnosis of Ross syndrome. The finger wrinkle test is a simple and useful tool for revealing segmental sympathetic denervation.

A neonate with Joubert syndrome presenting with symptoms of Horner syndrome / 소아과

Joubert syndrome (JS) is characterized by the “molar tooth sign” (MTS) with cerebellar vermis agenesis, episodic hyperpnea, abnormal eye movements, and hypotonia. Ocular and oculomotor abnormalities have been observed; however, Horner syndrome (HS) has not been documented in children with JS. We present the case of a 2-month-old boy having ocular abnormalities with bilateral nystagmus, left-dominant bilateral ptosis, and unilateral miosis and enophthalmos of the left eye, which were compatible with HS. Brain magnetic resonance imaging (MRI) revealed the presence of the MTS. Neck MRI showed no definite lesion or mass around the cervical sympathetic chain. His global development was delayed. He underwent ophthalmologic surgery, and showed some improvement in his ptosis. To the best of our knowledge, the association of HS with JS has not yet been described. We suggest that early neuroimaging should be considered for neonates or young infants with diverse eye abnormalities to evaluate the underlying etiology.

Comparison of ocular function and refractive errors both Hansen patients and old people / 나학회지

BACKGROUND & OBJECTIVE: The purpose of this study is to prove the characteristics of refractive error and visual acuity about hansen patients and old people. To compare the pupil and ocular function was to investigate the characteristics of the eye of the hansen patients. METHODS: Subjects were intended for the elderly over 60 years old 85 years old or younger both hansen patients in Wonju(n=29) and old people in Yangpyeong(n=29). Auto refraction(Topcon KR-7000) to objective refraction was conducted after the subjective refraction. Pupil size at normal illumination and light reflex, response velocity and the reaction states about light reflex were measured and Color vision test were. Writing questionnaires and analyzed the subjective symptoms about two groups. Spss ver.18.0 was used for statistics analysis and significance level was based on a 0.05. Methods were used Crosstabs analysis ,Paired T test and descriptive statistics. RESULTS & CONCLUSION: VA of hansen patients and old people did not differ by 0.29 +/-0.18 and 0.35+/-0.23. (p>0.05). CC of hansen patients and old people did differ by 0.55+/-0.26 and 0.44+/-0.21(p0.05). Old people during normal illumination and light reflex is normal (p<0.05). Direct light reflex state of hansen patients was normal 17.20% and abnormal 82.8%. Pupil's response velocity during light reflex of hansen patients was normal 3.4% and abnormal 96.6%. Pupil's reaction state during light reflex of hansen patients was normal 13.8%and abnormal 86.20%. Direct light reflex state of old people was normal 96.60%and abnormal 3.4%. Subjective symptoms were ranking 1.Dim sighted(23times) 2.Eyestrain(18times) 3.Tears(15times) of Hansen, that were ranking 1.Dim sighted(25times) 2.Tears(8times) 3.Pruritus(8times)of old people. Hansen patients and old people were not differ about result of CV test.

Incidence of pupillary involvement, course of anisocoria and ophthalmoplegia in diabetic oculomotor nerve palsy.

Aims: To derive a reliable estimate of the frequency of pupillary involvement and to study the patterns and course of anisocoria in conjunction with ophthalmoplegia in diabetes-associated oculomotor nerve palsy. Materials and Methods: In this prospective analytical study, standardized enrolment criteria were employed to identify 35 consecutive patients with diabetes-associated oculomotor nerve palsy who were subjected to a comprehensive ocular examination. Standardized methods were used to evaluate pupil size, shape, and reflexes. The degree of anisocoria, if present and the degree of ophthalmoplegia was recorded at each visit. Results: Pupillary involvement was found to be present in 25.7% of the total number of subjects with diabetic oculomotor nerve palsy. The measure of anisocoria was < 2 mm, and pupil was variably reactive at least to some extent in all cases with pupillary involvement. Majority of patients in both the pupil-involved and pupil-spared group showed a regressive pattern of ophthalmoplegia. Ophthalmoplegia reversed much earlier and more significantly when compared to anisocoria. Conclusions: Pupillary involvement in diabetes-associated oculomotor nerve palsy occurs in about 1/4th of all cases. Certain characteristics of the pupil help us to differentiate an ischemic insult from an aneurysmal injury to the 3rd nerve. Ophthalmoplegia resolves much earlier than anisocoria in diabetic oculomotor nerve palsies.

A Case of Iatrogenic Horner's Syndrome after Video-Thoracoscopic Surgery for Primary Pneumothorax

PURPOSE: To report a case of iatrogenic Horner's syndrome after video-thoracoscopic surgery for primary pneumothorax. CASE SUMMARY: An 18-year-old man with ptosis in the right eye was referred to our clinic. The patient had undergone wedge resection via video-thoracoscopic surgery for primary pneumothorax three weeks previously. On ocular examination, the palpebral fissure width was 7 mm in the right lid and 8 mm in the left lid, the marginal reflex distance 1 (MRD 1) was 2 mm in the right lid and 3 mm in the left lid, and the bilateral levator muscle function was good. Anisocoria was present, and pupil size in a dark room was 2.5 mm in the right eye and 4 mm in the left eye. The patient complained of facial anhidrosis on the right side of the face. CONCLUSIONS: Although iatrogenic Horner's syndrome is rare complication of video-thoracoscopic surgery for primary pneumothorax, diagnosis after surgery of the thoracic cavity should be made carefully.

Atypical Miller-Fisher Syndrome Presenting as an Isolated Internal Ophthalmoplegia Following Epstein-Barr Virus Infection / 대한소아신경학회지

Acute external ophthalmoplegia, areflexia, and ataxia compose the classic Miller-Fisher syndrome (MFS). Pupillary dysfunction is present in half of patients with MFS. However, isolated internal ophthalmoplegia is rare in children with MFS. We report a case with MFS presenting with isolated internal ophthalmoplegia following an acute Epstein-Barr virus (EBV) infection. An 11-year-old girl with flu-like symptoms was diagnosed with having an acute EBV infection on prior admission. After discharge, she complained of anorexia, poor oral intake and dizziness associated with blurred vision when in the upright position. Physical examination on readmission 10 days later showed anisocoric, mydriatic pupils with an abnormal light reflex. The patient displayed no external ophthalmoplegia, ataxia or areflexia. Cerebrospinal fluid analysis showed elevated protein with no white blood cells. Serum anti-GQ1b antibody was negative. MFS was diagnosed under clinical findings and CSF albuminocytologic dissociation. Isolated internal ophthalmoplegia can be the sole manifestation of MFS. Therefore, MFS must be considered in the differential diagnosis for children presenting with unexplained acute internal ophthalmoplegia.

Isolated Oculomotor Nerve Palsy due to Direct Invasion of Recurrent Diffuse Large B-Cell Lymphoma

PURPOSE: Compared with multiple cranial nerve palsies, isolated nerve palsy is very rare in Non-Hodgkin's lymphoma. We experienced a case of isolated oculomotor nerve palsy as the first sign of recurrent diffuse large B-cell lymphoma (DLBCL). CASE SUMMARY: A 45-year-old woman visited the ophthalmology clinic with a one-month history of left upper eyelid ptosis and diplopia. She had a history of DLBCL of the nasopharynx and has been in complete remission for three years after systemic chemotherapy. Ophthalmologic evaluation showed ptosis of the upper eyelid, anisocoria, but no definite limitations in ocular movement. After six weeks, aggravated ptosis, exodeviation of the left eye in the primary position, and ocular movement limitations in all directions except abduction were observed. Brain magnetic resonance angiography taken four weeks earlier demonstrated no intracranial vascular lesion, but an enhancing lesion in the cistern along the left oculomotor nerve was shown in brain magnetic resonance imaging. We diagnosed isolated oculomotor nerve palsy due to direct invasion of recurrent DLBCL. Cerebrospinal fluid analysis revealed tumor cells, and follow-up MRI showed progression of lymphoma into the cavernous sinus. CONCLUSIONS: Although not common, isolated oculomotor nerve palsy can be the first sign of malignant lymphoma, even after complete remission.

Síndrome disautonómico de Ross: variaciones de un tema / Disautonomic Ross syndrome: variations about one theme

El síndrome de Ross fue descrito en 1958 como una afección degenerativa del sistema nervioso autónomo definido por la tríada de anhidrosis generalizada, disminución de los reflejos tendinosos y pupila tónica. Desde su descripción inicial se han descrito cerca de cuarenta casos. Comunicamos tres pacientes con variantes de interés que incluyen la presencia de espasmos cíclicos espontáneos del esfínter de iris, el desarrollo conjunto de síndrome de Holmes-Adie en un lado y síndrome Horner posganglionar en el otro, trastornos del desarrollo piloso en el lado de la anhidrosis, alteraciones de la motilidad intestinal, lengua sin papilas gustativas y disfunción sexual.

Ross Syndrome was described in 1958 as a degenerative condition of the autonomic nervous system defined by a triad of generalized anhidrosis, reduction of tendon reflexes and tonic pupil. Since its initial description about 40 cases have been described. We communicate three cases with variants of interest involving the presence of the simultaneous development of syndrome of Holmes-Adie on one side and Horner syndrome in the other, disorders of pilous follicle development on the side of anhidrosis, spontaneous disturbances of intestinal motility, tonque without papillae and sexual dysfunction.

Temporary Severe Neurogenic Blepharoptosis after Reconstruction of Orbital Medial Wall Fracture

PURPOSE: To report two cases of temporary severe neurogenic blepharoptosis after successful reconstruction of orbital medial wall fracture. CASE SUMMARY: A 36-year-old woman and a 52-year-old man received orbital medial wall reconstruction with Medpor(R) for large fractures. Before the operation, the patients had only moderate swelling of the lid and periorbital ecchymosis. There were no limitations of extraocular muscles or ptosis. The operations were successful, although the patients developed unilateral complete ptosis with totally impaired levetor muscle function immediately after recovering from anesthesia. There were no anisocoria or limitations of the extraocular muscles. After oral steroid therapy, the patients began to improve on postoperative day 4 and after one month, respectively, and recovered to normal lid height and levator function after two months. CONCLUSIONS: Blepharoptosis after orbital medial wall reconstruction may result from ischemic damage at the end of the superior branch of the oculomotor nerve in the orbit due to compressive and tractional manipulation. Although very rare and temporary, this complication should be considered important because the occurrence can be unpredictable and may cause dissatisfaction to the surgeon and the patient after a successful operation.

Spontaneous Intracranial Epidural Hematoma Originating from Dural Metastasis of Hepatocellular Carcinoma

Spontaneous intracranial epidural hematoma (EDH) due to dural metastasis of hepatocellular carcinoma is very rare. A 53-year-old male patient with hepatocellular carcinoma, who was admitted to the department of oncology, was referred to department of neurosurgery because of sudden mental deterioration to semicoma with papillary anisocoria and decerebrate rigidity after transarterial chemoembolization for hepatoma. Brain computed tomography (CT) revealed large amount of acute EDH with severe midline shifting. An emergent craniotomy and evacuation of EDH was performed. Active bleeding from middle cranial fossa floor was identified. There showed osteolytic change on the middle fossa floor with friable mass-like lesion spreading on the overlying dura suggesting metastasis. Pathological examination revealed anaplastic cells with sinusoidal arrangement which probably led to spontaneous hemorrhage and formation of EDH. As a rare cause of spontaneous EDH, dural metastasis from malignancy should be considered.

A Case of Waardenburg Syndrome Type 2 With Anisocoria

PURPOSE: To report a case of Type 2 Waardenburg syndrome with anisocoria. CASE SUMMARY: A 53-year-old woman with congenital deafness visited the clinic complaining of photophobia and heterochromic iris in the left eye. Her best-corrected visual acuity was 1.0 in the right eye and 0.7 in the left eye. The patient's left eye showed complete iris hypopigmentation, and the right eye showed segmental iris hypopigmentation. Fundus examination demonstrated generalized decrease in retinal pigment with focal hypopigmented lesions in both eyes. The pupil sizes were 3 mm in the right eye and 5 mm in the left eye. A pure tone audiogram showed profound bilateral sensorineural hearing loss. CONCLUSIONS: In the present case, mydriasis was found to exist in a completely hypopigmented iris. Therefore, the neural crest from which the pupillary constrictor muscle originates may play a role in the pathogenesis of Waardenburg syndrome.