Treatment of orbital lymphatic malformation with oral sirolimus: a case report / Malformação linfática orbital tratada com sirolimo oral: relato de caso

ABSTRACT It is estimated that lymphatic malformations in children account for 6% of all benign vascular malformations. New medical therapies have been developed for the management of lymphatic orbital disease. The purpose of this article was to describe a clinical case of orbital venolymphatic malformation in a 10-year-old boy, causing proptosis and palpebral edema. The lesion was initially treated with local sclerotherapy. However, the lesion relapsed, and was successfully treated with oral sirolimus. Prospective studies are warranted to determine the appropriate dose and extend the indications of sirolimus in these patients.

RESUMO A incidência de malformações linfáticas em crianças é estimada em 6% de todas as malformações vasculares benignas. Têm sido desenvolvidos novos tratamento para doenças linfáticas orbitárias. Nosso objetivo é descrever um caso clínico de malformação venolinfática orbitária em um menino de 10 anos de idade, causando proptose e edema palpebral. A lesão foi tratada inicialmente com escleroterapia local. No entanto, a lesão teve recidiva e foi tratada com sucesso com sirolimo oral. Ainda são necessários estudos prospectivos para estabelecer a dose apropriada e a duração do tratamento com sirolimo nesses pacientes.

Ophthalmic manifestations of nasopharyngeal carcinoma (NPC): A systematic review of reported cases with and without prior history of nasopharyngeal carcinoma

ABSTRACT Purpose: We aimed to study reported cases of nasopharyngeal carcinoma presenting with ophthalmic manifestations with and without a prior diagnosis of nasopharyngeal carcinoma. Methods: We conducted a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). A literature search was conducted using the MEDLINE database in PubMed and Google Scholar. We included patients with a previous diagnosis of nasopharyngeal carcinoma in Group I and those without a prior diagnosis of nasopharyngeal carcinoma in Group II. Data included demographics, clinical presentation, history of nasopharyngeal carcinoma, treatment, histopathological description, World Health Organization classification, and outcome. Results: Fifty-eight patients (26 in Group I and 32 in Group II) were included. The male-to-female ratio was 3:1. The mean age of the patients (53.3 ± 11.7 years and 54.8 ± 16.2 years, respectively) and gender did not differ significantly between the two groups. The most common ocular presentations were diplopia and proptosis in the first group (each in 34.6%), whereas visual disturbance was most common in the second group (46.9%). Treatment options and World Health Organization grading were comparable. The outcome in 38 patients (after a comparable follow-up period) was significantly better in group II (p=0.003). There was no statistically significant difference in the outcome of 23 patients in correlation with World Health Organization grades II versus III irrespective of group (p=0.094). Conclusions: The demographics of patients with nasopharyngeal carcinoma presenting with ophthalmic manifestations were similar between the two study groups, with a wide age range and male predominance. Patients presenting initially to ophthalmologists with no history of nasopharyngeal carcinoma have a more favorable outcome. World Health Organization grading may have less value as a prognostic indicator.

Quality of life in patients with Graves' orbitopathy submitted to orbital decompression: comparison between balanced and inferomedial techniques

ABSTRACT Purpose: To compare inferomedial wall orbital decompression to balanced medial plus lateral wall orbital decompression in patients with Graves' orbitopathy in the inactive phase with regard to exophthalmos reduction and the effects on quality of life. Methods: Forty-two patients with inactive Graves' orbitopathy were randomly divided into two groups and submitted to one of two orbital decompression techniques: inferomedial wall orbital decompression or medial plus lateral wall orbital decompression. Preoperative and postoperative assessments included Hertel's exophthalmometry and a validated Graves' orbitopathy quality of life questionnaire. The results of the two groups were compared. Results: Compared to preoperative measurement, exophthalmos reduction was statistically significant in both groups (p<0.001) but more so in patients undergoing medial plus lateral wall orbital decompression (p=0.010). Neither orbital decompression techniques increased the visual functioning subscale score on the Graves' orbitopathy quality of life questionnaire (inferomedial wall orbital decompression p=0.362 and medial plus lateral wall orbital decompression p=0.727), but a statistically significant difference was observed in the score of the appearance subscale in patients submitted to medial plus lateral wall orbital decompression (p=0.006). Conclusions: Inferomedial wall orbital decompression is a good alternative for patients who do not require large exophthalmos reduction. However, medial plus lateral wall orbital decompression offers greater exophthalmos reduction and greater improvement in appearance (higher Graves' orbitopathy quality of life questionnaire scores), making it a suitable option for esthetic-functional rehabilitation.

Systemic sarcoidosis presenting as acute rapidly progressive proptosis / Sarcoidose sistêmica apresentando-se como proptose aguda rapidamente progressiva

ABSTRACT Sarcoidosis is a generalized systemic chronic inflammation that rarely involves the orbit. As a chronic inflammation, sarcoidosis typically manifests with an insidious onset and slowly progressive course. We report a case of acute-onset proptosis resulting from a rapidly growing diffuse orbital mass that simulated malignant growth, which was biopsy proven to be the first manifestation of systemic sarcoidosis. The patient demonstrated complete resolution of proptosis and systemic involvement with long-term corticosteroid treatment.

RESUMO A sarcoidose é uma inflamação sistêmica generalizada que raramente envolve a órbita. Sendo uma inflamação crônica, a sarcoidose geralmente se manifesta com um início insidioso e uma evolução lentamente progressiva. Neste artigo, é relatado um caso de proptose de início agudo, resultante de uma massa orbitária difusa de rápido crescimento, simulando um crescimento maligno, mas cuja biópsia comprovou ser a primeira manifestação de uma sarcoidose sistêmica. O paciente apresentou resolução completa da proptose e do envolvimento sistêmico com tratamento de longo prazo com corticosteroides.

Exoftalmos bilateral por compromiso orbitario debido a enfermedad relacionada con IgG4 / Bilateral exophthalmos due to orbital involvement due to IgG4-related disease

La enfermedad relacionada con IgG4 (ER-IgG4) se caracteriza por el compromiso fibrótico e infiltrado linfoplasmocitario con predominio de células IgG4 en uno o varios órganos. El fenotipo limitado a la cabeza y el cuello abarca el compromiso de las glándulas lagrimales y salivares, enfermedad orbitaria, paquimeningitis, hipofisitis, tiroiditis, mastoiditis y compromiso de senos paranasales. Las manifestaciones orbitarias pueden incluir proptosis uni o bilateral, miopatía cicatrizal de los músculos extraoculares, inflamación orbitaria y dolor que pueden simular a la oftalmopatía de Graves. La enfermedad oftálmica relacionada con IgG4 puede presentarse con compromiso de músculos extraoculares y de grasa orbitaria, y usualmente se confunde con afección orbitaria por enfermedad de Graves. Se presenta el caso de un varón de 58 años, con historia de exoftalmos bilateral de más de 15 años de evolución, en quien se arriba al diagnóstico de ER-IgG4 y cuya resonancia magnética de órbitas evidencia un patrón característico de compromiso de músculos extraoculares.

IgG4-related disease (IgG4-ER) is characterized by fibrotic involvement and lymphoplasmacytic infiltrate with IgG4 cells predominance in one or several tissues. The head and neck phenotype includes lacrimal and salivary gland involvement, orbital disease, pachymeningitis, hypophysitis, thyroiditis, mastoiditis, and sinus involvement. Orbital manifestations may include unilateral or bilateral proptosis, extraocular muscles scarring, orbital inflammation, and pain that may mimic Graves' ophthalmopathy. IgG4-related ophthalmic disease may present with extraocular muscles and orbital fat involvement and is often confused with orbital Graves' disease. We present the case of a 58-year-old man, with a history of bilateral exophthalmos of more than 15 years, in whom the diagnosis of IgG4-ER was reached and whose orbits magnetic resonance showed a characteristic pattern of extraocular muscle involvement.

Características de orbitopatía asociada al tiroides y tasa de requerimiento de cirugía / Thyroid-associated orbitopathy characteristics and surgery requirement rate

RESUMO Objetivo: Conheça as características demográficas e clínicas da Órbita Associada da Tiroide (OAT), bem como a taxa de exigência da cirurgia orbital em pacientes do Centro Médico Nacional do Oeste. Métodos. Estudo observacional, transversal, descritivo e retrospetivo realizado analisando os registos de pacientes diagnosticados com OAT tratados num centro de cuidados de terceiro nível de janeiro de 2005 a julho de 2016. Os resultados. Um total de 236 órbitas de 118 pacientes foram avaliados, com uma idade média de 47,3 (13,2 anos, 74,6% eram do sexo feminino e 25,4% masculinos. 4,2% dos doentes foram tratados com hipotiroidismo, 94,1% com hipertireoidismo e 1,7% com goiter tóxico difuso. 44,9% dos doentes estudados com restrição de movimento ocular,10,2% com queratopatia de exposição e 51,7% com hipertensão intraocular. 34,7% dos doentes avaliados no serviço necessitaram de descompressão orbital, 16,1% de cirurgia palpebral e 8,5% de correção do hatrabisma. Na gestão conservadora destes doentes, 48,3% exigiam o uso de lubrificantes tópicos dos olhos, enquanto 52,5% dos pacientes necessitavam do uso de hipotensivos oculares em número variável. As conclusões. A OAT foi associada principalmente ao hipertiroidismo, sendo mais comum em pacientes do sexo feminino entre os 40 e os 59 anos; mais de 50% dos pacientes necessitaram do uso de hipotensivos oculares. Da mesma forma, a gestão cirúrgica foi realizada em mais de 50% dos pacientes, sendo a descompressão orbital a intervenção mais frequente.

ABSTRACT Objective. To know the demographic and clinical characteristics of Thyroid Associated Orbitopathy (TAO), as well as the requirement rate of orbital surgery in patients of the Orbit Service in the National Medical Center of the West, IMSS. Methods. Observational, cross-cutting, descriptive and retrospective study carried out analyzing the records of patients diagnosed with TAO and treated at a third-level care center from January 2005 to July 2016. Results. A total of 236 orbits of 118 patients were valued, with an average age of 47.3 ± 13.2 years, 74.6% were female and 25.4% male. 4.2% of patients were treated with hypothyroidism, 94.1% with hyperthyroidism and 1.7% with diffuse toxic goiter. 44.9% of patients studied had eye movement restriction,10.2% exposure keratopathy and 51.7% intraocular hypertension. 34.7% of patients valued in the service required orbital decompression, 16.1% palpebral surgery and 8.5% strabism correction. In the conservative management of these patients 48.3% required the use of topical eye lubricants, while 52.5% required the use of eye hypotensives in variable numbers. Conclusions. TAO was mainly associated with hyperthyroidism, being more common in female patients between the age of 40 and 59; more than 50% of patients required the use of eye hypotensives. Likewise, surgical management was performed in more than 50% of patients, with orbital decompression being the most frequent intervention.

Clinical features of ophthalmology in thyroid disease / Шинэ санаа Шинэ нээлт

Background@#TED (thyroid eye disease) is an inflammatory disease of the orbit caused by autoimmune diseases of the thyroid, which adversely affect the vision, appearance, and quality of life. Exophthalmos and eyelid retraction are the main features of TED, which can lead to ocular motility, diplopia, optic neuropathy, and permanent vision loss. The study aims to determine the most common clinical signs of TED in Mongolians and define whether there is a correlation with the levels of thyroid autoantibodies.@*Methods@#The study involved 102 patients with TED and 81 patients with Graves’ ophthalmopathy. The clinical features of TED were identified and evaluated by activity score (CAS) and severity of GO using the European Group of Graves’ Orbitopathy (EUGOGO).@*Results@#The mean age of TED patients was 42.6±11.2, which was younger than GD patients (P=0.012). The current smoker was 24 patients (23.5%) with TED, which is relatively higher than GD (P=0.0001). The most common ocular signs were eyelid retraction 80 (78.4%), proptosis 77 (75.5%), diplopia 14 (13.7%) and 4% vision loss. There were no differences in proptosis between the right (18 mm, median) and left eye (17.8 mm, median) (P>0.05). The mean CAS score was 3.09±1.72 and varied depending on gender and smoking. According to EUGOGO, 62.7% of the patients were moderately severe. Only 7 % of the patients were in the sight-threatening stage, presenting optic neuropathy and corneal breakdown. The mean TSI level in patients with TED was 37.95 ± 35.41 IU / ml, which was 2.7 times higher than the mean in patients with GD.@*Conclusions@#Eyelid retraction and exophthalmos are the most common clinical signs of TED. Early diagnosis of these features can prevent complications of the disease. Determining serum TSI levels will help in the treatment and monitoring of TED.

Ocular motility changes after inferomedial wall and balanced medial plus lateral wall orbital decompression in Graves' orbitopathy: a randomized prospective comparative study

OBJECTIVES: To compare the surgical outcomes of inferomedial wall orbital decompression (IM-OD) and balanced medial plus lateral wall orbital decompression (ML-OD) in patients with inactive Graves' orbitopathy (GO) with regard to exophthalmos reduction and ocular motility abnormalities. METHODS: Forty-two patients with inactive GO eligible for OD were randomly assigned to either the IM-OD or ML-OD groups. Pre and postoperative evaluations included Hertel exophthalmometry, sensory, and motor extraocular motility assessment, standardized photographs in the nine gaze positions, and computed tomography (CT) of the orbits. ClinicalTrials.gov: NCT03278964. RESULTS: Exophthalmometry reduction was statistically significant in both groups (p<0.001), but was greater in the ML-OD group (p=0.010). New-onset esotropia occurred in 11.1% and 23.5% of patients who underwent IM-OD and ML-OD, respectively, with no statistically significant difference in the frequency of pre and postoperative strabismus in either group. The mean increase in preoperative esotropia was 24±6.9 and 12±8.8 prism diopters in patients who underwent IM-OD and ML-OD, respectively. In the IM-OD group, abduction and elevation worsened at the first (p<0.05) and third (p<0.05) postoperative visits but were restored at 6 months. The versions did not change postoperatively with ML-OD. The preoperative CT-measured medial rectus muscle area predicted new-onset strabismus (p=0.023). Significant postoperative medial rectus muscle enlargement occurred in both groups (p<0.001). Restriction in elevation and abduction was significantly associated with enlarged inferior (p=0.007) and medial rectus muscle areas (p=0.002). CONCLUSIONS: IM-OD is as safe as ML-OD with regard to new-onset strabismus, and represents a good alternative for patients who do not require significant exophthalmos reduction. ML-OD offers greater exophthalmos reduction and smoother postoperative recovery. Patients with preoperative enlarged medial rectus muscle on CT are at risk for new-onset esotropia, and preoperative esotropia is likely to increase after OD.

Hematoma subperiosteal orbitário secundário a barotrauma em aviação civil / Orbital subperiosteal hematoma secondary to barotrauma in civil aviation

RESUMO Os diagnósticos diferenciais que compõem as proptoses agudas são, muitas vezes, desafiadores. A anamnese e o exame clínico exigem do oftalmologista atenção especial aos detalhes que permitem diferenciar quadros relativamente benignos e autolimitados de quadros que evoluirão com incapacidades permanentes. Relatamos o caso de uma paciente de 49 anos que, durante viagem de avião, apresentou dor ocular, hematoma periorbitário e proptose do olho esquerdo súbitos. Referia diplopia aguda incapacitante. Exames de tomografia e angiorressonância magnética confirmaram diagnóstico de sinusopatia do seio etmoidal esquerdo e hematoma subperiosteal da órbita esquerda, associado ao barotrauma. Apesar de raro, o diagnóstico de hematoma subperiosteal não traumático deve ser considerado diferencial em relação a proptoses agudas, sendo a anamnese fundamental para essa elucidação diagnóstica.

ABSTRACT Differential diagnoses of acute proptosis are often challenging. History and clinical examination require from ophthalmologists special attention to details, which make it possible to differentiate relatively benign and self-limited conditions from those that will progress to permanent disabilities. We report a 49-year-old female patient who had sudden eye pain, periorbital hematoma and proptosis of the left eye during a commercial flight. She also complained of disabling acute diplopia. Computed tomography and magnetic resonance angiography imaging confirmed the diagnosis of subperiosteal hematoma of the left orbit, associated with left ethmoid sinus disease. Although rare, non-traumatic subperiosteal hematoma should be considered in differential diagnoses of acute proptosis, and history taking is fundamental to elucidate the picture.

Meningioma primario intraóseo esfenotemporal: presentación de caso / Primary spheno-temporal intra-osseous meningioma: a case report

RESUMEN Fundamento: los meningiomas ectópicos, definidos como aquellos que no tienen ninguna conexión con la duramadre, poco frecuentes. Son una variante rara y representan cerca del uno por ciento de todos los meningiomas intracraneales. Objetivo: presentar una variante poco frecuente de un meningioma ectópico como causa de proptosis y oftalmoparesia encontrado en una paciente joven. Presentación del caso: paciente de 40 años de edad, que hace seis meses comenzó con dolor en el ojo derecho de moderada intensidad, aumento de volumen de la región frontorbitaria, disminución de la agudeza visual y visión doble. Los estudios de tomografía axial computarizada y resonancia magnética de cráneo y órbita mostraron lesión extraaxial a nivel de la pared lateral de la órbita con extensión extra e intraorbitaria con compresión de estructuras adyacentes que provocó desplazamiento anterior del globo ocular. Se realizó tratamiento quirúrgico con excéresis y el estudio histológico concluyó un meningioma meningotelial ectópico del hueso grado I. Conclusiones: los meningiomas ectópicos resultan poco frecuentes, el tratamiento quirúrgico con la resección total de la lesión es la elección para evitar recurrencias y pueden tener indicación de tratamiento oncológico complementario.

ABSTRACT Background: ectopic meningioma, defined as those that have no connection with the dura mater, are rare. They are a rare variant and represent approximately 1 % of all intracranial meningioma. Objective: to present a rare variant of an ectopic meningioma as a cause of proptosis and ophthalmoparesis found in a young patient. Case report: patient of 40 years of age, who 6 months ago began with pain in the right eye of moderate intensity, increased volume of the front-orbital region, decreased visual acuity and double vision. Computed tomography and MRI of the skull and orbit showed extra-axial lesion at the level of the lateral wall of the orbit with extra and intra-orbital extension with compression of adjacent structures that caused anterior displacement of the eyeball. Surgical treatment was performed with resection and the histological study concluded an ectopic meningotial meningioma of bone grade I. Conclusions: ectopic meningioma are infrequent, surgical treatment with total resection of the lesion is the choice to avoid recurrences and may have an indication of complementary oncological treatment.

Treatment of restrictive strabismus of thyroid associated ophthalmopathy by rectus muscle release and recession combined with orbital fat resection / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM:To investigate the clinical effect of rectus muscle release and recession combined with orbital fat resection on restrictive strabismus of thyroid associated ophthalmopathy.<p>METHODS: From March 2018 to June 2019, 27 cases of restrictive strabismus with thyroid associated ophthalmopathy were retrospectively analyzed, 34 eyes in total. Under general anesthesia, all patients underwent rectus muscle release and orbital fat resection. The range of orbital fat resection was selected according to the degree of the ophthalmologic process in the subnasal, subtemporal, superior nasal, or superior temporal quadrants.Inferior rectus recession was performed in 25 eyes, medial rectus recession in 5 eyes and superior rectus recession in 4 eyes. According to the degree of exophthalmos, he internal and external fat of inferior nasal, infratemporal, Supranasal or supratemporal quadrant muscles were excised respectively during the operation. According to the preoperative strabismus degree and the degree of contraction of external rectus muscle, the recession amount of rectus operation was designed,and the strength of passive rotation test was estimated intraoperatively. The amount of fat removed during the operation was designed according to the degree of protrusion before operation, and it was designed to remove about 1mL fat and retract the eyeball by 1mm.The postoperative follow-up time was once a week, once a month after 1mo, and irregular follow-up after 3mo. All patients were followed up for more than 6mo.<p>RESULTS: The amount of fat removed was 2.1-3.4mL, with an average of 2.6mL.The visual(LogMAR)acuity was 0-0.2 in the 1mo after operation, with an average of 0.11±0.16. The visual acuity was 0-0.3 in the 6mo after operation, with an average of 0.12±0.17.There was no significant difference in visual acuity before and after operation(<i>P</i>>0.05). The degree of eyeball protrusion was 13-16mm, with an average of 14.4±0.8mm, and the degree of corrected eyeball protrusion was 2-4mm, with an average of 2.4mm(<i>q</i>=10.737, <i>P</i><0.01). The degree of eyeball protrusion at 6mo after surgery was 13-15.5mm, with an average of 14.5±0.6mm, showing no significant difference from 1mo after surgery(<i>q</i>=0.624, <i>P</i>=0.173). 1mo after surgery, the strabismus was 2△-10△, with an average of 6.7△±2.3△, all of which were under correction(<i>q</i>=18.068, <i>P</i><0.01). Before surgery, all the patients had diplopia at the first sight, and the first diplopia disappeared one month after surgery. All patients showed significant improvement in compensatory head position. The strabismus(undercorrection)of the patients at 6mo after surgery was 0△-7△, with an average of 3.4△±1.2△, and the difference was statistically significant compared with 1mo after surgery(<i>q</i>=5.385, <i>P</i>=0.015). The patient had no diplopia at the first and lower transitory position. The mean intraocular pressure after surgery(17.12±1.89mmHg)was lower than that before surgery, and the difference between preoperative and postoperative intraocular pressure was statistically significant(<i>q</i>=4.258, <i>P</i>=0.018).The mean intraocular pressure at 6mo after surgery was 17.53±1.67mmHg, with no significant difference from 1mo after surgery(<i>q</i>=0.729, <i>P</i>=0.154).<p>CONCLUSION: Orbital fat resection can improve the protrusion of the eyeball, but has no significant influence on the surgical design and effect of restrictive strabismus. It is a kind of controllable operation scheme to treat the limited strabismus with exophthalmos of thyroid associated ophthalmopathy with rectus muscle release and recession combined with orbital fat resection.

Graves hyperthyroidism in pregnancy: a rare presentation

Hyperthyroidism in pregnancy is much less common occurring in 0.1-0.2% of women with Grave’s disease being the most common cause accounting for 90% of the cases. It is important to diagnose hyperthyroidism in pregnancy because fetal loss in untreated patients is high and may even be life threatening for the mother. We are presenting a case of 29 years old G3P2L1 who presented to our emergency with amenorrhea of 7 months and history of loose stools for the last 20 days. It was associated with generalized weakness. She also had history of palpitations, weight loss and sleep disturbances. She was a known case of hyperthyroidism for the past 1-2 years and was already taking anti-thyroid drugs. B/L exophthalmos was apparently present. Patient was severly anaemic with haemoglobin of 5.5gms/dl. Ultrasound showed fetal demise at 28weeks. Patient was given 3 units of blood transfusion and was induced with prostaglandins. She delivered a dead male baby weighing 1.2kgs. Her postpartum period was uneventful. Timely diagnosis of graves hyperthyroidism and its optimal treatment throughout pregnancy is vital in reducing maternal, fetal and neonatal complications.

Histiocitosis de células de Langerhans en su presentación focalizada en órbita. Revisión del tema a propósito de un caso / Langerhans cell histiocytosis in their orbitfocused presentation. Review of the topic regarding a case

Resumen Objetivo: Describir un caso de histocitosis de células de Langerhans en un paciente pediátrico. Método: Paciente de 4 años con proptosis, diplopía, fiebre y pérdida de agudeza visual, con imagen en tomografía computarizada evidente de masa orbitaria con erosión ósea. Se realizó biopsia excisional por craneotomía coronal, el resultado histopatológico fue histiocitosis de células de Langerhans. Resultados: El estudio anatomopatológico transoperatorio mostró una neoplasia de células pequeñas redondas y azules, compatible con rabdomiosarcoma. Las pruebas de inmunohistoquímica concluyeron el diagnóstico de histiocitosis de células de Langerhans. Conclusión: La histiocitosis de células de Langerhans es una enfermedad poco frecuente que requiere de una sospecha clínica y diagnóstico oportuno, adecuar el tratamiento y mejorar la sobrevida de los pacientes.

Abstract Objective: To describe a case of Langerhans cell histiocytosis in a pediatric patient. Method: A 4-year-old patient with proptosis, diplopia, fever and loss of visual acuity, with evident image in a computed tomography of orbital mass with bone erosion. An excisional biopsy was performed by coronal craniotomy. The histopathological result was Langerhans cell histiocytosis. Results: The anatomopathological study showed a neoplasm of small round and blue cells, compatible with Rhabdomyosarcoma. Immunohistochemical tests concluded the diagnosis of Langerhans cell histiocytosis. Conclusion: Langerhans cell histiocytosis is a rare disease that requires clinical suspicion and a timely diagnosis to adjust the treatment and improve the survival of patients.

Acupoint selection rules of hyperthyroidism and related exophthalmos treated with acupuncture / 中国针灸

To explore the clinical acupoint selection rules for hyperthyroidism and related exophthalmos treated with acupuncture. By taking "hyperthyroidism" "acupuncture and moxibustion" as keywords，literature regarding acupuncture for hyperthyroidism and related exophthalmos published was collected in the Chinese Journal Full-text Database (CNKI), VIP Database (VIP) and WANFANG database. The literature was organized, the database of acupuncture prescription was established and the characteristics and rules of acupoint selection were analyzed. A total of 46 papers were included, involving 89 acupoints, the frequency of acupoint application was 449 times. The most commonly used 6 acupoints for hyperthyroidism treated with acupuncture were Sanyinjiao (SP 6), Neiguan (PC 6), Zusanli (ST 36), Shuitu (ST 10), Hegu (LI 4), Taichong (LR 3). And the most meridians of acupoints were the stomach meridian and the pericardium meridian. The most commonly used 6 acupoints for hyperthyroidism related exophthalmos treated with acupuncture were Fengchi (GB 20), Shangtianzhu (Extra), Hegu (LI 4), Sanyinjiao (SP 6), Cuanzhu (BL 2), Yangbai (GB 14). And the most meridian of acupoints was the gallbladder meridian. The most commonly used specific acupoints for hyperthyroidism treated with acupuncture were crossing points, -source points and five- points. The most commonly used specific acupoints for hyperthyroidism related exophthalmos treated with acupuncture were crossing points, -source points and five- points. Acupuncture masters in modern times have achieved significant therapeutic effect in the treatment of hyperthyroidism，which has showed the principles of searching for the primary cause of disease in treatment and giving consideration to both the root cause and symptoms. But there is a lack of simple and effective treatment methods that can be rapidly promoted in clinical practice.

The Clinical Result of Medial Orbital Decompression in Patients with Thyroid-associated Orbitopathy

PURPOSE: To evaluate the clinical effects of medial orbital decompression in patients with thyroid orbitopathy. METHODS: Forty-three orbits of 28 patients who underwent medial orbital decompression for cosmetic purposes between January 2014 to January 2017 were retrospectively reviewed. Changes in visual acuity, intraocular pressure, exophthalmos, strabismus, and diplopia were checked before, 3 months, and 1 year after surgery. RESULTS: The average exophthalmos reduction was −2.99 ± 0.96 mm at postoperative 3 months and −3.07 ± 1.24 mm after 1 year (both, p < 0.001). In patients who underwent unilateral orbital decompression, the mean difference in exophthalmometry between the two eyes was significantly reduced from 3.06 ± 0.78 mm to 0.38 ± 0.44 mm after 3 months, and to 0.50 ± 0.46 mm after 1 year (p = 0.011 and p = 0.012, respectively). After surgery, the final postoperative intraocular pressure decreased significantly at postoperative 3 months and 1 year (both, p < 0.001). The mean preoperative horizontal deviation was 0.88 ± 4.85 prism diopters (PD) and 5.50 ± 6.74 PD at postoperative 3 months, which demonstrated significant esodeviation postoperatively (p = 0.007). Three patients had new onset esotropia (8.33%), but no surgical treatment was needed. CONCLUSIONS: Medial orbital decompression is a less invasive and safe surgical procedure for patients with asymmetric or mild thyroid-associated orbitopathy, which can be beneficial for reducing proptosis.

Paquimeningitis y glomerulonefritis crescéntica en vasculitis p-ANCA / Pachymeningitis and crescentic glomerulonephritis in p-ANCA vasculitis

Resumen Las vasculitis asociadas a anticuerpos contra el citoplasma del neutrófilo configuran patologías multisistémicas caracterizadas por afectar pequeños vasos. Se ha descrito compromiso de sistema nervioso central en 2-8% de los casos, rara vez al inicio del cuadro clínico. Este corresponde al caso de un hombre de 67 años de edad con vasculitis p-ANCA, quien debutó con proptosis, paquimeningitis y disfagia severa. Posteriormente deterioro renal, necesidad de hemodiálisis, ciclofosfamida y recambio plasmático terapéutico con lo cual alcanza criterios de remisión parcial y egresa de diálisis. Las vasculitis ANCA se relacionan con una amplia gama de signos y síntomas inespecíficos. La edad de aparición, creatinina inicial y requerimiento de diálisis, son factores pronósticos. El inicio oportuno del tratamiento es fundamental, requiriendo por tanto un alto índice de sospecha. (Acta Med Colomb 2018; 43: 107-110).

Abstract Vasculitis associated with antibodies against the neutrophil cytoplasm they form multisystemic pathologies characterized by affecting small vessels. Central nervous system involvement has been described in 2 - 8% of cases, rarely at the beginning of the clinical picture. This corresponds to the case of a 67-year-old man with p-ANCA vasculitis, who debuted with proptosis, pachymeningitis and severe dysphagia. Subsequently, renal deterioration, the need for hemodialysis, cyclophosphamide and therapeutic plasma exchange, with which it reaches criteria of partial remission and dialysis discharge. ANCA vasculitis is associated with a wide range of nonspecific signs and symptoms. The age of onset, initial creatinine and dialysis requirement are prognostic factors. The timely initiation of treatment is fundamental, requiring therefore a high index of suspicion. (Acta Med Colomb 2018; 43: 107-110).

Undifferentiated pleomorphic sarcoma of the orbital region: a case report / Sarcoma pleomórfico indiferenciado da região orbitária: relato de caso

ABSTRACT Undifferentiated pleomorphic sarcoma (UPS) is an extremely rare tumor that occurs in the head and neck region. Here, we report a unique case of a primary undifferentiated pleomorphic sarcoma in the orbital region. A 35-year-old woman presented with a progressive proptosis and periocular edema for 1 year. She had no previous history of surgery, skin malignancy, or radiation. Imaging tests showed an extraconal mass, not involving the muscles. The tumor was surgically removed and adjuvant radiotherapy was required after histological examination, which showed an undifferentiated pleomorphic sarcoma of the orbit. There was no recurrence after 1 year of follow-up. Though rare, undifferentiated pleomorphic sarcoma should be included in the differential diagnoses of orbital tumors.

RESUMO O sarcoma pleomórfico indiferenciado (SPI) é um tumor extremamente raro na região da cabeça e pescoço. Relatamos um caso de um sarcoma pleomórfico indiferenciado primário na região orbital. Uma mulher de 35 anos apresentou proptose progressiva e edema periocular há um ano. Ela não tinha histórico prévio de cirurgia, malignidade da pele ou radiação. Exames de imagem mostraram uma massa extraconal, poupando os músculos. O tumor foi removido cirurgicamente e foi necessária radioterapia adjuvante após o resultado histopatológico. O exame histológico demonstrou um sarcoma pleomórfico indiferenciado da órbita. Não houve recidiva após 1 ano de seguimento. Apesar de raro, o sarcoma pleomórfico indiferenciado deve ser incluído no diagnostico diferencial de qualquer tumor originado na órbita.

Presentación atípica de un prolactinoma gigante en un adolescente de 15 años / Atypical presentation of a giant prolactinoma in a 15-year-old boy

El prolactinoma gigante es un adenoma pituitario poco frecuente caracterizado por su gran invasión local. Se reporta el caso de un varón de 15 años de edad con dolor retroocular izquierdo y exoftalmos ipsilateral de 4 meses de evolución secundario a un tumor en la base del cráneo que invadía la órbita. Los estudios hormonales revelaron prolactina sérica de 6913,7 ng/ml (valor normal < 20), que confirmó el diagnóstico de prolactinoma gigante. El paciente inició un tratamiento con el agonista dopaminérgico cabergolina en dosis crecientes. Luego de 7 meses de seguimiento, la prolactina había descendido a 349,8 ng/ml y el volumen del tumor se redujo un 70%, sin efectos adversos al tratamiento. El paciente se encontraba asintomático y había reiniciado la pubertad. La rápida remisión de los síntomas sin necesidad de tratamientos invasores subraya la importancia de considerar el diagnóstico de prolactinoma entre los posibles diagnósticos diferenciales de tumor de la base del cráneo.

Giant prolactinomas are rare pituitary adenomas characterized by their great local invasion. In this paper, we report a 15-year-old male with left retro-ocular pain and ipsilateral exophthalmos of 4 months of evolution, secondary to a tumour in the base of the skull that invaded the orbit. Hormonal studies revealed serum prolactin of 6913,7 ng/ml (normal value < 20), confirming the diagnosis of giant prolactinoma. The patient started treatment with the dopaminergic agonist cabergoline in increasing doses. After 7 months of follow-up the prolactin had decreased to 349.8 ng/ml and the tumor volume was reduced by 70%, without presenting adverse effects to the treatment. The patient was asymptomatic and had restarted puberty. The rapid remission of symptoms without the need for invasive treatments underlines the importance of considering the diagnosis of prolactinoma among the possible differential diagnoses of tumor of the skull base.

Sphenoid greater wing dysplasia associated with neurofibromatosis type Ⅰ: a case report and review of the literature / 中华整形外科杂志

Objective@#To discuss the etiology, pathogenesis, clinical manifestation, diagnosis and therapy of sphenoid wing dysplasia(SWD) associated with neurofibromatosis type Ⅰ(NF-Ⅰ).@*Methods@#We retrospectively reviewed its clinical manifestations, imaging, surgical treatment, complications and postoperative outcome of one NF-Ⅰ patient with SWD.@*Results@#A 14 years-old girl presented with pulsating exophthalmos, loss of vision and café au lait spots. Radiological studies showed right-side orbital enlargement and complete absence of the greater wing of the sphenoid. Titanium mesh was tailored intraoperatively to close the defect as a barrier between the orbital cavity and the cranium and then covered by periosteum.The patient developed postoperative infectious which was controlled by after antibiotic treatment and proper drainage. Proptosis improved significantly after surgery within a month. Ocular pulsation subsided and clinical symptoms improved at 28-month follow-up.@*Conclusions@#Sphenoid greater wing dysplasia associated with neurofibromatosis type Ⅰ is a rare inherited autosomal dominant disorders. The treatment should be customized to each patient. Titanium mesh reconstruction is patients with symptomatic sphenoid dysplasia. It can correct the proptosis and pulsating exophthalmos without the risk of bone resorption and recurrence.However, high risk of infection is associated with the procedure.

Exophthalmos, low back pain and hypertension: a report of one case with literature review / 中华风湿病学杂志

Objective By analyzing the clinical manifestations and pathologic features of Erdheim-Chester's disease (ECD) to improve the recognition of the disease.Methods The clinical characteristics,diagnosis and treatment of a young male with ECD were reported and the related literature was reviewed.Results A previously healthy young male patient with bilateral exophthalmos,blurred vision of right eye,polyuria and hypertension without obvious causes for nine months were admitted into our hospital.He developed low back pain two months ago.Thoracic vertebra Magnetic resonance imaging (MRI) showed multiple nodules extending from the 2th-7th thoracic vertebrae intra-medullary.MRI of the brain showed multiple masseswith abnormal intensities within the retro-ocular intraconal muscle cone,sellar and cavernous sinus,maxillary sinus.Biopsy specimens from the right orbital lesion demonstrated proliferation of fibrous connective tissue and fat tissue infiltrating with lymphocytes,plasma cells,eosinophils,foam cells,spindle cells,and multinucleated giant cells accompanied by fat necrosis.Immunohistochemistry showed infiltrated lymphocytes stained positive for CD68,CD20,CD3,LCA and negative for CD1a,S-100 protein and langerin.The clinical symptoms of exophthalmos and low back pain relieved after treated with methylprednisolone and interferon-α.Conclusion Understanding the characteristics of ECD can help to make the correct diagnosis and treatment.