ABSTRACT
Los linfangiomas orbitarios son malformaciones vasculares benignas, de crecimiento lento, abortivas y no funcionales, que se presentan principalmente en la primera década de la vida. Las opciones terapéuticas en estos casos presentan una resolutividad limitada, algunos tratamientos suelen ser agresivos y provocar daños del aparato visual. Se presenta un caso de una paciente femenina de 6 años de edad atendida por proptosis del ojo izquierdo a la que se le realizó el diagnóstico clínico-imagenológico de linfangioma de la órbita, con el objetivo de mostrar el resultado alcanzado en el manejo de la misma mediante el uso del sildenafilo por vía oral, modalidad terapéutica en estudio a nivel mundial en el tratamiento de estas afecciones. El tratamiento con sildenafilo en el linfangioma orbitario demostró ser eficaz en la mejoría del cuadro clínico y por imágenes. Durante el tratamiento no se reportaron reacciones adversas(AU)
Orbital lymphatic malformations are benign, slow-growing, abortive, nonfunctional vascular malformations that occur mainly in the first decade of life. Therapeutic options in these cases present limited resolution, some treatments are usually aggressive and cause damage to the visual apparatus. We present a case of a 6-year-old female patient treated for proptosis of the left eye. The clinical-imaging diagnosis of lymphangioma of the orbit was made to show the results achieved in its treatment through the use of oral sildenafil, a therapeutic modality under study worldwide in the treatment of these conditions. The treatment with sildenafil in orbital lymphangioma proved to be effective in the improvement of the clinical and imaging picture. No adverse reactions were reported during treatment(AU)
Subject(s)
Humans , Female , Child , Vascular Malformations/therapy , Lymphangioma/etiologyABSTRACT
PURPOSE: When there is a mass in the superior temporal orbit area, a lacrimal gland tumor should be suspected. We report a rare case of orbital lymphatic malformation that was histologically diagnosed in a patient with typical clinical features of the lacrimal gland. CASE SUMMARY: A 55-year-old female with no underlying disease and no ophthalmic history visited our clinic with a right upper eyelid edema associated with an enlarged painless eyelid mass 1 month prior to her visit. The patient stated that she discovered the mass 1 year previously.The palpebral lobe of the lacrimal gland protruded slightly with congestion of the surrounding conjunctiva. Enhanced computed tomography showed a 3 cm well-defined heterogeneous mass in the right lacrimal gland area and several well-defined round calcifications within the mass. Orbital tissue or bone involvement was not observed. The pleomorphic adenoma of the lacrimal gland was the most clinically suspicious, so complete resection of the mass was performed using lateral orbitotomy. Histopathologically, lymphangioma (lymphatic malformation) originating from the lacrimal gland was diagnosed. CONCLUSIONS: Orbital lymphatic malformation can occur in the lacrimal gland. The present case showed that differential diagnosis can reveal the presence of an adult lacrimal gland tumor.
Subject(s)
Adult , Female , Humans , Middle Aged , Adenoma, Pleomorphic , Conjunctiva , Diagnosis, Differential , Edema , Estrogens, Conjugated (USP) , Eyelids , Lacrimal Apparatus , Lymphangioma , OrbitABSTRACT
Introduction Lymphangioma is a rare congenital vascular malformation of the head and neck region isolated from the systemic circulation. It has a benign etiology, and represents 13% of all orbital tumors. These hamartomas often present in the pediatric population with a slightly female predilection. They have a lymphocytic composition, and may increase in size with episodes of viral infection, causing proptosis. Discussion The management of this lesion is controversial, hardly curative, and depends on the clinical presentation. The treatment options include partial surgical resection of the major cyst, needle aspiration, surgical debulking, systemic steroids, sildenafil, intralesional injection of the sclerosing agents, and local radiotherapy. Case Report In the present report, we describe an uncommon case of lymphangioma in a 6-year-old female who was first submitted to neurosurgery for tumor resection and received sildenafil therapy later, with promising results. Conclusion The treatment of orbital lymphangiomas remains a controversial topic, and the use of sildenafil along with needle aspiration and microsurgical removal is a viable option of treatment. However, many issues, such as the ideal duration of the therapy, the dosage regimen and the recurrence rate, still remain unclear. Our case report adds promising data on this pathology, even though larger trials are needed to properly elucidate the remaining questions.
Introdução Linfangioma é uma malformação vascular rara congênita da cabeça e da região cervical isolada da circulação sistêmica. Apresenta uma etiologia benigna e representa 13% de todos tumores orbitais. Estes hematomas geralmente se apresentam na população pediátrica com uma pequena predileção pela população feminina. Têm uma composição linfocítica e podem aumentar em tamanho com episódios de infecções virai causando proptose. Discussão O manejo destas lesões é controverso, dificilmente curativo e depende na apresentação clínica. Opções de tratamento compreende ressecção cirúrgica parcial do cisto de maior volume, aspiração por agulha de punção, redução cirúrgica, esteroides sistêmicos, Sildenafil, administração intralesional de agentes esclerosantes, e radioterapia local. Relato de Caso No presente relato, descrevemos um caso incomum de linfangioma em uma paciente feminina de seis anos de idade, inicialmente submetida a procedimento neurocirúrgica para ressecção tumoral, com posterior terapia com Sildenafil, apresentando resultados promissores. Conclusão O tratamento de linfangiomas de órbita permanece um tópico controverso, e o uso de Sildenafil em conjunto com aspiração por agulha de punção e ressecção microcirúrgica é uma opção viável de tratamento. Entretanto, muitas quesitos tais como tempo de terapia, regime de dose e taxa de recorrência permanecem incertos. Nosso relato de caso contribui com dados promissores referente a esta patologia, ainda que ensaios maiores são necessários para elucidação apropriada acerca das questões pendentes.
Subject(s)
Humans , Female , Child , Orbit , Lymphangioma , Lymphangioma/pathologyABSTRACT
El linfangioma es un tumor benigno raro y predominante en la infancia, debido a su crecimiento puede comprometer al órgano donde se desarrolla, se han propuesto varias opciones de trata-miento, sin embargo, la cirugía continúa siendo la primera opción. La neuronavegación permite realizar exéresis con gran precisión y de utilidad en cirugía ocular, por lo que disminuye el riesgo de secuelas después de una exéresis de linfangioma orbitario.
Lymphangioma is a benign tumor predominantly in childhood, due to growth that can compromise the organ where it grows. Several treatment options, have been proposed however,surgery remains the first choice. Neuronavigation allows successful excision and use in eye surgery,which decreases the risk of sequels following excision of orbital lymphangioma.
Subject(s)
Humans , Female , Child, Preschool , Orbital Neoplasms/surgery , Surgery, Computer-Assisted/methods , Neuronavigation/methods , Lymphangioma/surgery , Orbital Neoplasms/diagnostic imaging , Imaging, Three-Dimensional/methods , Lymphangioma/diagnostic imagingABSTRACT
El tratamiento del linfangioma orbitario es desafante. El sildenafl se ha propuesto como una opción terapéutca para pacientes con linfangiomas orbitariosirresecables.Se reporta el caso de una paciente de 10 años de edad quien presenta extrusión parcial del globo ocular derecho de curso progresivo rápido, asociado apérdida parcial de la visión, limitación de la movilidad y resistencia a la retropulsión del globo ocular ipsilateral diagnostcado como hemangiolinfangiomacerebral quirúrgicamente irresecable. Se inicia tratamiento con sildenafl a 40mg/día durante 12 semanas, basado en los datos de un estudio pilotorealizado en la Universidad de Stanford; evidenciando regresión marcada del proceso con mejoría clínica, estétca y radiológica.En el presente caso, el tratamiento tras 12 semanas con sildenafl disminuyó el crecimiento de la malformación linfátca. Se requieren estudios conmayor número de pacientes para determinar la efcacia de este tratamiento.
Treatment of orbital lymphangioma can be challenging. Sildenafl has been proposed as a therapeutc opton in patents with unresectable orbitallymphangiomas.We report a 10 year old patent with rapid partal extrusion of the right ocular globe associated with visual impairment whose orbital lymphangiomatreated with 40 mg of sildenafl improved afer 12 weeks.In this case report, treatment with sildenafl for 12 weeks improved surgically irresectable orbital lymphangioma. Larger clinical trials are needed toestablish efcacy of this treatment.
Subject(s)
Humans , Lymphangioma/diagnosis , Lymphangioma/drug therapy , Orbit/abnormalitiesABSTRACT
We report a case of significant unilateral left proptosis after a fall in a 10-year old child. Magnetic resonance imaging showed an extraconal hyperintense orbital mass extending into the maxillary sinus which was opacified. After drainage the proptosis resolved. The cause of the acute proptosis was haemorrhage within an orbital lymphangioma.
Se reporta el caso de una proptosis izquierda unilateral significativa en un niño de diez años de edad después de una caída. La tomografía por resonancia magnética mostró una masa orbitaria hiperintensa extracónica que se extendía hasta adentro del seno maxilar, opacificándolo. Después del drenaje, la proptosis se resolvió. La causa de la proptosis aguda fue la hemorragia dentro de un linfangioma orbitario.
Subject(s)
Child , Humans , Male , Lymphangioma/diagnosis , Orbital Neoplasms/diagnosis , Exophthalmos/etiology , Hemorrhage/complications , Magnetic Resonance ImagingABSTRACT
PURPOSE: To report the first domestic case of orbital lymphangioma treated with intralesional OK-432 injection. METHODS: Initial treatment with systemic corticosteroid had failed. After aspirating the blood, we injected OK-432 (0.05 mg) into the cystic space of the orbital lymphangioma. RESULTS: Although there were minor complications, such as fever and mild tenderness, the patient had an uneventful recovery. After 6 weeks, her visual axis was restored. CONCLUSIONS: We successfully treated this infant patient with an intralesional OK-432 injection. Based on this success, we suggest that treatment of orbital lymphangioma which is refractory to conservative treatment and difficult to resect, may include an intralesional OK-432 injection.
Subject(s)
Humans , Infant , Axis, Cervical Vertebra , Fever , Lymphangioma , Orbit , PicibanilABSTRACT
Orbital lymphangioma is a benign vascular hamartoma that may be found in the conjunctiva, eyelids, orbit, or elsewhere in the head and neck region. Deep lymphangiomatous lesions are classically characterized by acute onset of a fulminant proptosis resulting from spontaneous hemorrhage within the orbit. Complete surgical excision is often difficult because of the infiltrative nature of the tumor. Moreover, because of the hemorrhagic and friable nature of the tumor, conventional surgical techniques are frequently complicated by bleeding. We treated a case of pathologically-proven orbital lymphangioma with the ultrapulse CO2 laser. The ultrapulse CO2 laser permits a precise form of treatment with the ultimate form of hemostasis[tissue vaporization], resulting in minimal trauma, edema, and scarring, and thus offers a safe alternative to surgical excision of this lesion. The ultrapulse CO2 laser is an best ideal instrument for subtotal excision of this lesion because of its hemostatic cutting-vaporizing actions.