A Clinical Update and Radiologic Review of Pediatric Orbital and Ocular Tumors

Rao, Ajay A.; Naheedy, John; Chen, James Y.-Y.; Robbins, Shira L.; Ramkumar, Hema L.

doi:10.1155/2013/975908

Cited by 99 publications

(163 citation statements)

References 86 publications

(149 reference statements)

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“…When the growth is outward into the eyelid, cysts pre sent in early childhood, and when they grow inward into the orbit, they present later in life. 21 A displacement of the globe could be found in some cases. Deep orbital dermoid cysts may present with proptosis and ocular motility disturbances or orbital nerve compression.…”

Section: Discussionmentioning

confidence: 98%

Ectopic cilia associated with an orbital dermoid cyst and sinus tract: case report

Krahulík¹,

Karhanová

Vaverka³

et al. 2015

PED

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Ectopic cilia are extremely rare congenital anomalies in which eyelash follicles appear in an abnormal place on the eyelid, most typically on the lateral quadrant of the anterior surface of the upper eyelid. In the majority of cases, simple surgical excision of ectopic cilia is indicated because of its cosmetic aspect. There is usually no associated medical co-morbidity with this anomaly. The authors report an unusual case of ectopic cilia associated with an orbital dermoid cyst and sinus tract. A 3-year-old boy was initially diagnosed with ectopic cilia on the left upper eyelid. There was no history of inflammation or swelling of the eyelid. An ophthalmological examination revealed only 1 mm of ptosis; no proptosis, inferior displacement, or palpable orbital mass was present. During surgical excision of the ectopic cilia, a thin sinus tract was identified, leading posteriorly to the orbit. Magnetic resonance imaging performed after the excision showed a supraorbital extraconal mass just below the roof of the left orbit. A supraorbital 2-piece craniotomy was performed with total extirpation of the dermoid cyst. The cyst was removed en bloc without damage to the extraocular muscles, but the sinus tract could no longer be identified. Follow-up MRI was performed 6 months after surgery and showed no evidence of recurrence. A follow-up ophthalmological examination showed no signs of inferior displacement or proptosis. To the best of the authors’ knowledge, this case is the first reported instance of ectopic cilia associated with a dermoid cyst and sinus tract in which no typical clinical signs and symptoms of possible orbital pathology were present. This case highlights the value of radiological examination in all cases of ectopic cilia prior to surgical excision.

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Section: Discussionmentioning

confidence: 98%

Ectopic cilia associated with an orbital dermoid cyst and sinus tract: case report

Krahulík¹,

Karhanová

Vaverka³

et al. 2015

PED

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“…Los estudios de imagen pueden ayudar a confirmar el diagnóstico en los que las lesiones tipo nodulares con calcificaciones son distintivas de este tipo de tumor, y pueden observase mediante tomografía computada o ultrasonido en 95% de los casos. 4 Por otro lado, la neoplasia de órbita más frecuente en pediatría es el rabdomiosarcoma. Dicha localización representa 10% de todos los rabdomiosarcomas, y tiene una edad media de presentación de 6 a 8 años.…”

Section: Comentario Clínicounclassified

“…Sin embargo, los hallazgos histológicos permitieron descartar esta entidad. 4 Una tercer posibilidad diagnóstica igualmente descartada fue la presencia de metástasis de tumor neuroectodérmico primitivo, este es un tumor de células pequeñas redondas y azules, de gran similitud al neuroblastoma, sin embargo, esta última entidad es casi exclusiva de menores de 5 años.…”

Section: Comentario Clínicounclassified

“…4 La enfermedad inflamatoria de la órbita puede estar asociada a condiciones sistémicas no oncológicas, principalmente enfermedades del sistema inmune y endocrinológico, en cuyo caso la presentación bilateral es más frecuen-te y suele acompañarse de hallazgos clínicos extra-oculares como fiebre, linfadenopatías, malestar general, nerviosismo, intolerancia al calor, pérdida de peso, cabello quebradizo, piel húmeda y caliente, taquicardia, temblor, mixedema peritibial, entre otros. 3 En este contexto se sugiere realizar biometría hemática completa, velocidad de sedimentación globular, proteína C reactiva, pruebas de función tiroidea, anticuerpos anti-tiroideos, anticuerpos anti-citoplasma del neutrófilo y examen general de orina.…”

Section: Comentario Clínicounclassified

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Adolescente femenino con granulomatosis de Wegener fulminante

et al. 2017

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Adolescente femenino de 13 años de edad, originaria de Guerrero, grupo étnico mixteco, padres analfabetos y con poco entendimiento del español. Sin otros antecedentes de importancia para el padecimiento. Inició un mes previo con la aparición de una pápula hipercrómica en párpado superior derecho que en los 10 días previos al ingreso aumentó de volumen generando proptosis. Al interrogatorio se negó dolor, lagrimeo, fiebre, o pérdida de peso. Ingresó con fiebre (39.0°C), palidez generalizada, proptosis derecha con exposición de córnea y conjuntiva, eritema periorbitario, movimientos oculares y agudeza visual disminuidos (i.e. contaba dedos a 1 m), pupila con tendencia a midriasis, hiperreactividad a la luz, y fondo de ojo con papila hiperémica edematosa y tortuosidad peripapilar. El resto de exploración no mostró datos relevantes. Se hospitalizó para iniciar tratamiento antimicrobiano parenteral de amplio espectro (i.e. ceftriaxona y vancomicina) y abordar proptosis unilateral.

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“…It accounts for approximately 4% of orbital masses in this age group. The mean age of diagnosis is 6-8 years; however, it is very rare at birth [1,2]. …”

Section: Introductionmentioning

confidence: 99%

Congenital Orbital Rhabdomyosarcoma

2017

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Rhabdomyosarcoma (RMS) is the most common malignant tumor of the orbit in children, but it is rarely present at birth. We report a large congenital orbital RMS with intracranial extension in a newborn baby. A newborn baby girl was referred to our hospital due to severe right-eye exophthalmia. Imaging studies showed an orbital mass with intracranial extension. Treatment was started with a course of neoadjuvant chemotherapy followed by right orbital exenteration and intracranial resection of the tumor. Histologic examination of the mass showed undifferentiated malignant small-cell tumor. Immunohistochemical study proved it to be RMS. The patient was categorized as intermediate-risk RMS and chemotherapy was continued accordingly with VAC regimen. In spite of treatment, the infant developed intracranial recurrence at the age of 6 months and died 1 month later. Congenital orbital RMS has a poor prognosis. Reconstruction surgery should be deferred due to high rate of recurrence and low chance of survival. A multidisciplinary approach might increase the survival of these patients.

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A Clinical Update and Radiologic Review of Pediatric Orbital and Ocular Tumors

Cited by 99 publications

References 86 publications

Ectopic cilia associated with an orbital dermoid cyst and sinus tract: case report

Ectopic cilia associated with an orbital dermoid cyst and sinus tract: case report

Adolescente femenino con granulomatosis de Wegener fulminante

Congenital Orbital Rhabdomyosarcoma

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