Tumoração retro-orbitária sugestiva de meningocele da bainha do nervo óptico / Retro-orbital tumor suggestive of optic nerve sheath meningocele

Meningocele da bainha do nervo óptico é uma condição extremamente rara, com poucos casos relatados na literatura. Exames de imagem revelam alargamento tubular-cístico do nervo óptico, com espessamento do mesmo. Os sintomas são muitas vezes relacionados com o comprometimento do nervo, ocasionando diminuição de lenta a acelerada da acuidade visual. O tratamento cirúrgico precoce por meio da descompressão da bainha do nervo óptico pode proporcionar melhora da função visual. Apresenta-se um caso de paciente com as características clínicas e radiológicas desta condição patológica rara. Paciente masculino, atendido no serviço com queixa de proptose do olho direito (OD) desde nascimento, com progressão nos últimos meses associada à dor. Melhor acuidade visual corrigida de conta dedos a 50 cm do OD. Olho esquerdo sem anormalidades. Ressonância Magnética de OD demonstrou formação expansiva cística de limites definidos em situação intraconal em órbita direita, em íntima relação com nervo óptico, determinando compressão, deformidade e deslocamento anterior do bulbo ocular, além de apresentar sinal semelhante ao líquor em todas as sequências obtidas. Suscitou-se hipótese diagnóstica de meningocele da bainha do nervo óptico direito e o paciente foi encaminhado para cirurgia descompressiva.

Meningocele of the optic nerve sheath is an extremely rare condition with a few cases reported in literature. Image studies reveal tubularcystic enlargement of the optic nerve although with the same thickness. Symptoms are often related to the involvement of the optic nerve, leading from slow to accelerated decreasing of the visual acuity. The early surgical treatment is the decompression of the optic nerve sheath, which it could provide improvement of visual function. We are presenting a case report of a patient who showed clinical and radiological signs of this rare pathological condition. Male patient attended at service complaining of proptosis of right eye (OD) since birth, in progress during the last months associated to stabbing pain. Best corrected visual acuity (BCVA) of OD was movements at 50 cm far; OS showed no abnormalities. Nuclear Magnetic Resonance of the OD showed expansive formation with cystic aspect, defined boundaries, located in an intraconal situation on the right orbit cavity and in a closing anatomical relationship to the optic nerve, inducing compression, deformity and anterior displacement of this eye besides presenting signal similar to spine liquor in all sequences obtained. The first hypothesis was meningocele of right optic nerve sheath. Then, patient was referred for surgical decompression.

Congenital proptosis secondary to orbital teratoma. Clinicopathological study.

CASE REPORT: A newborn male with right proptosis secondary to a retroocular mass. Ophthalmological examination also showed corneal ulcer and perforation, iris hernia, total ophthalmoplegia, chemosis and eyelid retraction. The histopathology diagnosis was mature teratoma. DISCUSSION: Teratomas are tumors composed of a mixture of mature tissues consisting of 3 germ layers. Congenital teratomas of the orbit are very rare and should be included as a possibility in cases with a primary tumor in the orbit.

[Orbital tumor--hemangiopericytoma--in a neonate]. / Guz oczodolu--haemangiopericytoma--u noworodka.

PURPOSE: To present a case of Haemangiopericytoma (HPC), a rare neopalsm which originates from the vascular pericytes. HPC occurs most commonly in adults. Only 5-10% of cases occur in children. Congenital orbital HPC is generally unknown in the field of ophthalmology. MATERIAL AND METHODS: A case of congenital, large exophthalmus is reported in a 1 day old male neonate. Imaging studies demonstrated a vascular, orbital mass involving skull base and cranial fossa. RESULTS: The diagnosis of HPC was established after histological exmination. Lesion did not qualify to surgical resection. The child was treated with chemotherapy for 10 months and a great regression of tumor was noted. There was no tumor recurrence during 4 years of a follow up. CONCLUSIONS: Chemotherapy may have a significant role in the treatment of infants with nonoperative malignant hemangiopericytoma.

Management of congenital unilateral proptosis from an orbital epithelial cyst by cyst aspiration.

A rare cause of axial proptosis, orbital epithelial cysts are presumed to be of conjunctival origin if the wall is composed of nonkeratinized squamous epithelium (especially if goblet cells are present). These cysts can occur at any age and are typically surgically resected if symptomatic. The subject of this report is the management of a congenital orbital epithelial cyst by needle aspiration in a newborn with ipsilateral esotropia and severe unilateral proptosis.

Congenitally enlarged extraocular muscles: can congenital thyroid eye disease exist in a euthyroid infant?

A 2-month-old boy presented with hypotropia, eyelid retraction, and proptosis of the left eye. CT and ultrasound demonstrated enlarged extraocular muscles. Both the infant and mother were euthyroid. The patient underwent inferior rectus recession, lower eyelid retractor disinsertion, and entropion repair. Biopsy of the inferior rectus and oblique muscles was normal. The clinical presentation and workup appear to be most consistent with thyroid eye disease, which, to our knowledge, would be the first reported case of euthyroid congenital thyroid eye disease with a euthyroid mother.

Congenital orbital sudoriferous cyst: radiological findings.

We report an extremely unusual case of a 4-month-old boy who presented with a sudoriferous gland cyst of the orbit. Congenital sudoriferous cyst is extremely rare in both the adult and pediatric populations. The CT and MRI findings are presented and the pertinent literature reviewed.

Congenital bilateral plexiform neurofibromas of the cavernous sinuses.

We report the CT and MRI findings of congenital bilateral plexiform neurofibromas of the cavernous sinuses in a 2-month-old girl. Contrast-enhanced CT showed enhancement of masses in both cavernous sinuses and enlargement of both superior orbital fissures. On MRI the masses were isointense with muscle on T1-weighted images, hypointense on T2-weighted images and showed strong homogeneous enhancement on contrast-enhanced T1-weighted images. A dural tail sign was observed. The diagnosis was proven by biopsy.

Subperiosteal orbital haematoma presenting as proptosis at birth.

We present a case report of a neonate with congenital subperiosteal orbital haematoma presenting with unilateral proptosis with review of current literature. The baby made an uneventful recovery following surgical drainage of the haematoma. This is the second report of orbital haematoma presenting at birth. Orbital haematoma should be considered in the newborn with unilateral proptosis. Early detection with computed tomography (CT) or magnetic resonance imaging (MRI) is required to determine the need for surgical drainage.

[Congenital intracranial teratoma with exophthalmos]. / Kongenitales intrakranielles Teratom mit Exophthalmus.

We report the rare case of a term newborn with an excessively large congenital, intracranial teratoma expanding into the right orbita and maxilla. The main symptoms were a total unilateral exophthalmos, a tumorous mass in the right cheek, macrocephaly with wide sutures, and a bulging fontanel. The diagnosis was confirmed by sonography, computed tomography and an exploratory excision from the retromaxillary region.

Congenital dacryocystocele with intranasal extension: correlation of computed tomography and magnetic resonance imaging.

A case of dacryocystocele with intranasal extension is described by magnetic resonance imaging (MRI) characteristics, which are correlated with the appearance on computed tomography (CT) scans. Computed tomography and MRI are equally sensitive in detecting dacryocystocele. Magnetic resonance imaging has the advantages of characterizing the cyst contents, allowing multiplanar imaging without the need for manipulation of the patient, and the absence of radiation exposure. Computed tomography has the advantage of detecting bone changes involving the bony nasolacrimal canal. Clinically, these abnormalities usually resolve spontaneously and only rarely require surgical intervention.

Unknown syndrome: microcephaly, hypoplastic nose, exophthalmos, gum hyperplasia, cleft palate, low set ears, and osteosclerosis.

We report a neonate, born at term, with microcephaly, a hypoplastic nose, exophthalmos, gum hyperplasia, cleft palate, and low set ears, who died at 86 minutes of age. Radiographs showed diffuse sclerosis of the bones.

A congenital orbital defect with periorbital-dural tissue adhesion.

This case demonstrates a previously unreported congenital orbital deformity. The patient was born with a unilateral exophthalmos. The etiology of the defect was demonstrated by CT scan as a convex bowing of the right superomedial orbital wall behind the axis of the eye. At surgery, this convexity was revealed to be a small bony defect through which the periorbital tissue and dura mater adhered. This sort of orbital deformity, although subtle, must be considered in the differential diagnosis of congenital unilateral exophthalmos.