Sclerotherapy for Orbital Lymphangioma - Case Series and Literature Review.

Orbital lymphangioma is a lymphatic system lesion that commonly presents in childhood. Management of these lesions is complex. Sclerotherapy is a therapy used to treat and shrink lesions prior to or as an alternative to surgery. We present three cases of orbital lymphangioma that were treated with sclerotherapy. Case 1: A 6-month-old boy was presented in 2010 with right ptosis and proptosis. Magnetic resonance imaging (MRI) identified a lesion involving the right orbit and face. Case 2: A 3-year-old girl was presented in 2011 with intermittent right periorbital swelling and medial canthal bleeding. MRI identified a soft-tissue lesion in the right orbit, extending into the face. Case 3: A 3-year-old girl was presented in 2012 with vomiting, and painful right proptosis. MRI identified an intra-conal lesion in the right orbit with fluid filled levels. All three patients were treated with sclerotherapy (sodium tetradecylsulfate). Sclerotherapy is a promising treatment for orbital lymphangioma. Its use may prevent the need for, or minimise the amount of surgical management. Several sclerosants are now commonly used to treat these lesions.

An Unusual Case of Traumatic Internal Carotid Artery Dissection during Snowboarding.

The presentation of Horner's syndrome following blunt trauma is uncommon, but is of important clinical significance. Identification of the constellation of signs of Horner's syndrome should, therefore, prompt urgent neuro-radiologic imaging. Early diagnosis and initiation of appropriate treatment can lead to excellent outcomes in the majority of cases and prevent devastating cerebral ischaemic damage. A progressive case of Horner's syndrome following blunt injury to the neck in an amateur snowboarder is presented. Key pointsBlunt injury to the neck can result in Horner's syndrome.Horner's syndrome should alert clinicians to the possibility of a silent ICAD.MRI and MRA of the head and neck constitute the imaging modality of choice to look for ICAD.The treatment of choice for ICAD is anticoagulation for 3-6 months.

Multidisciplinary management of adult orbital rhabdomyosarcoma*.

We report the case of a 52-year-old man who presented with a 10-day history of right eye and eyelid inflammation and intermittent diplopia following blunt trauma to the right eyebrow. The CT and MRI scans revealed an extraconal soft tissue mass on the orbital floor with maxillary and ethmoid sinus wall destruction, which on orbital biopsy was proven to be an Alveolar Rhabdomyosarcoma. The patient had a central retinal vein occlusion due to mass effect that resulted in total visual loss at 2 months. He was referred to oncologists who treated him according to the paediatric RMS protocol and is still in remission at 2-year follow-up. Rhabdomyosarcoma is a rare tumour in adults which requires multi-disciplinary management. This highlights the necessity of considering rhabdomyosarcoma in the differential diagnosis of orbital tumours in any age group.

Repair of cicatricial ectropion in a harlequin baby.

The ichthyoses are a collection of scaling skin diseases or keratinizing skin disorders giving the appearance of "fish skin," of which harlequin ichthyosis is the most severe form. It is characterized by profound thickening of the keratin skin layer, armorlike scales that cover the body, and contraction abnormalities of the eyes, ears, and mouth. We report a case of a 6-week-old boy with harlequin ichthyosis and severe bilateral upper and lower eyelid cicatricial ectropion who underwent surgical repair with full-thickness postauricular skin autografts. To our knowledge, this is the youngest reported case and the only case of harlequin ichthyosis in which postauricular skin grafts were used.

A rare diagnosis of dedifferentiated liposarcoma of the orbit.

We report the case of a 56-year-old female who presented to the ophthalmic casualty department with proptosis and diplopia. Initially, non-specific orbital inflammation was suspected while investigations were ongoing. A trial of steroids failed to resolve the proptosis to a significant extent. A CT scan showed a well-defined orbital mass, which on orbital biopsy was shown to be a primary orbital liposarcoma of the dedifferentiated variety. This was confirmed by expert histopathological analysis. An exenteration followed by removal of orbital bone was required to achieve total tumour removal. Radiotherapy was delivered to reduce the chance of micro-metastasis. Orbital liposarcoma is an extremely rare tumour. The dedifferentiated variety is even rarer, with only a few reported cases. Lack of information about this potentially aggressive tumour may create therapeutic dilemmas as to the best treatment approach. Similarly, prediction of an accurate prognosis for the patient may be difficult due to the rarity of this condition.

Outcome of periocular basal cell carcinoma managed by overnight paraffin section.

BACKGROUND: Surgical excision of periocular skin cancer allows for optimum control in terms of tumour recurrence. Although Mohs' technique gives the best outcome, it is not widely available. Processing paraffin sections is slower but histologically superior to Mohs' frozen sections. We report the results of using a standard paraffin section (non-Mohs') technique to confirm histological clearance. METHOD: A retrospective study between 1/6/95 and 1/6/99 of all consecutive patients who had excision of periocular cancer was performed. All patients had surgical excision of the tumour with a 3-mm margin. Rapid (24-hour) paraffin sections were done and reconstruction performed if histological clearance was confirmed. If tumour was still present, a further 3-mm margin was excised at the appropriate edge(s) before reconstruction took place. RESULTS: This study yielded 93 basal cell carcinomas (BCCs) of which 86 were of primary origin and 7 were recurrent tumours. The tumour characteristics were as follows: 88% nodular BCCs, 82% had a maximum surface measurement less than or equal to 10 mm, 39.8% were inner canthal and 49.5% were localised to the lower lid. In the 30 (35.4%) cases that required further excision based on the initial histological reports, tumour was seen in only four (11.4%) cases. Overall histological clearance for primary BCCs was achieved in 81/86 (94.2%) cases. There was only one late recurrence at 4 years and 5 months. Among recurrent BCCs, there was one recurrence (12.5%) and this was despite histological clearance having been reported. CONCLUSION: For primary BCCs the recurrence rate in this study was low and comparable to that following Mohs' technique. This approach, using routine pathological facilities, therefore offers a viable technique for managing periocular BCCs.

Beware the empty curette!

BACKGROUND AND OBJECTIVE: Chalazions are a common occurrence in the eyelids due to chronic inflammation in the tarsal plate. Treatment of non-resolving cases may involve incision and curettage. Chalazions that are recurrent should arouse suspicion. We present a case of a pleomorphic adenoma of the palpebral portion of the lacrimal gland, which was misdiagnosed as a chalazion. PATIENT AND METHODS: A 41-year-old Caucasian lady initially presented with a swelling in the outer part of her right upper eyelid. A diagnosis of chalazion was made and two attempts at incision and curettage failed to resolve the lesion. She was then referred to the oculoplastics/adnexal unit. A more detailed examination revealed involvement of the palpebral part of her lacrimal gland. A CT-scan confirmed this and she proceeded to have a lateral orbitotomy to remove the tumour. RESULTS: Histological confirmation of a pleomorphic adenoma of the palpebral portion of the lacrimal gland was obtained. The lacrimal gland had been completely excised. CONCLUSION: Persistent or recurrent meibomian cyst should be treated with suspicion. A careful orbital examination including lid eversion should always be performed to outrule a more sinister pathology.

Enophthalmos associated with primary breast carcinoma.

BACKGROUND AND OBJECTIVE: The most common source of metastases to the orbit is from breast carcinoma. The orbital presentation can be the presenting sign of the cancer. Proptosis or exophthalmos is the more usual way metastases present, associated with ocular motility problems or diplopia. We present a case of enophthalmos associated with breast carcinoma whereby there is as yet no radiological evidence of an actual orbital metastatic lesion. PATIENTS AND METHODS: A 51-year-old Caucasian woman with a known history of breast cancer presented with a 10-month history of vertical diplopia, particularly on upgaze. She noted that her left eye was becoming more "sunken" and examination confirmed an 8-mm enophthalmos on that side with restricted vertical eye movements and abduction. RESULTS: A provisional diagnosis of metastatic breast cancer was made. CT and MRI scans were performed. The main feature noted was that of orbital fat atrophy with no evidence of an orbital mass. She has been followed up for a period of 2(1/2) years with 6-monthly scans. There has been no progression of her clinical signs and no orbital lesion found so far. We are continuing to review her. CONCLUSION: Although no actual orbital mass has been found yet, we are treating her case as enophthalmos associated with scirrhosing breast carcinoma; she may, however, have spontaneous orbital fat atrophy, triggered by the cancer.

An indeterminate malignant soft-tissue tumour treated by exenteration.

PURPOSE. To present a case of malignant soft-tissue tumour, which has yet to be categorised despite review by an international panel of experts, and which was treated by exenteration. PATIENT & METHODS. A 15-year-old Caucasian girl presented with progressive, non-axial proptosis and diplopia. CT scan showed an extraconal mass in the supero-medial part of the orbit suggestive of a vascular neoplasm. After initial incisional biopsy, the pathology of the tumour was felt to be malignant but so unique that further tissue was required for classification/categorisation and that exenteration was the best way to proceed. RESULTS. Despite there being an adequate amount of tissue and opinions being sought from international experts, including the Children(1)s Soft Tissue Panel (of the United Kingdom), this tumour has yet to be categorised. Reports have suggested it to be anything from a malignant desmoplastic round-cell tumour or a synovial sarcoma to an epithelioid haemangioendothelioma, which is our current working diagnosis. CONCLUSION. This case is unique in that it has not been possible to classify the tumour. Categorisation is important in order to give a reasonably accurate prognosis to the patient. Exenteration was required to offer complete tumour excision and will hopefully prevent local recurrence and metastatic spread.