Usefulness of colour Doppler flow imaging in the management of lacrimal gland lesions.

OBJECTIVE: To assess the role of colour Doppler flow imaging (CDFI) in the diagnosis and management of lacrimal fossa lesions. METHODS: Institutional ethical committee approval was obtained. Fifty-one patients with 62 lacrimal fossa lesions were retrospectively included from 2003-2015. All patients underwent conventional ultrasonography and CDFI, with a qualitative and quantitative analysis of the vascularization. All patients had lacrimal gland surgery. Definitive diagnosis was based on pathological examination. RESULTS: The study included 47 non-epithelial lesions (NEL) and 15 epithelial lesions (EL), with 24 (39 %) malignant lesions and 38 (61 %) benign lesions. NEL were significantly more likely to present with septa (p < 0.001), hypoechogenicity (p < 0.001), high vascular intensity (p < 0.001), both central and peripheral vascularization (p < 0.001), tree-shape vascularization (p < 0.05) and a low resistance index (RI) (p < 0.0001). EL were significantly more likely to present with the presence of cysts (p < 0.001), and a higher RI. Receiver operating characteristic curves identified a RI value of 0.72 as the best cut-off to differentiate NEL from EL, with a sensitivity and specificity of 100 %. CONCLUSION: CDFI is a valuable tool in the differential diagnosis of lacrimal fossa lesions. Resistance index measurement enables substantial distinction between EL and NEL, thus providing crucial data for surgical management. KEY POINTS: • CDFI is a valuable tool in lacrimal fossa lesions. • Resistance Index measurement enables substantial distinction between epithelial and non-epithelial lesions. • Management of patients becomes more appropriate.

[Aneurysmal bone cyst: A rare cause of orbital disease]. / Kyste anévrysmal osseux : une atteinte exceptionnelle au niveau orbitaire.

Aneurysmal bone cyst is a rare benign bone neoplasm of unknown cause. The most commonly affected anatomical sites are the vertebral column and long bones. We report two uncommon cases of primary orbital aneurysmal bone cyst presenting as an acute orbital compartment syndrome due to subperiosteal hemorrhage. Case 1 is a 45-year-old woman. Imaging studies revealed a small cystic frontal bone tumour associated with a subperiosteal hematoma. The patient achieved full visual recovery after drainage of the hematoma, with no recurrence after treatment. Case 2 is a 74-year-old woman whose visual acuity was light perception due to severe papilledema. Imaging studies of the orbit revealed a large cystic frontal bone tumor associated with a subperiosteal hematoma causing globe and optic nerve compression. Preoperative arteriography showed a moderate vascular blush. Drainage of the hematoma was performed. A local recurrence with hematoma formation occurred two years after the surgery.

[Management of orbital cavernous hemangioma - evaluation of surgical approaches: report of 43 cases]. / Prise en charge des hémangiomes caverneux orbitaires - évaluation des voies d'abord chirurgicales: à propos de 43 cas.

Cavernous hemangioma is the most frequent benign orbital tumor in adults. The purpose of this study was to examine its clinical features, to define surgical indications, and to determine the roles of the various surgical approaches praticed in ophthalmology: transconjunctival (increasingly utilized), anterior transcutaneous, and lateral orbitotomy. The records of all patients treated for orbital cavernous hemangioma (OCH) since 2004 at the Fondation Rothschild (Paris, France) were retrospectively reviewed. Forty-three patients were treated for orbital cavernous hemangioma. Fifty-eight percent were women, mean age 50.2; 79 % of the tumors were intraconal. Among those patients, 36 underwent surgical removal, 5 were followed periodically, and 2 were lost to follow-up. The main surgical indications were: optic nerve compression (26 patients), proptosis (24 patients) and diplopia (3 patients). Transconjunctival, anterior transcutaneous and Kronlein approaches were used in 16, 12 and 4 patients respectively. Four patients had intrapalpebral hemangiomas easily reached transcutaneously. Two patients demonstrated transient partial 3rd nerve palsy (one with the lateral orbitotomy approach and one with the transconjunctival approach), one patient with the lateral orbitotomy approach developed a palsy of the superior branch of the 3rd nerve, and one patient with the transcutaneous anterior approach developed mydriasis. Surgical excision of OCH's is required in the presence of clinical complications. The transconjunctival approach is a safe technique which can lead to complete resection of the tumor in most cases.

[Evaluation of reconstructive techniques after orbital exenteration in 56 cases]. / Évaluation des techniques de reconstruction après exentération orbitaire. À propos de 56 cas.

PURPOSE: To describe the management of orbital exenterations and the surgical techniques for the reconstruction of orbital exenteration cavities. PATIENTS AND METHODS: This retrospective study includes 56 patients who underwent orbital exenteration between 2000 and 2009. Patients' age at the time of exenteration, diagnoses, complications and reconstructive techniques were studied. RESULTS: Fifty-six patients - 31 male and 25 female patients - who underwent orbital exenteration between 2000 to 2009 were included in the study. The mean age was 62.5 years at the time of exenteration. Average follow-up was 23 months. The principal diagnoses were basal cell carcinoma of the eyelids (25%) and conjunctival melanoma (20%). For orbital reconstruction, 18 patients (32%) had a muscle flap, and 24 patients (43%) underwent secondary reconstruction by bone-anchored implants after spontaneous epithelialisation of the orbit. Approximately 50% of the patients received postoperative radiation therapy. DISCUSSION: Surgical reconstruction using muscle flaps may mask recurrent tumor. Epithelialisation and bone-anchored implants supporting the prosthesis allow for recurrent cancer surveillance while providing better stabilization of the prosthesis. Radiation therapy seems to render the orbit more fragile and thus less stable for implants. CONCLUSION: Orbital exenteration is a mutilating technique. Rehabilitation techniques have been improved, in particular the bone-anchored implants which allow adaptation of the prosthesis with satisfactory cosmetic results.

[Surgical treatment of diffuse adult orbital lymphangioma: two case studies]. / Traitement chirurgical des lymphangiomes orbitaires diffus de l'adulte: à propos de deux cas.

PURPOSE: Orbital lymphangioma is a rare vascular malformation; it is a benign but severe anomaly because of its infiltrative, diffuse, and hemorrhagic nature, and its high morbidity rate. Surgical resection is a real challenge on account of the intricate architecture of the lesion. The authors report their surgical experience concerning two cases of diffuse orbital lymphangioma whose diagnosis was established in adulthood and whose surgical treatment was successful. CASE REPORTS: Two patients presented with adult orbital lymphangioma. Progression was slow during the first decade and then was quickly followed by complications: major exorbitism, compressive optic neuropathy, and corneal exposure. Neuroimaging showed a diffuse and cystic orbital malformation. Surgical resection was performed as completely as possible, in one case with a Krönlein orbitotomy and in the other case only via a conjunctive route. An aspirate drain was put in the orbit for 48 h so as to prevent dead spaces forming after resection, an essential risk factor of hemorrhagic or cystic recurrence. Systemic corticotherapy was administered for the 5 days following surgery. The resection was total in one case and subtotal in the other. The surgical follow-up was uneventful with an excellent aesthetic result and an improvement in visual acuity. After 12 months, no tumoral or hemorrhagic recurrence was noted. DISCUSSION: The surgical treatment of orbital lymphangiomas is challenging because of their infiltrative nature. In diffuse forms, a complete resection is rarely possible because of the risk of sacrificing visual function. In the two cases reported herein, the resection of the extraconal portion was complete, but the intraconal portion was completely removed only in one case. Using the aspirate drain, negative pressure was maintained in the orbital cavity, preventing the formation of chocolate cysts induced by surgery. Although the clinical result was very satisfying, long-term follow-up is necessary to evaluate recurrence. CONCLUSION: Diffuse orbital lymphangiomas can be treated successfully with a subtotal resection without clinical recurrence in the short term. These results are promising because they demonstrate that many orbital lymphangiomas can benefit from surgical treatment with an excellent esthetic and functional result.

Luteinizing hormone pulsatility in patients with major ovarian hyperandrogenism.

Hyperandrogenism and ovulatory dysfunction are common in women with either polycystic ovary (PCOS) or ovarian virilizing tumor. However, contrasting with the numerous studies that have extensively described gonadotropin secretory abnormalities, principally increased LH pulse amplitude and frequency, few studies have concerned gonadotropin secretion in patients with ovarian virilizing tumors; low gonadotropin levels have occasionally been reported, but never extensively studied. The goal of the present study was to further evaluate the pulsatility of LH secretion in women with ovarian virilizing tumor compared with that of PCOS patients. Eighteen women with major hyperandrogenism (plasma testosterone level >1.2 ng/ml) were studied (5 women with ovarian virilizing tumor, 13 women with PCOS, and 10 control women). Mean plasma LH level, LH pulse number and amplitude were dramatically low in patients with ovarian tumors when compared to both PCOS (p<0.001) and controls (p<0.001). In case of major hyperandrogenism, LH pulse pattern differs markedly between women with ovarian virilizing tumor or PCOS, suggesting different mechanisms of hypothalamic or pituitary feedback.

[Adenocarcinoma in a pleomorphic adenoma of the lacrimal gland: a case study]. / Adénocarcinome au sein d'un adénome pléomorphe de la glande lacrymale. A propos d'un cas.

PURPOSE: To report a patient with an adenocarcinoma in a pleomorphic adenoma of the lacrimal gland. Adenocarcinoma constitutes a distinct group of epithelial malignancies of the lacrimal gland. METHODS: The clinical presentation, workup, surgical approach, and pathological findings were reviewed. RESULTS: A 56-year-old man presented with a 3-month history of a lacrimal fossa mass. This patient presented a painless mass in the upper outer eyelid with significant displacement of the globe. The tumor was localized to the lacrimal gland. Radiological investigations showed a round, well-defined lesion in the fossa of the lacrimal gland. We performed a lateral orbitotomy; en bloc resection was accomplished. The final specimen showed an adenocarcinoma in a pleomorphic adenoma. No recurrences were detected during follow-up. CONCLUSION: Although adenocarcinoma has rarely been reported in association with a pleomorphic adenoma of the lacrimal gland, this combination can exist. If the malignant tumor is limited in the pleomorphic adenoma, the prognosis appears to be better than in cases of local extension. Adjuvant therapy, radiation or others, are not useful.

[Persistence of the pupillary membrane. Apropos of 3 cases]. / Persistance de la membrane pupillaire. A propos de 3 cas.

Persistent pupillary membrane has in most cases no functional consequence. Nevertheless, a thick membrane involving visual axis sometimes requires surgical removal. We describe three cases of this surgery with pathologic examination and functional results.

N-MYC amplification, loss of heterozygosity on the short arm of chromosome 1 and DNA ploidy in retinoblastoma.

Recurrent genetic alterations different from the alteration of the RB1 gene on chromosome 13q14 have been described in retinoblastoma, including structural alterations on the short arm of chromosome 1 and amplification of the N-MYC oncogene. These two genetic alterations are major prognostic factors in neuroblastoma, another embryonic neuro-ectodermal tumour. In order to assess the frequency of these alterations and their possible association with clinical parameters in retinoblastoma, we studied a series of 46 retinoblastoma tumour samples. Ploidy was assessed by flow cytometry, N-MYC copy number was evaluated by a spot-blot procedure using the pNb-1 probe and loss of heterozygosity was investigated by PCR analysis at mini- and microsatellites located on the short arm of chromosome 1. Most tumours were in the diploid or near diploid range; only one case exhibited tetraploidy. N-MYC amplification was observed in only one of the 45 tumours. Loss of heterozygosity on the short arm of chromosome 1 was observed in 9/43 tumours (21%); in particular, its incidence was higher in metastatic than in localised disease (P < 0.05). We suggest that alterations of one or several genes on chromosome 1p might play a role in the oncogenesis or progression of retinoblastoma. Analysis of the long term follow-up of these and additional patients should determine the prognostic value of this parameter.

Photorefractive keratectomy in 176 eyes: one year follow-up.

Beginning in March 1992, 176 eyes from 176 patients underwent photorefractive keratectomy with the Summit Technology Eximed UV200LA. This study was designed to evaluate the efficacy of this method.

An experience of the use of radioactive plaques after failure of external beam radiation in the treatment of retinoblastoma.

The authors have reviewed the results of radioactive plaques in recurrent retinoblastoma after external beam radiation in 34 eyes. They give their results on ocular conservation and on the visual outcome and compare these results with other series previously published. They think radioactive plaques are an alternative to enucleation in recurrent retinoblastoma after external beam.

[Eye manifestations and treatment of brain chordoma. Apropos of a case]. / Manifestations ophtalmologiques et traitement des chordomes intracrâniens. A propos d'une observations.

We report a case of intracranial chordoma revealed by an isolated abducent nerve palsy in a 4 years old boy. We discuss the possible symptoms of intracranial chordomas and their clinical, radiological and histological diagnosis as well as differential diagnosis. We summarize the available therapeutic modalities and we emphasize the future advantages of proton beam therapy in these lesions.

Recurrent ctb(7)(q31.3) and possible laminin involvement in a neonatal cutis laxa with a Marfan phenotype.

A 6-week-old girl presented with cutis laxa, emphysema, heart anomalies and a diaphragmatic hernia. She died at 22 weeks. A recurrent ctb(7)(q31.3) was found and the laminin gene was suspected to be involved in the disease. Anti-human laminin antiserum showed that this protein was absent from the skin. This case, together with 17 other similar cases, could represent a new type of connective tissue disease.