Secondary orbital exenteration for conjunctival melanoma: A study of 25 cases.

INTRODUCTION: The treatment of conjunctival melanoma is most often conservative, but exenteration is sometimes necessary in order to achieve local control of the disease. It can be performed as a primary procedure in cases of locally advanced disease or as a secondary procedure after one or more recurrences. No benefit to secondary exenteration on patient survival has been demonstrated to date for conjunctival melanoma, and it is generally considered a palliative procedure. PATIENTS AND METHODS: Single-center retrospective study performed in the ocular oncology department of the Institut Curie (Paris, France). We included all patients who underwent secondary orbital exenteration for conjunctival melanoma between January 2008 and January 2016. RESULTS: Twenty-five patients underwent secondary exenteration for conjunctival melanoma. The maximum number of local recurrences prior to exenteration was six. Metastases occurred in 11 patients after exenteration and were more common when there was a greater tumor thickness on histology, if the tumor had not been treated initially in an ocular oncology center, or if there had been a greater number of local recurrences before the secondary exenteration was performed. Seventy-five percent of patients developed metastases when the exenteration was performed after 5 or 6 local recurrences. CONCLUSION: This study suggests that early secondary exenteration (i.e. after a number of local recurrences less than or equal to 4) may reduce the occurrence of metastases (and therefore improve patient survival) in conjunctival melanoma. Thus, secondary exenteration might be a curative surgery in some patients with recurrent disease.

Anatomical and functional outcomes of transcanalicular diode laser assisted dacryocystorhinostomy as a primary procedure.

INTRODUCTION: The goal of this study was to assess the efficacy of transcanalicular diode laser assisted dacryocystorhinostomy (TDL-DCR) for the treatment of primary acquired nasolacrimal duct obstruction. MATERIAL AND METHODS: This was a single center retrospective study. We included all patients who underwent TDL-DCR between July 2015 and October 2017 for chronic epiphora or chronic dacryocystitis. All procedures were performed under local anesthesia with sedation. The anatomical and functional success rates were studied as a function of each operative indication. RESULTS: Thirty-seven patients were included (18 had chronic epiphora and 19 had chronic dacryocystitis). All but one patient had bicanalicular silicone stent intubation. Irrigation of the lacrimal system demonstrated postoperative patency in 78% of patients operated for chronic epiphora and 76% of those with chronic dacryocystitis. The functional success of the surgery (as defined by the Munk score) was higher if the operative indication was chronic dacryocystitis (62%) (whereas it was only 41% for the indication of chronic epiphora). CONCLUSION: TDL-DCR has a lower anatomical and functional success rate than those reported in the literature for external or endonasal dacryocystorhinostomy. However, it retains a place in the treatment of primary acquired nasolacrimal duct obstruction (in particular when it is complicated by chronic dacryocystitis), especially if there is an absolute or relative contraindication to the two other dacryocystorhinostomy techniques.

Lacrimal Puncta Agenesis in Kabuki Syndrome.

Kabuki syndrome (KS) is a rare congenital disorder characterized by multiple systemic anomalies and facial characteristics. Here, the authors present the first case, to the best of the authors' knowledge, of bilateral lacrimal puncta agenesis in a patient with KS.#8232;The proband patient was a 29-year-old woman diagnosed with this syndrome, brought to our office due to recurrent conjunctivitis where agenesia of lacrimal puncta was observed. Therapeutic options were exposed but, as the concomitant medication (topiramate) produced ocular dryness, conservative treatment was decided. Diagnosis of KS is challenging because it is a complex syndrome with many associated findings. The authors recommend taking into account the agenesis of lacrimal points in the differential diagnosis of KS if it is associated with other phenotypic alterations as well as including lacrimal examination in patients with KS diagnosis. The authors emphasize the importance of individualizing treatment since drugs used for the systematic management of these patients can influence tear symptoms.

Basal cell carcinomas of the eyelid: Results of an initial surgical management.

PURPOSE: Our objective was to assess the results of surgical management of palpebral basal cell carcinomas (BCC) followed by a second line treatment discussed during a Multidisciplinary Team Meeting (MTM). MATERIALS AND METHODS: This retrospective single-centred study includes all surgically-treated basal cell carcinomas of the eyelids between January 2005 and January 2015. After initial surgery, the cases were systematically discussed during a multidisciplinary team meeting in order to assess the need for additional treatment. Data relative to the patient, tumor and management were pulled from the medical record. RESULTS: A total of 171 patients were included, with a mean age of 74 years. Among the patients, 151 underwent pentagonal resection of the tumor, and 20 patients had a superficial excision. After surgical management, 120 patients (70.2%) were considered to have sufficient free margins. The other 51 patients (29.8%) had insufficient margins due to remaining tumor cells (38 patients) or free margins less than 1mm. Among these 51 patients with insufficient margins, 19 received a second surgical treatment, 17 patients received adjuvant radiotherapy, and 15 were followed closely with an intensive biannual follow-up program. No patients were lost to follow-up. With a mean follow-up of 42 months (min. 6 months-max. 128 months), 7 out of 171 patients (4.1%) developed a local recurrence. The mean time between surgical management and recurrence was 24 months. The recurrence rate was higher for the group of patients with a recurrent tumor (11.6%) than for the group of patients referred for initial management (2.8%). Incomplete resection was also associated with a higher recurrence rate (3 recurrences out of 51 patients). DISCUSSION: The management of basal cell carcinomas of the eyelid is first and foremost surgical with the goal of complete resection confirmed by histopathological analysis. The histological analyses (Mohs micrographic surgery, frozen section technic, paraffin fixation) and recommended sizes of the margins can vary in the literature, with recurrence rates from 1.8% to 9.5%. CONCLUSION: In our experience, multidisciplinary management of BCC of the eyelid, including initial macroscopic surgery, histopathological analysis stating the histological type and size of the margins, along with additional treatment discussed in a MTM, allows for a recurrence rate of 4.1%.

Outcomes after surgical resection of lower eyelid tumors and reconstruction using a nasal chondromucosal graft and an upper eyelid myocutaneous flap.

INTRODUCTION: Surgical excision of large malignant lower eyelid tumors may cause important full-thickness eyelid defects. The reconstruction of such defects must restore the physiologic function of the eyelid and also re-establish an acceptable aesthetic result. MATERIALS AND METHODS: We report the outcomes of full-thickness excision of tumors extending over half of the horizontal lid length, followed by reconstruction using a nasal chondromucosal graft (coming from the ipsilateral ala of the nose) and an upper eyelid myocutaneous flap. Histological analysis of the specimen identified the tumor type and surgical margins for each patient. RESULTS: A total of 25 patients were operated using this reconstruction technique between March 2009 and June 2015: 17 basal cell carcinomas, 3 spindle cell carcinomas and 5 conjunctival melanomas (out of which 2 were associated with lentigo maligna). Mean duration of follow-up after surgery was respectively 36, 41 and 17 months for each of these 3 tumor types. We found a single local tumor recurrence and this was a basal cell carcinoma in a xeroderma pigmentosum patient. After surgery, none of the patients had lagophthalmos or ocular surface complications. Only 4 patients had a 1mm scleral show postoperatively; 3 other patients developed a small retraction of the eyelid after adjuvant radiotherapy and a 1mm scleral show occurred. CONCLUSION: In malignant tumors, complete surgical excision with histological margins adapted to tumor type prevents local recurrence in most cases. Our repair strategy of nasal chondromucosal graft and skin-muscle flap for large inferior eyelid defects provides good functional and aesthetic results.

Evisceration and ocular tumors: What are the consequences?

PURPOSE: Evisceration can be performed for blind, painful eyes. This surgery can promote the dissemination of tumor cells within the orbit if an ocular tumor has been missed preoperatively. METHODS: We reviewed the medical records of patients who were eviscerated for blind, painful eyes between 2009 and 2014 and who were referred after the surgery to the Institut Curie or the Rothschild Foundation in Paris. We included the patients with a histological diagnosis of ocular tumor or orbital recurrence. Cytogenetic analysis was performed whenever possible. RESULTS: Four patients turned out to have an ocular tumor after evisceration (two choroidal melanomas, a rhabdoid tumor and an adenocarcinoma of the retinal pigment epithelium); two had a history of prior ocular trauma. The tumors were diagnosed either on histological analysis of the intraocular contents (2 patients) or biopsy of orbital recurrence (2 patients). Prior to evisceration, fundus examination was not performed in 3 patients. One had preoperative imaging but no intraocular tumor was suspected. At the time of this study, 3 patients had had an orbital recurrence and died. We also found 2 patients who had an evisceration despite a past history of choroidal melanoma treated with proton beam therapy. CONCLUSION: We showed that evisceration of eyes with unsuspected ocular malignancies was associated with a poor prognosis due to orbital recurrence and metastasis. The evisceration specimen should therefore always be sent for histological analysis in order to perform prompt adjuvant orbital radiotherapy if an ocular tumor is found.

[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.