Sven Holger Baum, Michael Oeverhaus, Franziska Saxe & Christopher Mohr
This study aims to analyse the various modifications of orbital exenteration.
Patients undergoing orbital exenteration from March 1978 to October 2019 were included in this retrospective study. The patients were evaluated on the basis of the indication, type of exenteration, reconstruction technique, overall survival (OS), and disease-free survival (DFS).
In total, 300 patients were enrolled in this study. As many as 24 patients had lid and conjunctiva sparing anterior exenteration, 16 had lid sparing anterior exenteration, 83 had anterior exenteration, 14 had lid and conjunctiva sparing total exenteration, seven had lid sparing total exenteration, 44 had total exenteration, one had lid and conjunctiva sparing extended exenteration, 23 had lid sparing extended exenteration, and 88 had extended exenteration. As many as 39 patients had a primary wound closure. Six patients underwent reconstruction with a split-thickness skin graft, 177 patients with a local or regional flap, and 40 patients with a microvascular flap. A total of 38 patients did not undergo reconstruction. The mean follow-up was 40 months (range 6–216 months). The OS rate was 82.2% after 1 year, 58.5% after 5 years, and 49% after 10 years for all patients with malignant tumours. The DFS rate was 67.7% after 1 year, 45.6% after 5 years, and 31.7% after 10 years.
Individual types of orbital exenteration allow patient-adapted therapies. The preservation of uninvolved orbital tissue facilitates orbital reconstruction. The type of exenteration did not have any influence on overall survival.
A. Martel, A. Oberic, A. Moulin, L. Zografos, L. Bellini, F. Almairac & M. Hamedani
To report our 14-year experience with orbital exenteration and assess risk factors for poor prognosis by focusing on conjunctival melanoma.
Patients and method
A retrospective study was conducted in our tertiary care centre (Jules Gonin Eye Hospital, Lausanne, Switzerland) between 2003 and 2017. Inclusion criteria were patients aged ≥18 years with a follow-up >12 months, without metastatic spread at the time of surgery. Data recorded were age, gender, tumour histology, surgical technique, postoperative complications, surgical margin status, local recurrence, postoperative radiation beam therapy and metastatic status.
Twenty-five patients with a mean age of 63.2 years (38–92) were included. Conjunctival melanoma was the most frequently identified tumour (n = 14, 56%) followed by conjunctival squamous cell carcinoma (n = 4, 16%), sebaceous carcinoma (n = 3, 12%), choroidal melanoma (n = 2, 8%) and basal cell carcinoma (n = 2, 8%). Eighteen tumours (72%) originated from the conjunctival tissue. Clear surgical margins were achieved in 21 (84%) patients. Fourteen (56%) patients experienced distant metastases and died from metastatic spread after a mean follow-up of 52.3 months (6–120). The 1-, 3- and 5-year overall survival (OS) was 96%, 72% and 60%, respectively. In the univariate analysis, positive surgical margins, local recurrence and metachronous metastases were associated with a decreased OS (p = 0.002, p = 0.005 and p = 0.007, respectively). In the multivariate analysis, positive surgical margins and metachronous metastases were also associated with a decreased OS (p = 0.02 and p = 0.042, respectively). Conjunctival melanoma was not associated with a poorer prognosis (p = 0.280).
Free surgical margins are needed to increase OS. To achieve clearer surgical margins, neoadjuvant targeted therapies/immunotherapies may be considered.
Thonnie Rose O. See, Gustav Stålhammar, Tina Tang, Joshua S. Manusow, David R. Jordan, Jeffrey A. Nerad, Robert C. Kersten, Marc Yonkers, Nasreen A. Syed, Seymour Brownstein, Hans E. Grossniklaus
Primary ductal adenocarcinoma (PDA) is a rare epithelial tumor of the lacrimal gland. Herein we report 5 cases and review 29 published cases of PDA of the lacrimal gland. Among these 5 cases, the most common clinical presentation was painless swelling and/or proptosis of their eye. The size of the lesions ranged from 1.6 to 2.5 cm. Histopathologic examination revealed proliferations of ductal or gland-like cells with vesiculated pleomorphic nuclei and prominent nucleoli. Tumor cells stained positive for epithelial and apocrine differentiation markers. Immunohistochemistry for human epidermal growth factor 2 was positive in 2 of the 4 cases. Four of the five patients were alive at the last follow-up visit. One died with bone metastases, which were diagnosed 25 months after exenteration and then survived an additional 51 months. On reviewing of twenty-nine previously published cases of PDA, the mean age of diagnosis was 58 years, with a male predominance (75%). Fifteen patients (54%) had distant metastases, 1 (4%) had local recurrence, and 10 (37%) suffered from a PDA-related death. PDA is a high-grade aggressive epithelial tumor of the lacrimal gland. Although rare, awareness and recognition of this malignancy are important to help determine prognosis and treatment options.
Ruiters, Sébastien; Mombaerts, Ilse
Purpose of review To comprehensively review the applications of advanced three-dimensional printing technology in the management of orbital abnormalities.
Recent findings Three-dimensional printing has added value in the preoperative planning and manufacturing of patient-specific implants and surgical guides in the reconstruction of orbital trauma, congenital defects and tumor resection. In view of the costs and time, it is reserved as strategy for large and complex craniofacial cases, in particular those including the bony contour. There is anecdotal evidence of a benefit of three-dimensional printing in the manufacturing of prostheses for the exenterated and anophthalmic socket, and in the fabrication of patient-specific boluses, applicators and shielding devices for orbital radiation therapy. In addition, three-dimensional printed healthy and diseased orbits as phantom tangible models may augment the teaching and learning process of orbital surgery.
Summary Three-dimensional printing allows precision treatment tailored to the unique orbital anatomy of the patient. Advancement in technology and further research are required to support its wider use in orbital clinical practice.
Sagiv, Oded; Ding, Stephanie; Ferrarotto, Renata; Glisson, Bonnie; Altan, Mehmet; Johnson, Faye; Elamin, Yasir; Thakar, Sudip D.; Nagarajan, Priyadharsini; Esmaeli, Bita
Purpose: To test the hypothesis that the US Food and Drug Administration approval of vismodegib in early 2012 has reduced the prevalence of orbital exenteration for locally advanced periocular basal cell carcinoma (BCC).
Methods: Following institutional review board approval, the authors reviewed clinical and pathological data of patients with locally advanced periocular BCC (T4 per the eyelid carcinoma classification in the 8th edition of the AJCC Cancer Staging Manual) treated by the senior author during 2006–2018. Patients were grouped into those who were treated before February 2012 (“before vismodegib approval”) and those who presented later (“after vismodegib approval”).
Results: Forty-two patients with locally advanced periocular BCC were treated during the study period, of whom 31 were men. The median age at presentation was 66 years (range, 43–90). Twenty-two patients had T4a and 20 had T4b tumors. Thirteen patients were treated before and 29 were treated after vismodegib approval. The 2 groups did not differ in age distribution (p = 0.164), sex distribution (p = 0.270), prevalence of recurrent tumor at presentation (p = 0.317), or duration of treatment with vismodegib (p = 0.605). Orbital exenteration was significantly more prevalent in patients treated before vismodegib approval than after (46% vs. 10%, p = 0.016), and vismodegib treatment was significantly more prevalent in patients treated after vismodegib approval than before (when vismodegib was given in clinical trials; 69% vs. 23%, p = 0.008). There was a trend toward more patients retaining their eyes at last follow-up in patients treated after vismodegib approval (83% vs. 54%, p = 0.066).
Conclusions: The prevalence of orbital exenteration as a necessary surgical procedure in patients with a locally advanced periocular BCC has fallen since the Food and Drug Administration approval of vismodegib. Although vismodegib is not specifically approved for organ-sparing, it has changed the authors’ practice and enabled eye preservation in patients with locally advanced periocular BCC, who would otherwise require an orbital exenteration.
Verona E. Botha, Kent T. K. Chow, Paul J. M. Salmon, Rami H. El-Khayat, Michelle T. Sun, Dinesh Selva & Stephen G. J. Ng
Desmoplasia is the formation of a dense collagenous stroma around a neoplasm. It occurs in a variety of malignancies including squamous cell carcinoma (SCC). While desmoplasia is uncommonly seen in cutaneous SCC, it is an independent risk factor for recurrence and metastasis. We report a case series of desmoplastic SCC in the periorbital region. Seven cases were identified: the median age was 68, four were men. The mean follow-up was 48 months. Two patients (29%) had aggressive local recurrence: one required salvage surgery including orbital exenteration, parotidectomy, and neck dissection to excise involved parotid and cervical lymph nodes; the other required repeat excision and adjuvant radiotherapy. Desmoplastic SCC is an uncommon but highly aggressive subtype. In the periorbital region, due to the high risk of orbital invasion, it is potentially sight and life-threatening.
Ebube E. Obi, David Saunders, Joyce Burns & Ragavan Sampath
No abstract available
Leon Rafailov, Roger E. Turbin & Paul D. Langer
To describe the novel use of a bilayer dermal substitute to reconstruct exenterated orbits. A retrospective chart review was performed in the practices of two surgeons (RET and PDL) of all patients who had undergone orbital exenteration between April 2014 and June 2016 and whose subsequent reconstruction included lining the socket with Integra bilayer. Patient demographics, pathologic diagnoses, surgical and post-operative complications, graft integrity, and patient acceptance were recorded. The charts of 7 patients (4 men and 3 women, ages 60–87 years) were reviewed. In all cases, the Integra graft had taken well to the socket bed at the time of silicone removal 3-4 weeks after surgery. Epithelialization of the socket occurred rapidly over the Integra graft (within several weeks) without incident in each case and with minimal postoperative management. No intraoperative or postoperative complications were noted. Integra dermal substitute is an ideal graft for the lining of an exenterated orbit. It is readily available in large quantities, handles easily, lines the socket smoothly, epithelializes rapidly, and requires minimal postoperative care. It offers minimal morbidity compared to skin grafting or free flap reconstruction, but greatly speeds epithelialization compared to laissez faire management. Surgeons should consider reconstructing exenterated orbits with the Integra matrix, both for its ease of use and its ability to epithelialize rapidly.
Himika Gupta, Deepa Nair, Aliasgar Moiyadi, Prathamesh Pai
Orbital exenteration is a destructive and disfiguring surgery and involves removal of the entire orbital contents, soft tissue and often lids as well. We report a case of an eight month old female, with malignant orbital teratoma who underwent lid sparing exenteration for the destructive, locally advanced disease. Three month post surgery she developed recurrence with intracranial extension as well as socket infection with pus discharge. Repeat surgery involved a multispeciality approach for removal of the tumor which was abutting the cavernous sinus posteriorly and ethmoid sinus medially, apart from filling the entire bony orbit. The focus of infection was found to be the retained lacrimal sac. The unhealthy lid skin also had to be sacrificed. The challenges in repeat exenteration of an 8 month old, and the utility of autologous dermis fat graft as a reconstructive option are discussed.
Elkhamary, Sahar M.; Galindo-Ferreiro, Alicia; Akaishi, Patricia; Muiños-Diaz, Yerena; Cechetti, Sheila P.; Cintra, Murilo B.; Cruz, Antonio Augusto V.
Purpose: To describe CT scan findings following orbital exenteration in 27 patients and to identify the factors involved in the development of post exenteration hyperostosis.
Methods: Noncomparative case series. The authors reviewed the charts of 27 patients ranging in age from 33 to 99 years, who underwent unilateral exenteration at King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia and at the School of Medicine of Ribeirão Preto, University of São Paulo, Brazil. Data regarding patient demographics, surgical procedure, clinical diagnosis, and preoperative and postoperative CT imaging of the orbits were obtained. The relationship between hyperostosis and postoperative time, gender, age, adjuvant radiotherapy, and cavity coverage was evaluated by multivariate stepwise logistic regression.
Results: Seventeen (73.9 %) orbits had postoperative orbital hyperostosis. No soft tissue masses were detected in the affected orbits except in 2 cases with tumor recurrence. The only factor associated with hyperostosis was immediate intraoperative socket rehabilitation (odds ratio = 0.13, 95% confidence interval: 0.01–0.89). There was an 87.0% lower chance of hyperostosis in patients whose socket was covered with musculocutaneous flaps. Sequential CT scans showed that orbital hyperostosis followed a specific pattern. Initially, bone thickening appeared as either uniform or undulating endo-osteal minimal thickening along the roof and then on the lateral and medial walls. More advanced hyperostosis had a laminated/lamellated appearance progressing to homogeneous and diffuse circumferential bone thickening. New bone formation and bone overgrowth were late findings. Hyperostosis extended to involve the adjacent facial bone, more obviously on the maxilla. Some patients had minimal thickening of the adjacent frontal and squamous temporal bone. Over-pneumatization of the paranasal sinuses was evident in all cases of hyperostosis.
Conclusions: Development of hyperostosis following exenteration is not rare. Radiologists and surgeons should be aware of the need to monitor the orbital healing process closely to avoid misdiagnoses of tumor recurrence/radionecrosis or infection. Obliteration of the orbital cavity with musculocutaneous flaps significantly reduces the chances of bone hyperostosis.