ABSTRACT Congenital arhinia-microphthalmos syndrome or BOSMA syndrome is an exceptionally rare clinical syndrome characterized by unilateral or bilateral complete absence of the nasal cavity associated with several craniofacial, ocular, and systemic anomalies. Lacrimal drainage anomalies are secondary to absent nasolacrimal duct and usually present as dilated lacrimal sac or mucoceles. While navigation-guided dacryocystorhinostomies into the contralateral nasal cavity are described for unilateral arhinia, the way forward for the complete absence of the nose and nasal cavity is still unclear. A multidisciplinary team from the specialties of genetics, plastic surgery, ophthalmic plastics and reconstructive surgery, otorhinolaryngology, and endocrinology should get involved very early on for better continuity of care.

ABSTRACT Disseminated intravascular coagulation (DIC) is characterized by abnormal activation of the coagulation cascade, which leads to simultaneous hypercoagulation and excessive bleeding. While it typically occurs in systemic diseases, such as infection, inflammation, obstetric complications, and malignancy, it can rarely manifest postoperatively. This case report describes a patient who presented with prolonged, refractory bleeding after ectropion repair via a lateral tarsal strip procedure. Due to the inability to control the patient’s bleeding with conservative measures followed by surgical exploration and electrocautery, the patient underwent a hematologic work-up. Laboratory studies were consistent with DIC, attributed to his large burden of endovascular stents. He was treated with anticoagulation using apixaban in addition to tranexamic acid to achieve lasting hemostasis. This case highlights the importance of thorough preoperative assessments, even for minor surgical procedures, and systemic workup for atypical postoperative bleeding.

ABSTRACT Purpose Description of clinical features, radiological characteristics, and management strategies in primary orbital intraosseous venous malformation (OIVM) with pertinent literature review. Methods A retrospective analysis including clinical, radiologic, operative, and histopathological data of six cases of histopathologically proven OIVM was done. A comprehensive literature review was conducted using online databases and augmented with manual search to identify reported cases of OIVM. Results Study data showed five females and one male in young to middle-age group, with an average age of 30 years (range: 20–48 years). Proptosis was noted in five cases (83.33%), and the duration of symptoms ranged from 6 months to 10 years. Frontal and zygomatic bones were most frequently affected and expansile bony lesion was the most common CT scan finding. Three patients underwent pre-operative embolization of feeders followed by en bloc excision of mass and surgical reconstruction (50%); one patient was managed with partial excision (16.66%) while two were regularly followed-up after incision biopsy (33.33%). Histopathology revealed vascular spaces with endothelial lining, separated by bony trabeculae in all patients. Follow-up periods ranged from 6 to 48 months and no recurrence or progression were noted. Conclusions OIVM is an exceptionally rare disorder with a gradually progressive benign course. Ophthalmologists need to be mindful of this entity during patient evaluation as it has propensity for large volume blood loss intra-operatively, owing to its vascular nature. Complete excision with reconstruction of resultant defect is the preferred treatment strategy and without known recurrence.

"Primary conjunctival amyloidosis presenting as upper lid ectropion." Orbit, ahead-of-print(ahead-of-print), p. 1 Disclosure statementNo potential conflict of interest was reported by the author(s).Additional informationFundingThe author(s) reported there is no funding associated with the work featured in this article.

ABSTRACT Purpose To review the literature on the location of the anterior ethmoidal foramen (AEF) and trauma during transconjunctival medial wall decompression. Methods A comprehensive literature search was conducted using the PubMed, Embase, and Scopus databases, combining the terms “olfactory fossa” and “fovea ethmoidalis” with “trauma,” “cerebrospinal fluid leak,” “pneumocephalus,” “orbital decompression,” and “anterior ethmoidal artery” (AEA). All cases of cranial base trauma during medial orbital decompression and the anatomical studies on the location of the AEF and the course of the AEA were reviewed. Results Ninety-four articles were identified, of which 37 were related to the AEF, 41 reported the course of the AEA, and 16 to reported cases of cranial base trauma. Out of these cases, 10 were related to transconjunctival medial orbital decompression, affecting 11 patients. Most AEFs are situated at the frontoethmoidal suture, but up to 38.15% of AEFs are located above the suture on the frontal bone. Most AEFs are adjacent to the roof of the ethmoidal sinus. The distance of the AEF to the cranial base increases in the presence of supraorbital ethmoidal cells (SOEC). Conclusions The position of the AEF is variable and should not be considered a safe landmark for all patients.

"A “tuck” in time saves nine." Orbit, ahead-of-print(ahead-of-print), p. 1 Disclosure statementThe authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.Additional informationFundingThe author(s) reported there is no funding associated with the work featured in this article.

ABSTRACT Paraneoplastic syndromes (PNSs) are remote effects of the primary tumor on tissues and organs, not related to direct invasion or metastasis. Ophthalmological involvement has been reported in 0.01–0.1% cases of PNSs. It may present as retinopathy, optic neuritis, myasthenia-like syndromes, or orbital myositis (OM), among others. An 89-year-old male with bilateral ocular pain and chemosis, was given an initial diagnosis of bilateral acute conjunctivitis. After 5 days, the patient presented worsening of the pain and bilateral complete ophthalmoplegia. Cranial CT scan showed diffuse bilateral thickening of the four rectus muscles. Inflammatory markers, thyroid hormones, and thyroid antibodies were normal. An abdominal ultrasound test was performed, observing a mass in the right kidney. After confirmation of the lesion with a CT scan, the radiological characteristics of the lesion were highly suggestive of renal cell carcinoma. Treatment with intravenous corticosteroids was ensued with complete resolution of all ophthalmological symptoms. Paraneoplastic orbital panmyositis was first described in 1994. Since then it has been reported scarcely, remaining an extremely rare entity. To our knowledge, this is the first report of its association with renal cell carcinoma. In the absence of inflammatory or dysthyroid blood markers, bilateral orbital panmyositis warrants further investigation for a possible underlying oncological pathology.

ABSTRACT Traditionally, for patients who are poor candidates for surgery and/or radiotherapy, palliative chemotherapy is often offered but with significant toxic side effects. However, recent advancements in our understanding of tumor biology and molecular genetics have brought new understanding to the molecular pathways of certain tumors and cancers. This has ushered in a new era of precision medicine specific to a tumor or cancer treatment pathway (targeted therapy) or directed to host-tumor responses (immunotherapy). This article will focus on recent updates in the application of available targeted and immunotherapy for managing orbital and periorbital tumors and tumor-like conditions, which include cutaneous basal cell carcinoma, cutaneous squamous cell carcinoma, cutaneous melanoma, Merkel cell carcinoma, sebaceous gland carcinoma, solitary fibrous tumor, dermatofibrosarcoma protuberans, orbital meningioma, neurofibromatosis, Langerhans cell histiocytosis, ocular adnexal lymphoma, orbital lymphatic malformation, and adenoid cystic carcinoma.

ABSTRACT We present a patient who presented with an orbital mass lesion which was a metastatic lesion from a porocarcinoma of the scalp with progressive deterioration of the patient. A 78-year-old male presented with functional decline and a rapidly growing scalp lesion of 3 months duration. In addition to the scalp lesion, Computed Tomography showed an incidental finding of a left lateral orbital wall tumour. Fine-needle aspiration of the two lesions revealed malignant cells with similar morphologies. Punch biopsy of the scalp lesion showed histological features suggestive of a porocarcinoma. Patient underwent palliative radiotherapy and immunotherapy and subsequently succumbed to the disease.