Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery最新文献

Eyelid xanthogranuloma is a rare non-Langerhans cell histiocytic disorder that typically occurs in children as juvenile xanthogranuloma or in adults as part of systemic diseases such as necrobiotic xanthogranuloma or adult -onset asthma with periocular xanthogranuloma. Isolated eyelid involvement without systemic association is distinctly uncommon. We report a 26-year- old immunocompetent male presenting with a solitary, firm, nodular swelling over the lateral right lower eyelid for three months. Magnetic resonance imaging (MRI) orbit revealed a well-defined subcutaneous lesion (10x 8 mm) without orbital extension. Complete surgical excision was performed and histopathology confirmed non- Langerhans cell xanthogranuloma. Systemic evaluation was unremarkable. The patient received a single postoperative intralesional corticosteroid injection to reduce recurrence risk and remained asymptomatic with no recurrence or systemic disease over one year of follow-up. This case highlights the importance of considering xanthogranuloma in the differential diagnosis of isolated eyelid masses in young adults and demonstrates excellent outcome with surgical excision and adjunctive intralesional steroid therapy. A thorough systemic evaluation is mandatory to exclude underlying systemic disease and to guide appropriate long- term surveillance.

Giant cell reparative granuloma (GCRG) is a rare, benign fibro-osseous lesion, with orbital involvement being particularly uncommon in young children. We describe a 4-year-old boy with progressive right orbital inferior dystopia and proptosis, without history of trauma, sinus disease, or systemic illness. Imaging showed a well-circumscribed superonasal orbital mass with osseous erosion. Through an upper conjunctival fornix approach combined with superior lateral cantholysis, the mass was debulked to preserve vital orbital structures. The lesion appeared highly vascular and yellowish-brown intraoperatively, and histopathology confirmed GCRG. Postoperatively, a short course of low-dose oral corticosteroids was given. The patient achieved complete resolution with no recurrence at 22-month follow-up. This case represents one of the youngest reported patients with isolated orbital GCRG and demonstrates that conservative, function-preserving surgery with adjuvant corticosteroids can be effective. The favorable outcome supports a possible inflammatory component in its pathogenesis and suggests that radical excision may not be necessary in all pediatric cases.

A 51-year-old male presented with a rapidly growing right caruncular nodular lesion. Excisional biopsy performed elsewhere showed an apocrine adenocarcinoma of the caruncle with positive margins. Clinically, there was no obvious residual disease, mapping biopsies were negative, and cryotherapy of the surgical base was performed.The tumor recurred both anteriorly in the lower fornix and deeper in the anterior orbit a year later. These were surgically excised with adjuvant radiotherapy (60 Gy in 30 fractions). Unfortunately, the tumor continued to recur posteriorly to progressively first involve optic canal and cavernous sinus, then planus sphenoidale (with loss of right eye vision), and then intracranially with each recurrence treated with stereotactic radiosurgery. Six years from last treatment, the latest MRI revealed the involvement of Meckel's cave and optic chiasm, which is now being treated with hormonal treatment. This is the first case of invasive apocrine adenocarcinoma of the caruncle with intracranial extension treated multimodally with surgery, radiotherapy, repeated stereotactic radiosurgery, and hormone therapy.

Congenital oculopalpebral synkinesis is a rare phenomenon in which specific ocular movements induce paradoxical eyelid responses. Most reported cases involve excitatory forms, whereas inhibitory variants are distinctly uncommon. We present a 15-year-old boy with congenital right ptosis who demonstrated bilateral gaze-evoked synkinetic eyelid movements. In addition, paradoxical right upper lid retraction was observed on downgaze, and vertical lid twitching was evident in upgaze and lateral gazes. Ocular motility and pupillary reactions were normal, and no jaw-winking was present.

Pilomatrix carcinoma is a rare, low-grade malignant tumour of the hair follicle matrix, often misdiagnosed due to its histologic similarity to other more common basaloid epithelioid tumours. While it predominantly affects the head and neck region, periorbital involvement is exceptionally rare, with only five reported cases. We present a novel case of pilomatrix carcinoma in the right lower eyelid, representing the sixth documented periorbital occurrence. An 85-year-old male presented with a painless, rapidly enlarging mass on the right lower eyelid, accompanied by red discoloration and ectropion. Examination revealed a firm, nodular medial lower lid margin lesion measuring 3 × 2 × 4 mm. An excision of the lesion with lid reconstruction was performed, and histopathology confirmed the lesion as pilomatrix carcinoma with clear margins. This case highlights the importance of considering pilomatrix carcinoma in atypical periorbital lesions.

An unusual case of bilateral upper eyelid symblepharon after routine ptosis repair uncovered occult Sjogren's syndrome, underscoring the need for thorough pre-surgical evaluation in all patients and systemic workups for post-operative cicatrization.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder characterized by progressive demyelination of peripheral nerves, leading to motor and sensory deficits. Ophthalmologic involvement, though uncommon, may manifest as ophthalmoplegia, papilledema, optic neuropathy, or proptosis. This report presents a 49-year-old male with refractory CIDP and extensive orbital involvement, including bilateral proptosis and enlargement of multiple cranial nerves, resulting in an orbital apex-like syndrome and compressive optic neuropathy. The patient's condition was refractory to medical management with intravenous immunoglobulin, corticosteroids, plasma exchange, and rituximab, and he ultimately required surgical decompression. Improvement in visual symptoms was noted in the early post-operative period. This case highlights the importance of a multidisciplinary approach in the management of complex CIDP cases with significant cranial nerve involvement and underscores the need for further research into its pathophysiology and treatment optimization.

Purpose: Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is the most common form of ocular adnexal lymphoma (OAL). In Taiwan, hepatitis B virus (HBV) infections are detected in about 15-20% of adults. This study aims to assess whether HBV infections influence the prognosis of ocular MALT lymphoma.

Methods: This study retrospectively reviewed patients diagnosed with ocular MALT lymphoma at two Taiwanese tertiary medical centers between 1988 and 2018. HBV infection was defined according to positive HBsAg serological tests. Outcomes were classified as complete remission or incomplete remission. Survival was also analyzed.

Results: This study enrolled 130 patients, 24 of whom were HBV-positive (18.5%) with mean age of 51.08 years old. The most common Ann Arbor clinical stage was IE in both HBV and non-HBV group (70.83% vs. 79.25%, respectively, p = 0.753). In HBV group, higher incomplete remission rate (50%, p = 0.021), shorter disease-free survival time (p = 0.039), and progression-free survival time (p = 0.016) were noted.

Conclusions: In conclusion, patients with systemic HBV infection have higher incomplete remission rate and shorter disease-free and progression-free survival time of ocular MALT lymphoma. This is the first study discussing the relationship between HBV infections and ocular MALT lymphoma on Taiwanese population.

Purpose: Dacryocystorhinostomy (DCR) involves creating an osteotomy in the frontal process of the maxilla (FPM). This study quantifies FPM thickness and angulation from an endoscopic perspective to inform surgical technique and instrument design.

Methods: CT scans from 94 patients were digitally segmented to create 3D models of the FPM. From a nostril-level point in the mid-sagittal plane, line vectors were projected through the FPM to simulate endoscopic access. For each vector, bone thickness and entry (α) and exit (β) angles were calculated. The angular difference (Δ = |α - β|) was used to assess alignment with common rongeur jaw angles.

Results: The maximum FPM thickness averaged 11.5 mm, reaching 20.8 mm postero-superiorly. The antero-superior region had the largest Δ (25.0°), indicating suboptimal alignment. The average α and β angles were 34.5° and 36.2°, respectively, with rather minimal regional variation.

Conclusions: The FPM's thickness and geometry hinder full bone removal during unpowered endoscopic DCR, particularly in the superior region. In 12% of cases, over one-third of the FPM may be inaccessible with standard rongeurs, risking incomplete sac exposure. These anatomical findings highlight the need for preoperative imaging and may guide the selection of surgical instruments to improve operative outcomes.

Purpose: Throat packs are used in endonasal surgery to absorb blood and secretions, theoretically reducing aspiration risk and post-operative nausea/vomiting. However, complications have been documented in the literature, and there is no clear consensus on the indications and method of use. This study aims to review the literature on throat pack use in dacryocystorhinostomy (DCR), and investigate the practices and views of anaesthetists, oculoplastic and ENT surgeons.

Methods: A systematic search of the PubMed/MEDLINE, Embase, and CENTRAL databases was performed. Data was collected and risk of bias assessed in accordance with PRISMA guidelines. A survey was also distributed to UK and Australian anaesthetists, ENT, and oculoplastic surgeons.

Results: The systematic review identified a lack of evidence supporting use of throat packs in DCR. Of the 82 respondents to the survey, 48% only use a pack if requested by another clinician, 23% never used, 12% used in every DCR, 11% used only in DCR with high risk of bleeding/aspiration, and 6% selected "other." ENT surgeons were least likely to use throat packs (64% never use, p = .002). Clinical experience did not influence usage. Individuals who believed throat packs were equally beneficial for both endonasal and external surgical approaches used them more often (p = .002). The most common complications of throat pack use observed were throat pain (57.3%) and mucosal injury (30.5%).

Conclusions: The findings highlight the arbitrary nature of throat pack use in DCR and the need for evidence-based guidelines, particularly in the presence of clear complications that have been observed.