IgG4-Related Dacryoadenitis With Fibrous Mass in a 19-Month-Old Child: Case Report and Literature Review.

A 19-month-old boy presented with eyelid swelling, proptosis, and upgaze limitation in the OD. The radiological study showed a homogeneous mass enclosing the lacrimal gland. Near-total tumor excision revealed IgG4-related orbital disease. Eosinophilia, serum IgG4, and IgE elevations were detected without extraorbital involvement. The patient received oral prednisolone for 4 months postoperatively and remained relapse-free for 27 months. Among the 17 well-documented pediatric cases of IgG4-related orbital disease in the literature (including this case), 59% were female, and the median age was 10 years; 2 patients were under 2 years old. The disease was unilateral in 82% of the patients and caused a soft tissue mass in 88%, involving the lacrimal gland in 53%. Nine patients received immunosuppression only, 4 surgery and immunosuppression, and 2 only surgical excision. Treatment results were reported in 13 patients, and all were favorable. IgG4-related dacryoadenitis with a fibrous mass may occur in very young children, responding well to surgical excision and steroids. Although pediatric IgG4-related orbital disease is not well-characterized yet, it may manifest differently from its adult variant.

Dumbbell-Shaped Congenital Conjunctival Cyst of the Orbit.

Congenital conjunctival cysts often occur in the medial orbit without adjacent bone destruction and have not been previously reported in a dumbbell configuration. A 46-year-old female patient presented with left proptosis and headache. A radiological study revealed a large, cystic, bilobed mass in the orbital-temporal area with an osseous defect in the lateral wall. A histological examination of the excised lesion showed that the cyst wall consisted of non-keratinized stratified epithelium with goblet cells. There was no recurrence during the follow-up. Of the previously reported 54 cases, only 1 had bone erosion due to a recurrent cyst in the frontal bone. Although rare, a primary conjunctival cyst can create a dumbbell-shaped lesion in the lateral orbit and temporal fossa.

Nasolacrimal canal morphology with or without idiopathic obstruction in Caucasian adults: a multidetector CT study.

PURPOSE: To compare the morphological features of the bony nasolacrimal canal (NLC) in Caucasian adults with and without primary acquired nasolacrimal duct obstruction (PANDO). METHODS: The study included one eye each from 38 patients with PANDO and 38 age- and gender-matched controls without PANDO, all of whom underwent multidetector computed tomography. In tomographic images, length, and orientation angles of the NLC, transverse canal diameters at the duct entrance and lower end, and minimum (narrowest) transverse and anterior-posterior canal diameters were measured. RESULTS: The two groups were similar for NLC length and angulations. The transverse entrance diameter was significantly narrower in the PANDO group (mean, 4.6 mm vs. 5.1 mm) (p = 0.09). The narrowest site was most frequently in the middle duct or slightly above the middle in both groups (p > 0.05). The minimum canal diameters were significantly smaller in the PANDO group (p = 0.010 and p = 0.003). When gender subgroups were compared, the significant differences continued for the transverse entrance and minimum diameters in females with PANDO (p = 0.006) and for the minimum anterior-posterior diameter in males with PANDO (p = 0.02). CONCLUSION: Narrowness of the upper and/or middle part of the bony nasolacrimal duct may play a role in the development of PANDO in the adult Caucasian population.

Transnasal or transglabellar semicircular flap for medial canthal reconstruction.

PURPOSE: To review the results of medial canthal reconstruction with the medial (transnasal or transglabellar) semicircular flap. METHODS: Medical charts of 38 patients who underwent the described procedure were reviewed. After tumor excision, a semicircular flap created along the nasal bridge or glabella was advanced to the canthal defect; if necessary, this flap was combined with other reconstructive methods. RESULTS: The patients (19 male, 19 female; mean age, 66 years) had basal cell (n = 36) or squamous cell (n = 2) carcinomas. The mean tumor diameter was 9.2 mm (range, 3-21 mm). Tumor epicenters were in the midcanthal area in 21 patients (55%) and in the infra- or supracanthal areas in 17 patients (45%). After excision, 22 patients had only canthal defects, and 16 had an associated upper and/or lower eyelid defect. To cover the defect, the medial semicircular flap alone was used in 19 patients (50%) and in association with other flaps in 19 patients (50%). The excisional defect was primarily closed in 37 patients (97%). Flap necrosis or infection did not occur. During follow-up (range, 1-91 months; median, 19 months), 10 patients (26%) developed a total of 17 complications. Three patients (8%) required secondary surgery for eyelid reconstruction-related complications. CONCLUSIONS: Transnasal or transglabellar semicircular flap may be a good alternative for medial canthal reconstruction. For large or complex defects, the medial semicircular flap can be combined with other periocular flaps. In the latter case, postoperative complications requiring secondary surgery may develop.

Reconstruction of Orbital Exenteration Defect With Cheek or Combined Cheek and Forehead Advancement Flaps.

PURPOSE: To review the outcomes of orbital exenteration defect reconstruction using cheek or combined cheek-forehead advancement flap. METHODS: Charts of 14 patients who underwent reconstruction of the exenterated orbit with cheek advancement flap were reviewed. In surgery, a cheek flap elevated via a nasofacial sulcus incision, and preperiosteal dissection was advanced over the defect. The upper orbital defect, if necessary, was covered with a forehead flap, which was dissected through an incision in the midline or temporal forehead and advanced inferiorly. RESULTS: In all patients (7 women, 7 men; mean age, 65 years), total (n = 7) or extended (n = 7) exenteration was performed for a malignant tumor. In 12 patients (86%), the defect was primarily closed with cheek flap alone (n = 6) or cheek plus forehead (n = 6) advancement flaps. Eight patients received radiotherapy before and after surgery. Four patients (29%) had a total of 6 postoperative complications (skin graft infection, orbital cavitary abscess, osteomyelitis, chronic skin ulcer, and 2 sino-orbital fistulae). The mean follow-up duration was 43 months (range, 11-79 months). CONCLUSIONS: Cheek advancement flap can be used alone or together with a forehead advancement flap to cover the orbital defects after total or extended exenteration. This repair may be resistant to radiotherapy-related complications in some cases.

Rituximab Therapy for Recalcitrant Idiopathic Sclerosing Orbital Inflammation.

Three patients (3 female patients; aged 7, 35, and 61 years) who had recalcitrant idiopathic sclerosing orbital inflammation were treated with rituximab. The disease was bilateral in 1 patient (4 orbits in total): diffuse in 2 and localized in 2 orbits. It caused optic neuropathy in 1 orbit of each patient. Conventional immunotherapy and tumor debulking surgery were unsuccessful in controlling the disease. After rituximab infusions (375 mg/m2/week for 4 weeks), all patients improved symptomatically. Radiologically, the local lesions resolved completely and diffuse lesions partially. Two patients with recurrent inflammation during follow up (78, 58, and 51 months) responded well to immediate, short-term steroid treatments. Short-term rituximab therapy can induce effective remissions in patients with refractory idiopathic sclerosing orbital inflammation. Early and local lesions may respond better to treatment than diffuse lesions. Nevertheless, inflammatory exacerbations can occur during late follow up.

Reconstruction of Large Upper Eyelid Defects With Bilobed Flap and Tarsoconjunctival Graft.

PURPOSE: To describe the reconstruction of large upper eyelid defects with bilobed flap and tarsoconjunctival graft. METHODS: The medical records of 5 patients who underwent upper eyelid tumor excision and eyelid reconstruction with a bilobed flap were reviewed. Various parameters, including demographic and clinical data, defect diameter, primary defect closure, complications, and follow-up time, were recorded. After tumor excision, the posterior lamella was reconstructed with an autologous tarsoconjunctival graft and anterior lamella with a superiorly based lateral bilobed flap. RESULTS: All 5 patients (3 women, 2 men; age: 42-87 years) had malignant epidermal (n = 2) or adnexal (n = 3) tumors. Mean excisional defect diameter was between 18.5 and 25 mm. In all patients, the anterior lamellar defect was closed primarily with a bilobed flap. After surgery, a total of 4 complications occurred in 3 patients. One patient required orbital exenteration because of tumor recurrence. In the other patients, the functional and esthetic results were satisfactory. Follow-up time ranged from 4 to 102 months. CONCLUSION: Lateral periorbital bilobed flap can be a good alternative for the single-stage reconstruction of large upper eyelid defects.

Combined retro-peribulbar and subconjunctival anesthesia for evisceration surgery.

PURPOSE: To evaluate the efficacy of retro-peribulbar and subconjunctival anesthesia associated with intravenous sedation in patients undergoing evisceration and orbital implant placement. METHODS: The charts of 217 patients who underwent evisceration with trans-scleral implant placement were reviewed. Midazolam and fentanyl were used for intravenous sedation. For local anesthesia, a combination of lidocaine with epinephrine and bupivacaine was injected into the retrobulbar, upper peribulbar, and subconjunctival areas. The intraoperative pain and need for supplemental anesthetic injection were recorded prospectively. RESULTS: The surgery was performed with local anesthesia in 116 patients (53%) and with general anesthesia in 101 patients (47%). Patients were significantly older in the local anesthesia group than in the general anesthesia group (mean age, 59.9 years vs 45.2 years; P < .05). Supplemental retrobulbar anesthesia was required in 5 patients (4.3%). Transition to general anesthesia was required in 1 patient (0.9%) due to severe anxiety. Orbital hemorrhage developed after retrobulbar injection in 1 patient (0.9%), but did not preclude performing evisceration. CONCLUSIONS: Combined retro-peribulbar and subconjunctival anesthesia with intravenous sedation can provide safe and effective intraoperative analgesia for evisceration surgery with trans-scleral implant placement.

Isolated and Transient Nuclear Midbrain Blepharoptosis in a Young and Healthy Adult.

A 32-year-old otherwise healthy man presented with acute-onset bilateral blepharoptosis of 6 days' duration. On examination, he had severe ptosis bilaterally and mildly restricted abduction in the left eye. Brain magnetic resonance imaging showed a 10-mm-diameter lesion in the dorsal midbrain. The ptosis resolved spontaneously within two weeks. Systemic investigation did not uncover any aetiological factor. During 70 months' follow-up, neither any systemic disease nor ptosis relapse developed. Isolated nuclear midbrain ptosis has been previously reported in a few patients and these had neoplastic or inflammatory causes. In this patient, spontaneous resolution of the nuclear ptosis within weeks suggested that the underlying cause might be isolated ischaemic damage to the central caudal nucleus.

An Atypical Paediatric Optic Nerve Sheath Meningioma.

A 16-year-old male presented with a three year history of right proptosis. All other ocular findings were normal. Imaging demonstrated a large, calcified, contrast-enhancing mass in the apical orbit. The tumour had high gallium-68-DOTATATE uptake and low 18F-2-fluoro-2-deoxy-D-glucose uptake. An incisional biopsy revealed a diagnosis of psammomatous optic nerve sheath meningioma (ONSM). One year later stereotactic radiotherapy was performed due to tumour growth. Tumour size and visual acuity remained stable in the six months after treatment. This case differs from previously reported paediatric ONSMs by its histo-clinical characteristics (exophytic-calcified mass, visual preservation, psammomatous histology).

A Case of Diprosopus Tetraophthalmos: Ocular Findings and Surgical Treatment of Exposure Keratopathy.

Diprosopus is a rare variation of conjoined twinning. In this report, ophthalmic findings in an infant with diprosopus tetraophthalmos are presented. A male infant who was born at 33 weeks of gestation to a nonconsanguineous 42-year-old mother and 47-year-old father was examined for orbital abnormalities. The infant had a large head, 2 faces, and 4 eyes, of which 2 were placed laterally and 2 were fused in the midline. In the laterally placed eyes, the pupils were unresponsive to light, and the optic discs were hypoplastic. In the fused eyes, the lower and upper eyelids were also fused, horizontally wide, vertically short, and immobile, resulting in a progressive exposure keratopathy. On the 33rd day of life, a modified total tarsorrhaphy was performed with the use of inhalational mask anesthesia. The patient died due to pneumonia and septicemia on the 45th day of life.

Neonatal Orbital Abscess Secondary to Pseudomonas Aeruginosa Conjunctivitis.

Pseudomonas aeruginosa conjunctivitis, although rare in healthy infants, may cause serious ocular and systemic complications. A 30-day-old, otherwise healthy male infant was referred with the diagnosis of right orbital abscess. The patient had been diagnosed as having Pseudomonas conjunctivitis 9 days previously at the referring center. Despite antibiotic treatment, his ocular findings had worsened and marked proptosis had developed. Other examination findings were ptosis, restriction of eye movements, periorbital erythema, and chemosis. Radiologic studies showed a large, homogenous mass with a thick capsule in the lateral retrobulbar orbit. The abscess was drained through a lateral orbitotomy. A culture of the abscess yielded P. aeruginosa. After surgery, the ocular findings improved rapidly without any complication. No other focus of infection or immune system abnormality was found. The patient did not experience any other significant disease during a follow up of 23 months.

Thermal Burn Scar-Related Squamous Cell Carcinoma in the Eyelid.

A 63-year-old woman presented with a large, ulcerokeratotic mass in her right lower eyelid that had grown over the last 2 months in a burn scar. She had experienced a thermal burn involving both inferior periorbital areas 35 years ago and received medical treatment. The lesion was excised with wide margins. The histologic diagnosis was squamous cell carcinoma. No recurrence was observed during a 22-month follow up. In the previous literature, a total of 10 cases of eyelid burn scar neoplasm, of which 9 were basal cell carcinoma, have been reported. To the authors' knowledge, this is the first reported case of thermal burn-related squamous cell carcinoma of the eyelid.

Use of Direct Otoscope for Intranasal Examination After Dacryocystorhinostomy.

PURPOSE: To evaluate whether the use of a direct otoscope in the nose is an effective method to observe the nasolacrimal anastomosis site after dacryocystorhinostomy. METHODS: In 75 eyes undergoing external dacryocystorhinostomy, the lacrimal irrigation test was performed and fluorescein solution was instilled in the conjunctival sac. After applying topical anesthetics and decongestant, a direct otoscope was inserted in the middle meatus and directed toward the medial canthus. RESULTS: In 69 eyes (92%), intranasal lacrimal examination with the otoscope could be performed. In 6 eyes (8%), the otoscope could not be adequately advanced in the nose because of middle meatal stenosis (n = 5 eyes) or intolerance to the intranasal examination (n = 1). In 66 (96%) of 69 eyes, concordant with the results of the lacrimal irrigation test, the presence or absence of fluorescein dye flow in the nose was observed. In this examination, the main features that were useful in locating the anastomotic area were (1) the use of fluorescein dye, (2) motility of the nasal mucosa that was synchronized with eyelid movements, (3) appearance of mucosal hole or internal orifice in the nasal wall, and (4) dye or air-fluid bubble emergence upon blinking or finger pressure over the sac region. CONCLUSIONS: Direct otoscope can be effectively used in the nose for examining the nasolacrimal anastomosis in patients undergoing dacryocystorhinostomy.

Reconstruction of orbital exenteration defects with bilobed forehead flap.

To describe the use of bilobed forehead flap for reconstruction of orbital exenteration defect. The medical records of 2 patients in whom orbital exenteration defect repair was performed with bilobed forehead flap were reviewed. In both patients (male, ages 74 and 65 years), extended exenteration was performed because of basal cell carcinoma infiltrating the upper and lower eyelids and orbit. One patient had a history of multiple eyelid surgeries and periorbital radiotherapy. In the other, the tumor also involved the maxillary and ethmoid sinuses and nasal dorsum. The bilobed flap was combined with a cheek advancement flap in 1 patient. The excisional defect could be primarily covered in both patients. In 1 patient, a skin graft was needed to cover the forehead donor area defect. In both patients, transient, distal flap ischemia developed after surgery and, in 1 patient, eyebrow malposition required surgical correction at the late period. No other complication developed during follow-up (18 and 26 months). The bilobed forehead flap can be effectively used to reconstruct total or extended orbital exenteration defects.

Lacrimal Punctal Occlusion With Conjunctival Flap.

PURPOSE: To describe a technique of punctal occlusion with conjunctival flap and present its surgical results in a group of patients. METHODS: The records of 30 patients who underwent punctal occlusion with a conjunctival flap due to dry eye were reviewed. A medial segment of the eyelid margin, including the punctum, was superficially excised. The canalicular opening was closed with suturing, and then the margin defect was covered with a conjunctival transposition flap. RESULTS: Sixty-nine puncti (41 lower and 28 upper puncti) in 52 eyes were occluded with a conjunctival flap. During the follow-up period (mean, 4.8 months; range, 2-13 months), 65 puncti (94%) remained occluded and 4 puncti reopened. Because of postoperative epiphora, the procedure was surgically reversed in 2 lower puncti. No complication was encountered during or after surgery. CONCLUSION: Conjunctival transposition flap may be an alternative surgical method for effective and reversible occlusion of the lacrimal punctum in eyes with severe dry eye.

Orbital cavitary rhabdomyosarcoma: case report and literature review.

Orbital cavitary rhabdomyosarcoma has been previously reported in 2 cases. The case presented here was a 15-year-old boy who had proptosis, pain, periorbital hyperemia, and visual loss in his OD, which progressed in 6 weeks. Radiologic studies demonstrated a well-demarcated, multilobulated, large mass with cavities, extending from the anterior orbit to the apex, suggesting a diagnosis of venolymphatic malformation with an intrinsic hemorrhage. On surgery, a multilobular, hemorrhagic cystic mass was almost completely excised. Histologic examination revealed a diagnosis of embryonal rhabdomyosarcoma. After postoperative chemotherapy and radiotherapy, the tumor did not recur during a follow-up period of 45 months. This case, together with the previous ones, suggests that cavitary orbital rhabdomyosarcomas may have some distinct clinical, radiologic, and surgical characteristics.

Choroidal ganglioneuroma in a patient with orbitopalpebral neurofibromatosis.

Ganglioneuroma is a rare, benign tumor originating from the sympathetic ganglion cells. Choroidal ganglioneuroma in association with neurofibromatosis type 1 (NF-1) has been reported in a few cases. This study describes a 21-year-old woman with orbitopalpebral NF, who underwent evisceration for pseudophakic bullous keratopathy, intractable glaucoma, and absolute painful eyes and who was found to have choroidal ganglioneuroma and retrobulbar plexiform neurofibroma on histologic examination. This case, together with the previously described cases, suggests that eyes with choroidal ganglioneuromas may have some common clinical and prognostic characteristics, although this condition cannot be diagnosed preoperatively. In patients with NF-1 who may undergo eye removal surgery, the possibility of choroidal tumors should be kept in mind.