Transnasal endoscopic drainage of neonatal orbital abscess.

Neonatal orbital complications are rare and potentially fatal, demanding prompt diagnosis and adequate treatment. A 25-day-old neonate presented with rapidly progressive orbital complications as evidenced by proptosis, chemosis, lid oedema and restricted eye movements, developing within 3 days. There was no significant medical history or risk factors for developing infection. An initial conservative approach with antimicrobial therapy failed to show any resolve. An MRI brain, orbits and paranasal sinuses demonstrated that there were features suggestive of right orbital cellulitis with possibility of abscess formation with right ethmoidal mucoinflammatory disease and mass effect on the optic nerve causing stretching and compression by the surrounding inflammation.The patient was treated successfully with transnasal endoscopic drainage and decompression. Endoscopic access was challenging owing to the restrictive anatomy. Postoperatively, the patient showed improvement, with gradual decrease in proptosis and resolve in eye movements.

Successful Management of a Spontaneous Retrobulbar Hematoma After Heart Transplantation. A Case Report.

CASE DESCRIPTION: We present a case of a 65-year-old patient who underwent heart transplantation. After the surgery, left proptosis, conjunctival chemosis, and ipsilateral palpebral ecchymosis were found while he was still intubated. A retrobulbar hematoma was suspected, confirmed by a computed tomography scan. Initially, expectant management was considered, but with the appearance of an afferent pupillary defect, the patient underwent orbital decompression and posterior collection drainage, which prevented visual impairment. CONCLUSION AND IMPORTANCE: Spontaneous retrobulbar hematoma after heart transplantation is a rare condition that risks vision. We intend to discuss the importance of postoperative ophthalmologic examination after heart transplantation in intubated patients for early diagnosis and rapid treatment. Spontaneous retrobulbar hematoma (SRH) after heart transplantation is an exceptional condition that risks vision. Bleeding in the retrobulbar space provokes an anterior ocular displacement, extending the vessels and the optic nerve, which can generate ischemic neuropathy and, finally, a loss of vision [1]. A retrobulbar hematoma is usually associated with trauma or eye surgery. Though, in non-traumatic cases, the underlying cause is not evident. An adequate ophthalmologic examination is usually not performed in complex surgeries like heart transplantation. However, this simple measure can prevent permanent vision loss. Non-traumatic risk factors should also be considered, which include vascular malformations, bleeding disorders, use of anticoagulants, and increased central venous pressure usually triggered by a Valsalva maneuver [2]. The clinical presentation of SRH consists of ocular pain, decreased visual acuity, conjunctival chemosis, proptosis, abnormal extraocular movements, and elevated intraocular pressure (IOP). Its diagnosis is often clinical; however, it can be confirmed with computed tomography or magnetic resonance imaging. Treatment aims to reduce IOP with surgical decompression or pharmacologic measures [2]. In the reviewed literature, less than 5 spontaneous ocular hemorrhages related to cardiac surgery have been reported [3-6], of which only one is related to heart transplantation [3]. A clinical challenge of an SRH after heart transplantation is presented below. Surgical management was performed with a favorable result.

A clinical study of the effect of 3D reconstruction on exophthalmos after an operation on an old orbital wall fracture.

BACKGROUND: Orbital blowout fracture is common in ocular trauma. Accurate measurement of orbital volume after fracture is key in improving intraocular correction. OBJECTIVE: This study aims to explore the impact of 3D reconstruction technology in restoring normal exophthalmos in patients with old orbital wall fractures. METHODS: A total of 31 patients were randomly divided into an experimental group (n= 15) and a control group (n= 16). For orbital wall repair and reconstruction, the conventional group used the conventional surgical scheme, and the 3D group used 3D printing technology. RESULTS: There was no statistical difference between the preoperative mean extraocular muscle volume of the healthy eye and the affected eye. However, the mean orbital volume (24.76 vs 27.11, P= 0.005) and mean retrobulbar fat volume (17.53 vs 16.42, P= 0.006) were significantly different between the healthy eye and the affected eye. After an average follow-up of 16 weeks, the differences in pre- and post-surgery exophthalmos in the two groups were 0.42 ± 0.08 mm and 1.63 ± 0.51 mm, respectively. The difference between the two groups was statistically significant (t= 4.42, P= 0.003). The complications were not statistically different. CONCLUSION: Using 3D reconstruction technology preoperatively can significantly improve exophthalmos in patients with old orbital wall fractures.

Thrombosis of intraorbital arterio-venous fistula.

A 65-year-old male patient presented with a rare arterio-venous fistula (AFV). The symptoms included congestion, decreased visual acuity, and proptosis. Further investigation revealed a non-traumatic intra orbital AFV with ophthalmic vein thrombosis. The management strategy was craniotomy and the prescription of anticoagulants. The patient recovered 2 months after surgery demonstrating successful resolution of his presenting symptoms and an alternative approach to complicated cases of embolization.

Primary optic nerve sheath schwannoma: a case report.

BACKGROUND: The optic nerve is an unusual site of schwannoma as it lacks Schwannoma cells on it. We report a primary optic sheath schwannoma and to review the literature. CASE REPORT: A 29-year old female presented with progressive painless non-pulsatile proptosis. Ocular examination revealed only axial proptosis. Imaging showed a well-defined intraconal mass abutting optic nerve. A left frontal craniotomy with orbitotomy and tumor excision was done. The tumor was well encapsulated, posteriorly attached to optic nerve without any plane, probable site of origin. The postoperative duration was uneventful without any complications. The histopathology examination confirms the diagnosis of schwannoma. CONCLUSIONS: We suggest to consider orbital optic nerve schwannoma in differential diagnosis of orbital tumors despite its exceedingly rare occurrence.

Transpalpebral transorbital neuroendoscopic (TONES) repair of orbital meningoencephalocele: a technical note.

PURPOSE: Intraorbital encephalocele (OMEC) is a rare entity in adults, usually secondary to an orbital pathology or prior trauma, in particular orbital roof fractures. Treatment of the OMEC is warranted to alleviate the pulsating exophthalmos and prevent potential visual decline. OMEC and orbital roof fractures have been predominantly treated via a craniotomy with a reconstruction of the orbital roof using various implants. With the advances in the endoscopic techniques, neuroendoscopy found its application in the treatment of orbital pathologies. We report a minimally invasive alternative: endoscopic transorbital repair of OMEC. MATERIAL AND METHODS: The repair technique is described with illustrations and clinical images. Narrated operative video demonstrating the procedure is provided. RESULTS: Illustrative case: 50-year-old female presented with progressive right eye proptosis over 6 months. Computed tomography (CT) demonstrated bony erosion in the lateral orbital roof, and magnetic resonance imaging (MRI) showed a small hyperintense T2-weighted and T1-weighted contrast enhancing lesion in the orbit, in the area of the bony erosion. Intraoperatively, the lesion was found to be an orbital encephalocele. The orbital defect was successfully repaired by employing the 'sandwich' technique, in which a dural substitute reinforced with tissue glue were deployed without repair of the osseous orbital roof. The patient tolerated the procedure well with ultimate resolution of proptosis. The cosmetic outcome was excellent. CONCLUSION: The transorbital neuroendoscopic approach (TONES) presents a feasible, minimally invasive alternative treatment option for circumscribed intraorbital encephaloceles with minimal side effects, well tolerated by patients.

Fetal Orbital Epidermal Cyst Pre-postnatal Imaging Findings: A Case Report and Literature Review.

BACKGROUND: Fetal ultrasonographic evaluation is a routine part of pregnancy follow-up, and examination of orbital structures is also part of the routine evaluation. Although orbital developmental anomalies are common in the neonatal period, diagnosis in the intrauterine period is not common. To our knowledge, three cases with a diagnosis of congenital orbital epidermal cysts have been reported in the literature, and two of them had fetal imaging findings. In this article, we present the prepostnatal imaging findings of a case diagnosed with orbital cyst in the fetal period and histopathologically diagnosed as epidermal cyst in the neonatal period. CASE REPORT: A 25-year-old woman applied for ultrasonography (USG) examination at 22 weeks of gestation. A 35x45 mm cystic lesion causing proptosis, without solid component and vascularity, surrounding the optic nerve and causing its thinning was observed in the left orbit. In fetal magnetic resonance imaging (MRI), the intraorbital cystic lesion, which was hyperintense on T2W images and hypointense on T1W images, had no relationship with intracranial structures and no solid component. The lesion of the patient, followed up with a multidisciplinary approach, was shown similarly with computed tomography (CT) and magnetic resonance imaging (MRI) in the postnatal period. Subsequently, the patient underwent globe-sparing surgery, and the pathological diagnosis was made as the epidermal cyst. CONCLUSION: Orbital epidermal cysts are rarely seen, and detection in the fetal period is even rarer. It should be considered in the differential diagnosis of orbital cystic lesions that cause proptosis in fetal and neonatal periods.

[A man with bulging eyes]. / Een man met uitpuilende ogen.

Graves' orbitopathy may cause multiple symptoms, such as proptosis, redness or inflammation of the conjunctiva, excessive tearing, swelling of the eyelids and pain. Smoking, male gender and old age are significant risk factors for a more severe and active disease.

Robot-Assisted Orbital Fat Decompression Surgery: First in Human.

Purpose: To explore the safety and feasibility of robot-assisted orbital fat decompression surgery. Methods: Ten prospectively enrolled patients (18 eyes) with Graves' ophthalmopathy underwent robot-assisted orbital fat decompression surgery with the da Vinci Xi surgical system. Intraoperative blood loss, operative time, and complications were recorded. For every patient, the exophthalmos of the operated eyes and Graves' orbitopathy quality of life (GO-QoL) were measured both preoperatively and 3 months postoperatively to assess the surgical effect. Results: All surgical procedures were successfully performed. The mean duration to complete the whole procedure was 124.3 ± 33.2 minutes (range, 60-188). The mean intraoperative blood loss was 17.8 ± 6.2 mL (range, 7.5-28). There were neither complications nor unexpected events in terms of either orbital decompression surgery or robot-assisted procedures. The mean exophthalmos was 20.2 ± 1.8 mm before surgery and 17.9 ± 1.4 mm postoperatively (P < 0.0001). The preoperative and postoperative GO-QoL on the visual function arm was 84.38 ± 20.04 and 93.75 ± 9.32, respectively. The preoperative and postoperative GO-QoL on the appearance arm was 42.50 ± 14.97 and 64.38 ± 21.46, respectively (P = 0.027). Conclusions: The da Vinci Xi surgical system provided the stability, dexterity, and good visualization necessary for orbital fat decompression surgery, indicating the safety and feasibility of robot-assisted orbital fat decompression surgery. Translational Relevance: Based on a literature search using EMBASE and MEDLINE databases, we believe that this study reports the first in-human results of the safety and effectiveness of da Vinci robot-assisted orbital fat decompression surgery.

Factors Associated with Surgery and Imaging Characteristics in Severe Orbital Infections.

OBJECTIVES: To evaluate risk factors associated with surgical intervention and subperiosteal/orbital abscess in hospitalized children with severe orbital infections. STUDY DESIGN: We conducted a multicenter cohort study of children 2 months to 18 years hospitalized with periorbital or orbital cellulitis from 2009 to 2018 at 10 hospitals in Canada. Clinical details were extracted, and patients were categorized as undergoing surgical or medical-only management. Primary outcome was surgical intervention and the main secondary outcome was clinically important imaging. Logistic regression was used to identify predictors. RESULTS: Of 1579 patients entered, median age was 5.4 years, 409 (25.9%) had an orbital/subperiosteal abscess, and 189 (12.0%) underwent surgery. In the adjusted analysis, the risk of surgical intervention was associated with older age (age 9 to <14: aOR 3.9, 95% CI 2.3-6.6; and age 14 to ≤18 years: aOR 7.0, 95% CI 3.4-14.1), elevated C-reactive protein >120 mg/L (aOR 2.8, 95% CI 1.3-5.9), elevated white blood cell count of 12-20 000/µL (aOR 1.7, 95% CI 1.1-2.6), proptosis (aOR 2.6, 95% CI 1.7-4.0), and subperiosteal/orbital abscess (aOR 5.3, 95% CI 3.6-7.9). There was no association with antibiotic use before hospital admission, sex, presence of a chronic disease, temperature greater than 38.0°C, and eye swollen shut. Complications were identified in 4.7% of patients, including vision loss (0.6%), intracranial extension (1.6%), and meningitis (0.8%). CONCLUSIONS: In children hospitalized with severe orbital infections, older age, elevated C-reactive protein, elevated white blood cell count, proptosis, and subperiosteal/orbital abscess were predictors of surgical intervention.

Erdheim-Chester disease: look it in the eye. An orbital magnetic resonance imaging study.

Erdheim-Chester disease (ECD) is a rare L-group histiocytosis. Orbital involvement is found in a third of cases, but few data are available concerning the radiological features of ECD-related orbital disease (ECD-ROD). Our aim was to characterize the initial radiological phenotype and outcome of patients with ECD-ROD. Initial and follow-up orbital magnetic resonance imaging (MRI) from the patients with histologically proven ECD at a national reference center were reviewed. Pathological orbital findings were recorded for 45 (33%) of the 137 patients included, with bilateral involvement in 38/45 (84%) cases. The mean age (± standard deviation) of these patients was 60 (±11.3) years and 78% were men. Intraconal fat infiltration around the optic nerve sheath adjacent to the eye globe (52%), with intense gadolinium uptake and a fibrous component was the most frequent phenotype described. Optic nerve signal abnormalities were observed in 47% of cases. Two patients had bilateral homogeneous extraocular muscle enlargement suggestive of a myositis-like involvement of ECD-ROD. None had isolated dacryoadenitis but in 17 eyes dacryodenitis was described in association with other types of orbital lesions. Only seven patients (15%) had normal brain MRI findings. ECD-associated paranasal sinus involvement and post-pituitary involvement were detected in 56% and 53% of patients, respectively. A decrease/disappearance of the lesions was observed in 17/24 (71%) of the patients undergoing late (>12 months) followups. Interestingly, ECD-ROD only rarely (7/45; 16%) revealed the disease, with exophthalmos being the most frequently identified feature in this subgroup (3/45; 6%). Even though ECD-ROD can be clinically silent, it comprises a broad array of lesions often resulting in optic nerve signal abnormalities, the functional outcome of which remains to be established. ECD-ROD should thus be assessed initially and subsequently monitored by orbital MRI and ophthalmological follow-up.

Dupilumab as an adjunct treatment for a patient with steroid-dependent immunoglobulin G4-related disease complicated by asthma: a case report.

Introduction: Immunoglobulin G4-related disease (IgG4-RD) responds well to glucocorticoids but is often associated with relapses. Interleukin (IL)-4 and IL-13 are involved in the pathogenesis of IgG4-RD. We present the first case in which dupilumab was an effective adjunct treatment for a patient with steroid-dependent IgG4-RD complicated by asthma.Case study: A 57-year-old man was referred to our hospital for further investigation and treatment of proptosis with neck swelling in 2019. He developed a cough and swelling of the neck in 2016. He was diagnosed with asthma in 2017 and started receiving inhaled glucocorticoids and a long-acting beta-agonist. The patient started receiving oral prednisolone at a dose of 20 mg/day. Oral prednisolone reduced his symptoms, but he relapsed when treatment was tapered to less than 10 mg/day. He was diagnosed with IgG4-RD through a parotid gland biopsy.Results: Azathioprine was given to reduce systemic glucocorticoids. The prednisolone dose was gradually tapered to 10 mg/day, resulting in the relapse of proptosis and an asthma attack. We added dupilumab, and his asthma symptoms and proptosis improved. Serum IgG4 levels continued to decrease, and the prednisolone dose was tapered to 2 mg.Conclusion: Dupilumab might be useful as an adjunctive treatment for patients with steroid-dependent IgG4-RD complicated by asthma. Serum IgG4 levels can be used as a marker to monitor dupilumab treatment in IgG4-RD.

A comparative study of porous polyethylene versus absorbable polydextro- and polylevolactic-lactide plate in reconstruction of isolated medial orbital wall fracture.

BACKGROUND: Several materials for medial orbital wall reconstruction have been mentioned in the literature. Our main purpose was to investigate postoperative enophthalmos and diplopia after medial orbital wall reconstruction with polydextro- and polylevolactic (poly-L/DL) acid (P[L/DL]LA) mesh plates and porous polyethylene plates. METHODS: Using a retrospective study design, we enrolled a cohort of isolated medial blowout fracture patients treated during a 58-month interval. The predictor variable was medial orbital wall reconstruction materials (P(L/DL)LA mesh plate and porous polyethylene plate. The main outcome variables included the occurrence of postoperative enophthalmos and diplopia at 1 week, 1, 3, 6, and 12 months post-surgery. Appropriate descriptive, uni- and bivariate statistics were computed, and P < 0.05 was considered significant. RESULTS: Three hundred-two isolated medial blowout fracture patients were included (24.5% females, 67% treated with P(L/DL)LA mesh plate). Exophthalmos measured highest in both groups 1 week after surgery and decreased steadily for 6 months postoperatively. Statistically significant differences were observed between both groups at 1 week, 1 month, and 3 months after surgery, with a higher incidence of exophthalmos observed in the P(L/DL)LA mesh plate group (P < 0.001). No significant differences were observed at 6 and 12 months after surgery. CONCLUSION: The occurrence of enophthalmos after medial blowout fracture reconstruction with P(L/DL)LA mesh plate is comparable with the use of porous polyethylene plate. Both P(L/DL)LA mesh and porous polyethylene plates are, therefore, reliable implants for medial orbital wall reconstruction.

Midgut NET With Orbital, Myocardial, Testicular, Lymph Nodal and Pulmonary Metastases Presenting With Bilateral Proptosis-Role of 68Ga-DOTANOC PET/CT.

Neuroendocrine tumors (NET) are rare neoplasms and commonly metastasize to liver, lymph nodes and less frequently to bones and lungs. Metastases to other organs are extremely rare and we report a case of NET clinically presenting with bilateral proptosis secondary to metastases in orbits. Ga-DOTANOC PET/CT demonstrated somatostatin receptor overexpressing lesions in bilateral orbits, small intestine, lymph nodes, lungs, heart and testes in the absence of liver metastases.

Odontogenic parakeratinized cyst resulting in exophthalmos and palatine, maxillary, and zygomatic bone erosion in a dog.

A novel case of a canine odontogenic parakeratinized cyst (COPC) that resulted in exophthalmos and palatine, maxillary, and zygomatic bone erosion in a 5-year-old Chihuahua dog is reported. Final diagnosis was aided by cross-sectional imaging (magnetic resonance imaging and computed tomography) and confirmed with histologic examination of the cyst wall.

Endovascular Treatment of a Giant Dissecting Aneurysm in an Infant

Introduction The incidence of intracranial aneurysms in the pediatric population is low, and endovascular treatment is becoming a safe and minimally invasive treatment option. In the present study, the occurrence of special features of cerebral aneurysm in children, in comparison to adults, is also described. Case Report A 3-month-old female infant presented with progressive proptosis and divergent strabismus at the right eye, in addition to inconsolable crying. Cerebral resonance, angiotomography and angiography exams demonstrated angiodysplasia in the right internal carotid artery with two large paraclinoid dissecting aneurysms with wide neck. The right internal carotid artery was occluded with coils by endovascular approach, without detriment to the perfusion of the ipsilateral hemisphere and without neurological deficits. The patient achieved good recovery, and a late control angiotomography confirmed the exclusion of the aneurysms. Conclusion Parent artery sacrifice via endovascular approach is an effective therapeutic option, but a long-termfollow-up is necessary to avoid recurrence and bleeding.

Sight preserving orbital decompression: a novel multidisciplinary approach to managing severe proptosis in neurofibromatosis type 2.

We describe the importance of collaboration between multiple surgical specialties in managing a complex case of sight-threatening severe proptosis in a young woman with type 2 neurofibromatosis (NF2) complicated by pre-existing contralateral blindness. Trans-nasal and lateral orbital surgical approaches were aided by stereotactic navigation to debulk a large frontal/sphenoid wing meningioma, which had been exerting pressure onto the right globe and optic nerve. The patient made an excellent postoperative recovery along with preserved residual visual acuity, normal neurology and a good aesthetic outcome.