Levator palpebralis superioris histopathologic findings in congenital ptosis and surgical outcome after its resection: Is there any association?

PURPOSE: To evaluate the histopathologic findings of Levator palpebralis superioris (LPS) muscle biopsy after LPS resection for treatment of congenital ptosis and its possible relation with surgical outcomes.Please confirm if the author names are presented accurately and in the correct sequence (given name, middle name/initial, family name). Author 4 Given name: [Seyed Mohsen] Last name [Rafizadeh]. Author 6 Given name: [Seyed Ali] Last name [Sonbolestan].Also, kindly confirm the details in the metadata are correct.The author names and the sequence are correct. METHODS: Congenital ptosis patients were enrolled in this retrospective study. All of them underwent full ophthalmologic examination included of Margin-reflex distance 1 (MRD-1) and LPS function measurement preoperatively. The patients were followed for 3 months for the postoperative period and after that the measurements were repeated. Histologic parameters including percentages of fat, striated and smooth muscle, and fibrous tissue. The histopathologic findings and their possible correlation with the measurements are analyzed. RESULTS: Sixty-seven patients with unilateral congenital ptosis were enrolled. 45 patients (67.2%) were males. The mean age of patients was 16.10 ± 11.18 years. The patients' MRD-1 was improved significantly from 0.82 ± 1.26 mm to 3.85 ± 1.25 mm after LPS resection (P = 0.000). The success rate was 80.3%. There were no correlations between MRD change and histopathologic tissue percentages but significant correlation was found between success of surgery and fibrous tissue percentage of resected sample (P = 0.033). CONCLUSIONS: The histopathology of the LPS may be useful in prediction of surgical outcome after LPS resection in congenital ptosis patients. The percentage of fibrous tissue play an important role.

The role of Whitnall's ligament position in the success of levator resection surgery in congenital ptosis.

PURPOSE: This study aimed to investigate the role of Whitnall's ligament position in the success of levator resection surgery in congenital ptosis. METHODS: It was an interventional case series on patients with congenital ptosis who underwent levator muscle resection in Farabi Eye Hospital (2020-2022). Patients with incomplete follow-up, a history of trauma, poor Bell's phenomenon, previous ocular and lid surgeries, poor levator function (≤ 4mm), and syndromic ptosis or systemic diseases were excluded. During the surgery, several factors, including the distance between Whitnall's ligament and the upper edge of the tarsus (W-distance), the vertical length of the tarsus (T-length), and the amount of levator muscle resection (LMR), were measured. A successful outcome was defined as the inter-eye difference of margin reflex distance-1 (MRD1) ≤ 1 and post-op MRD1 ≥ 3 OR the inter-eye difference of MRD1 ≤ 0.5 with any value of post-op MRD1 in unilateral cases and Postop-MRD1 > 3 in bilateral cases during the 3-months period. RESULTS: Thirty four eyes of 34 patients were included, and 79.4% of patients achieved successful outcomes. In univariate analysis, Preop-MRD1 and Preop-LF had meaningful negative correlations with the amount of LMR to reach the successful outcome (p < 0.05), which was only meaningful for Preop-LF in multivariable analysis (p < 0.05). Noticeably, W-distance had a significant positive correlation in univariate and multivariable linear regression (p < 0.05). CONCLUSIONS: W-distance can be considered a significant new parameter other than Preop-LF influencing the amount of levator resection needed to achieve success in levator resection surgery.

Association between blepharophimosis-ptosis-epicanthus inversus syndrome and lacrimal system anomalies.

Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a congenital eyelid syndrome. Several associations, including the horizontal displacement of the puncta, canalicular stenosis, and ectropion have been so far described. Herein, we report a one-year-old boy presented to the Oculoplastic Clinic of Farabi Eye Hospital with complaint of watery discharge from both eyes since his birth. Based on the general appearance, the diagnosis of BPES was made. Mild tear regurgitation from the inferior punctum was noted. Detailed examination showed bilateral superior punctal agenesis with coloboma of both upper eyelids and lateral displacement of the inferior puncta. Multiple unsuccessful attempts of probing were suggestive of the presence of NLDO. The patient was managed by performing canaliculodacryocystorhinostomy. Osteotomy was performed to pass the canalicular and nasolacrimal obstruction followed by a successful canaliculoplasty. Finally, the lacrimal drainage system was intubated with a mono-Crawford from the inferior punctum into the nasal cavity. On the 1st-month follow-up visit, the complaint of watery discharge was resolved. This is an extremely rare report of nasolacrimal duct and sac anomaly in a patient with blepharophimosis. Thus, we recommend the evaluation of the nasolacrimal drainage system in these patients after the first month of birth.

COVID-19 associated rhino-orbito-cerebral mucormycosis, risk factors and outcome predictors; a multicentric study.

BACKGROUND: Since the onset of the Covid-19 pandemic, an increase in mucormycosis cases has been observed in many countries, including Iran. However, the role of covid-19 and associated risk factors have not been thoroughly investigated. OBJECTIVE: This study is designed to identify epidemiologic characteristics, risk factors, and outcome predictors of Covid-19-Associated Rhino-Orbito-Cerebral Mucormycosis (C-ROCM). METHODS: Data of pathology proven Covid Associated ROCM cases were retrospectively obtained from 7 tertiary care centers throughout Iran from February 20, 2021, to July 22, 2021. Univariate and multivariate analyses were performed using binary logistic regression to assess the effects of various factors on the outcome. RESULTS: A total of 132 patients with C-ROCM were included in the study. The mean age of patients was 61.6 ± 13.9 (60.6% male). In 12 patients (9.1%), both eyes were involved. Diabetes was the most common comorbidity (94.7%). The mortality rate was 9.1%, higher in males (12.5%) than females (3.8%). Severe vision impairment was seen in 58 patients (43.9%). Main factors that had a negative impact on the outcome in the univariate analysis include older age (P < 0.001), higher steroid dosage (P < 0.001), higher HbA1c level (P < 0.001), Covid-19 severity (P < 0.001), and brain involvement (P < 0.001). However, in the multivariate analysis, the effects of age (P = 0.062), steroid dosage (P = 0.226), and Covid-19 intensity (P = 0.084) decreased, and the difference was no longer statistically significant. CRAO was a predictor of mortality in the univariate analysis (P = 0.008, OR = 4.50), but in the multivariate analysis, this effect decreased and was no longer significant (P = 0.125). CONCLUSION: The risk of C-ROCM and its complications may increase in patients with more severe Covid-19, steroid over-prescription, ICU admission due to Covid-19, and poor glycemic control during and after Covid-19 treatment.

Severe unilateral congenital ptosis with poor levator function: tarsoconjunctival mullerectomy plus levator resection vs frontalis sling procedure.

AIM: To compare frontalis sling and tarsoconjunctival mullerectomy plus levator resection (TCMLR) in subjects with severe unilateral congenital ptosis with poor levator function (LF). METHODS: A prospective non-randomized non-blinded single center clinical trial. Fifty patients with severe unilateral congenital ptosis with poor LF were recruited. The frontalis sling and TCMLR were performed and the functional, cosmetic outcomes, complications, and success rate were evaluated at 1, 3, and 6mo postoperatively. The t-test, Chi-square, Fishers exact, and nonparametric Mann-Whitney tests were used by SPSS software. RESULTS: Frontalis sling and TCMLR procedures were performed on 26 and 24 patients respectively. The mean age was 10.97±10.67y. LF was significantly better in the TCMLR group at months 1, 3, and 6 (P=0.002). Lagophthalmos was more common in the TCMLR group (no significant difference). At month 3, mild punctate epithelial erosions were observed more in the frontalis sling group (P=0.002). Significant complete success rate of 1st and 6th month for the frontalis sling vs TCMLR groups were 50% vs 20.8% (P=0.02), and 38.4% vs 50% (P=0.03) respectively. CONCLUSION: Complete success rate of TCMLR is higher in long-term follow-up in contrast with the frontalis sling in the short-term. Transient complications are more detected in mid-term follow-ups in both groups.

Lacrimal Canalicular Bypass Surgery with Autologous Superficial Temporal Artery Graft.

Purpose: To describe superficial temporal artery graft as a new autologous tissue to reconstruct the upper lacrimal drainage system. Methods: We explain the history of a 30-year-old female with upper lacrimal drainage system obstruction, in whom conjunctivodacryocystorhinostomy (CDCR) failed to resolve epiphora. A superficial temporal artery graft was harvested, intubated with Masterka tube, and implanted between the conjunctiva and nasal cavity. Masterka was replaced with a thicker dummy tube 12 weeks postoperatively. The adequacy of the graft was checked with irrigation tests in follow-up visits from 1 to 26 months after the procedure. Results: Superficial temporal artery autograft successfully eliminated epiphora of the patient in whom (CDCR) with Jones tube failed to make her symptom free. Conclusion: Superficial temporal artery autograft as an autogenous tissue with adequate characteristics can be considered in selective patients of upper lacrimal obstruction to reconstruct the lacrimal drainage system.

Post periorbital carboxytherapy orbital emphysema: a case report.

Periorbital carboxytherapy is used as one of the non-surgical facial rejuvenation methods in recent years. In this modality, the sterile carbon dioxide is injected into the subcutaneous space, assuming that to improve blood supply and repair of the injection site. Here, we report a 24- year-old woman who presented with acute bilateral orbital emphysema starting one day after cosmetic periorbital carboxytherapy. On physical examination, bilateral non-tender, non-erythematous swelling of both upper and lower lids was noted with crepitus on palpation. Uncorrected visual acuity (UCVA) of the patient in both eyes was 10/10 and RAPD was negative. The orbital computed tomography (CT) scan of the patient confirmed bilateral preseptal emphysema extending into orbital space through the orbital septum. Oral prednisolone 50 mg per day was prescribed, and the patient was observed closely. Gradually, periorbital pain and swelling subsided, and the symptoms resolved within one week.

Orbital myiasis on recurrent undifferentiated carcinoma in the COVID-19 era: a case report and brief review of the literature.

BACKGROUND: Myiasis is defined as the infestation of living tissues by Diptera larvae. Ophthalmic involvement occurs in less than 5% of cases. As the most uncommon type of involvement, orbital myiasis usually affects patients with poor personal hygiene, a low socioeconomic status, a history of surgery, and cancer. FINDINGS: In January 2020, an 89-year-old man presented to the Oculoplastic Department of Farabi Eye Hospital (Iran) with a history of left-side progressive orbital mass for six months. A large infiltrative mass of the left orbit with extension to the globe, periorbita, and adnexa was remarkable at the presentation, and its appearance suggested malignancy. Our findings persuaded us to perform exenteration and histopathological evaluation which were reported as "undifferentiated carcinoma". Regular follow-up visits were recommended. In June 2020, with a 3-month delay, the patient presented with the recurrence of the mass complicated with mobile alive larva. Examinations revealed numerous maggots crawling out of an ulcerative and foul-smelling lesion. He stated that fear of COVID-19 infection postponed his follow-up visit. The patient underwent immediate mechanical removal of larvae, followed by wide local excision of the mass. CONCLUSION: Patients with carcinoma of the adnexal tissues seem to be more prone to myiasis infestation even though it is an uncommon disease. Since COVID-19 is an ongoing pandemic with no end in sight appropriate protocols should be implemented to prevent loss of follow-up in these high risk patients.

Pulled versus Pushed Monocanalicular Silicone Intubation in Adults with Lacrimal Drainage System Stenosis: A Comparative Case Series.

PURPOSE: To compare the success rate and complications of pulled versus pushed monocanalicular intubation in adults with incomplete lacrimal drainage system obstruction (lacrimal drainage system stenosis). METHODS: Patients with lacrimal drainage system stenosis (Munk grade ≥3), including both nasolacrimal duct (NLD) stenosis and common canalicular stenosis, were recruited in this prospective comparative case series. Patients underwent probing and either Monoka (51 eyes) or Masterka (48 eyes) intubation under general or local anesthesia. Tubes were removed 4-14 weeks after the procedure. Six months after tube removal, Munk grades 0 and 1 were defined as a complete success, Munk grade 2 was defined as a partial success, and Munk grade ≥3 was defined as failure. All complications were recorded. RESULTS: Ninety-nine eyes from 89 patients with lacrimal drainage system stenosis who underwent either Monoka (51 eyes) or Masterka (48 eyes) intubation were included. The mean (SD) age of the patients was 55.4 (12) years in the Monoka group and 53.5 (12.9) in the Masterka group. Groups were matched on demographics. Masterka intubation could not be performed in one eye. Complete and partial successes were observed in 52.9% (27/51) and 17.6% (9/51) of eyes in the Monoka group and 42.6% (20/47) and 12.8% (6/47) of eyes in the Masterka group, respectively (p=0.29). There was a trend toward a higher total success rate in patients with NLD stenosis treated with Monoka 66.7% (26/39) than Masterka 45.5% (15/33) intubation (p=0.07). This trend also existed in patients with common canalicular stenosis (83.3% (10/12) vs. 76.6% (11/14), p=0.75). Age, sex, bilateral involvement, and duration of intubation did not have a significant impact on the success rate. Early tube loss, slit puncta, and temporary superficial punctate keratopathy were observed complications. CONCLUSION: Intubation with the pulled monocanalicular silicone tube was associated with a slightly but not significantly higher success rate in adults with lacrimal drainage system stenosis. Patients with NLD stenosis may achieve better results with pulled silicone tubes.

Effect of Smoking on Retinal Thickness and Vascular Density in Thyroid Eye Disease.

PURPOSE: To evaluate the effect of smoking on retinal thickness and macular and peripapillary vascular density in thyroid eye disease (TED). METHODS: In this cross-sectional study, subjects diagnosed with TED were analyzed in three groups: smokers, passive smokers, and non-smokers. Ganglion cell complex thickness, total retinal thickness, macular superficial vascular plexus densities, deep vascular plexus densities, optic nerve head, and radial peripapillary capillary density were measured in each group. RESULTS: Twenty-two eyes (21.6%) of active smokers, 11 eyes (10.8%) of passive smokers, and 69 eyes (67.6%) of non-smokers constitute the study subjects. Twenty-one eyes (12.6%) had active status (clinical activity score ≥3), 77 eyes (46.1%) were neither active nor compressive, four eyes (2.4%) of two patients constituted the compressive group. Age and disease activity adjusted analysis was performed. Ganglion cell complex thickness of smokers was significantly higher than non-smokers in the inferior hemi-parafoveal sector (p = 0.04). Active smokers had significantly higher (p < 0.01) retinal thickness in all sectors compared to non-smokers, except the foveal sector. Smokers had lower superficial vessel density in the superior parafoveal sector compared to non-smokers (p = 0.04). Considering deep vessel densities between smokers and non-smokers, no significant difference was observed. Radial peripapillary capillary densities (significant difference was observed in the whole image and infranasal peripapillary sector), Macular vascular densities (significant difference was observed in parafoveal sectors), and optic nerve head (not reaching statistical significance level in any sectors) were highest in passive smokers. CONCLUSIONS: Smoking is associated with increased total retinal thickness. Macular vascular densities were not different between smokers and non-smokers in TED.

Histological and ultrasonographic alterations in skin and soft tissue in upper eyelid excised blepharoplasty due to ipsilateral eyebrow tattooing: (preliminary findings).

BACKGROUND: Eyebrow tattooing (ET) is a relatively common cosmetic procedure for middle-aged women which can hide age-related ptosis. AIMS: We aimed to evaluate the periocular soft tissue changes following ET and its effects on upper eyelid blepharoplasty (UEBL). PATIENTS/METHODS: In this non-randomized prospective case-controlled study, 28 subjects recruited, 14 with ET for at least 5 years and 14 without ET. Eyebrows ultrasonography was performed to measure the periocular soft tissue thickness including skin and subcutaneous tissue on the medial and lateral side of the eyebrow. Then, UEBL was performed with extended eyelid skin incisions. So, the excised tissues being evaluated histopathologically. RESULTS: The mean age of patients was 50.6 ± 0.6 and 51.2 ± 5.59 years in non-eyebrow tattooing (NET) and ET groups, respectively (P = .78). In the ET group, soft tissue thickness was, respectively, equal to 5.90 ± 1.10 and 6.3 ± 0.95 mm on the lateral and medial side of the eyebrow, which were significantly thicker compared to the NET group (4.68 ± 0.69 and 4.78 ± 0.56 mm, respectively)(P = .001). Histopathological findings were ranging from edema-congestion to chronic inflammation and dermal fibrosis which were more frequently seen in ET group. However, this difference was statistically significant only for dermal fibrosis (P = .02). Surgical wound complications were observed in 3 patients who were in the ET group (P = .22). CONCLUSION: Subjects with eyebrow tattooing, as compared to a control group, showed a thicker eyebrow skin on ultrasonography and higher upper eyelid dermal fibrosis on histopathological examination.

Lacrimal Drainage System Problems After Rhinoplasty.

BACKGROUND: To report case series of permanent nasolacrimal system problems following rhinoplasty METHODS: The documents of patients with epiphora and history of rhinoplastic surgery were reviewed. The data of patients with permanent epiphora (continued over 3 months or started after 3 months of post-rhinoplasty surgery) and lacrimal drainage system (LDS) problems were analyzed for demographics, the result of diagnostic probing and irrigation, findings of orbital and paranasal sinuses CT scan, abnormalities in nasal endoscopy, treatment, and follow-up data. RESULTS: Forty-three patients with epiphora and history of rhinoplasty were referred to our clinic. Ten of them had permanent epiphora and LDS problems. In these patients, the mean time between rhinoplasty and initial symptoms of LDS problems was 10.3 ± 15.1 (range, 0-45 months) and the mean time between rhinoplasty and the LDS surgery was 22.2 ± 19.5 months (range 4-60 months). Diagnostic probing and irrigation test revealed canalicular stenosis in four (40%) patients, pus reflux in four (40%), clear reflux without passage in four (40%), and partial nasolacrimal duct obstruction (NLDO) in two (20%) of patients. DCR was performed in eight (80%) patients. Therapeutic probing and lacrimal intubation were performed in two (20%) patients. CONCLUSION: A permanent injury of the LDS is one of the important complications of the rhinoplastic surgery that should be managed based on the onset and duration of the symptoms and the location of the injury. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Clinical, Radiological and Histopathological Features of Patients With Lacrimal Gland Enlargement.

BACKGROUND: The purpose of this study was to describe the radiologic and histopathologic features of lacrimal gland in patients presenting with lacrimal gland enlargement. METHODS: We retrospectively retrieved the data of patients with lacrimal gland enlargement in Farabi Eye Hospital between 2012 and 2017. These data included demographics, the patients' facial photographs, orbital CT-scans, and histopathological findings of lacrimal gland biopsies. RESULTS: Forty-seven patients (15 men and 32 women) were enrolled in this study with a median age of 37.9 years (range, 15-79 years). Histopathologic diagnoses were chronic dacryoadenitis in 26 cases (55.32%), IgG4-related disease in 6 patients (12.77%), two cases of acute dacryoadenitis, two cases of non-necrotizing granulomatous inflammation, two cases of Non-Hodgkin's B-cell lymphoma, two cases of adenoid cystic carcinoma and two cases of mixed tumor (4.26% each), as well as one case of conjunctival epithelial cyst, and one case of benign lymphoid tissue and fibrofatty tissue (2.13%). In two samples (4.26%), biopsy revealed normal lacrimal glands. Interestingly, in two cases with relapsing lacrimal gland enlargement, different histopathologic diagnoses were found in biopsies taken from each lacrimal gland at different times. The average size of enlarged lacrimal glands was 19.67 mm × 7.06 mm on axial CT scan and 19.44 mm × 6.20 mm on coronal CT scan. CONCLUSION: Tissue biopsy is needed for diagnosis of lacrimal gland enlargement because it is difficult to distinguish the type of the lacrimal gland pathology based solely on clinical or radiological presentation.

Monocanalicular Intubation in Children with Incomplete Complex Congenital Nasolacrimal Duct Obstruction Older Than Five Years of Age.

PURPOSE: To evaluate the role of monocanalicular intubation (MCI) in congenital nasolacrimal duct obstruction (CNLDO) in children older than 5 years of age. METHODS: A retrospective case series study was done on children over 5 years of age diagnosed with CNLDO who underwent MCI. Success rates were evaluated subjectively by asking their parents about persistent symptoms and objectively using the dye disappearance test in clinical examination. RESULTS: A total of 43 eyes of 37 patients with a mean age of 7.42 ± 2.33 (range, 5-15 years of age) were included. The success rate was 60.46%, and the rate of secondary surgical intervention was 25% of all cases (61.11% of failed cases). CONCLUSION: Primary MCI maintains a reasonable success rate in incomplete complicated CNLDO regardless of age.

Improved Function of Orbicularis Oculi by Dynamic Transfer of Contralateral Orbicularis Oculi Muscle in Patients with Facial Palsy.

PURPOSE: The purpose of this study is to introduce the results of a new surgical technique in patients with complete facial nerve palsy using the dynamic muscle transfer of orbicularis oculi muscle (OOM) flap from the contralateral side. METHODS: This case series presents a new surgical technique in three patients with complete facial palsy and lagophthalmos who were unresponsive to other modalities. In this technique, a rectangular flap of OOM was dissected from the upper lid of fellow eye and transferred to the affected eye through a subcutaneous tunnel over the nasal bridge. The flap was divided into two halves for upper and lower lids. Each half was incised longitudinally to increase the length of the flap and cover the lateral part of the affected eyelids. RESULTS: Improvement in exposure keratitis, lagophthalmos, and other related symptoms was observed as soon as the 1st week after the surgery. Partial blinking recovered and the operation was uneventful. CONCLUSION: Muscle flap transfer technique using contralateral OOM for complete facial palsy can be considered as a helpful alternative in patients who are still symptomatic despite conventional treatment modalities.

Solitary neurofibroma of the orbit with intracerebral extension associated with ocular surface melanocytoma: a case report.

Solitary or isolated neurofibroma is uncommonly observed in the orbit. Neurofibromas typically involve peripheral nerves and occasionally the cranial nerves. A 29-year-old man presented with recent onset left eye proptosis and exotropia. Physical examination was positive for hyperpigmented lesions of the ipsilateral ocular surface and hard palate. Imaging revealed an infiltrative orbital mass with extension through superior orbital fissure into the brain. There was also bone defect of greater sphenoid wing. Medial orbitotomy was performed to obtain biopsies of the orbital mass and the pigmented ocular surface lesions. Histopathologic diagnosis of neurofibroma was confirmed for the former and melanocytoma for the latter. His symptoms and examinations remained stable during the follow up. This case is unique due to several features, including extensive intracerebral spread of orbital neurofibroma in a patient without definite diagnosis of neurofibromatosis type 1 and association with ipsilateral ocular surface melanocytoma and palatal pigmented lesions. ABBREVIATIONS: CT: computed tomography; GFAP: glial fibrillary acid protein; MRI: magnetic resonance imaging; NF-1: neurofibromatosis type 1.

Orbital Exenteration: A 23-year Report.

PURPOSE: Orbital exenteration is a psychologically and anatomically disfiguring procedure which indicated in some patients with malignant or progressive diseases of orbital and periorbital area. In this study, we reviewed 176 patients that underwent orbital exenteration. METHODS: This was a retrospective study of medical records from all patients who underwent orbital exenteration from March 1991 to March 2014 in oculoplastic department at an eye care center. Demographic data, diagnosis, site of primary involvement and technique of surgery were determined in patients. RESULTS: One hundred seventy-six cases of orbital exenteration were included that had documented histopathology. The age of patients ranged from 1 to 91 years (mean age ± standard deviation, 55.43 ± 27 years). Ninety-seven (55.11%) males and 79 (44.88%) females were included. Fifteen different tumors were identified. The most common indication was patients with basal cell carcinoma 49 (28%) followed by 41 (23.5%) squamous cell carcinomas, 35 (20%) retinoblastoma, and 13 (7%) adenoid cystic carcinomas. In total, adnexal malignancies were the most common tumors, secondarily involving the orbit. Eyelids 89 (50.5%) and the globe 43 (24%) were the most frequent site of involvement. Three types of exenteration were performed, based on available data of 129 operation sheets, 46 (35.7%) subtotal, 62 (48.1%) total, and 21 (16.3%) cases of extensive exenterations. In total 97 cases were evaluated pathologically for perineural involvement, of which perineural invasion was noted in 9 (7%) reports. CONCLUSIONS: Frequency of exenteration in our center has increased in past 3 years and the majority of cases were eyelid basal cell carcinoma. Patient education considering periocular lesions can help in earlier diagnosis of malignant lesions and therefore reducing the number of exenteration.

A Rare Case of Congenital Orbital Cystic Neuroblastoma in an Infant.

Neuroblastoma, a relatively common tumor in infants, is rarely present at birth. A solid mass is the typical appearance of this neoplasm, while cystic formation is uncommon. Congenital cystic neuroblastoma is extremely rare and no previous report exists of its occurrence in the orbit. A newborn girl was referred with an extremely large mass on the right side of her face on the third day of her life. Prenatal ultrasonography had revealed a large mass (80 × 80 mm) in the frontal area. MRI revealed a heterogeneous mass composed of cystic and solid, contrast-enhancing components. Excisional biopsy was successfully performed and histopathologic diagnosis of neuroblastoma was confirmed. Systemic workup was negative for involvement of other organs and levels of catecholamine metabolites of serum and urine (homovanillic acid and vanillylmandelic acid) were normal. Postoperative course was uneventful with complete healing of the anophthalmic socket and normal physical and mental growth and development of the infant. To our knowledge, this is the first report of congenital cystic neuroblastoma primarily arising in the orbit.

Clinical and Ultrasonographic Evaluation of Infantile Periocular Hemangioma Treated With Oral Propranolol.

PURPOSE: To evaluate the clinical and ultrasonographic response of periocular infantile capillary hemangioma during treatment with oral propranolol. METHODS: Patients with infantile periocular hemangioma and visual or cosmetic concerns were enrolled in this prospective interventional case series. Propranolol was given at a dose of 2 mg/kg per day for at least 6 months. Evaluation of treatment response was performed at month 3 (time point 1) and month 6 (time point 2). Gray scale ultrasonography and color Doppler imaging were performed at baseline and month 3. RESULTS: Thirty-one patients with mean age of 4.1 ± 2.3 months were eligible for analysis. Complete or near complete clinical resolution was observed in 4 patients (12.9%) at time point 1 and 21 patients (67.7%) at time point 2. Longitudinal diameter, transverse diameter, thickness, arterial peak systolic velocity, and end diastolic velocity reduced significantly from baseline to 3-month follow up. Complete clinical response at time point 2 was significantly higher in patients with peak systolic velocity reduction >50% from baseline to month 3 than patients with peak systolic velocity reduction of 10% to 50% and <10%. CONCLUSIONS: Propranolol is safe and effective for infantile periocular hemangioma. Ultrasonography and color Doppler imaging are useful modalities to monitor and predict the treatment response.

Successful Total Resection of an Orbital Epithelioid Hemangioendothelioma with the Aid of Endovascular Embolization.

Hemangioendothelioma is rarely encountered in the orbit. We present a patient with a growing orbital mass for whom surgical excision was planned. Two previous attempts at removing the mass failed due to profuse bleeding. Endovascular embolization was performed before surgery to prevent massive hemorrhage. After embolization, retinal vascular accident (combined central retinal artery and vein occlusion) occurred. However, surgical excision (lateral orbitotomy and transcoronal craniotomy) was successful, and the vision improved postoperatively. Histopathologic examination and immunohistochemistry study confirmed the diagnosis of epithelioid hemangioendothelioma. The combined approach by a team of specialists, including an interventional radiologist performing preoperative embolization of the feeding vessel and joint surgery by a neurosurgeon and oculoplastic surgeon, was the key to the effective treatment of this vascular orbital neoplasm.