scholarly journals Primary orbital ganglioneuroblastoma: A case report

Open Medicine ◽  
2021 ◽  
Vol 16 (1) ◽  
pp. 1076-1081
Author(s):  
Ruixin Ma ◽  
Yujiao Wang ◽  
Weimin He

Abstract Background Ganglioneuroblastoma (GNB) is a neoplasm that arises from the primitive cells of the sympathetic nervous system during childhood. The current case is very unique because of the initial primary tumor manifestation in the orbit and an adrenal tumor being observed later during follow-up. Case presentation A 2-year-old girl presented to the Ophthalmology Department of our hospital complaining of swelling of the left upper eyelid for approximately one month. Orbital computed tomography (CT) revealed a left orbital mass with bone destruction. Thoracic and abdominal CT indicated no abnormalities. The mass was surgically resected, and histopathological analysis confirmed it as GNB. During follow-up, abdominal CT detected an adrenal tumor with internal calcification, a calcified nodule on the left side of the abdominal aorta, and mesenteric lymph nodes. Accordingly, primary orbital GNB and metastatic adrenal GNB were the possible considerations. We removed the adrenal tumor, and the patient underwent chemotherapy. However, the patient died 18 months after the ophthalmic surgery. Conclusion Primary orbital GNB in children is easily misdiagnosed because of its rare occurrence and atypical clinical findings. Imaging methods combined with histopathological examination contribute to the detection and diagnosis of primary and metastatic GNBs. Thus, timely surgery combined with adjuvant chemotherapy and long-term follow-up is essential for controlling the metastasis of GNB and improving the survival rate of patients.

2015 ◽  
Vol 16 (2) ◽  
pp. 203-206 ◽  
Author(s):  
David Krahulík ◽  
Marta Karhanová ◽  
Miroslav Vaverka ◽  
Světlana Brychtová ◽  
Dagmar Pospíšilová

Ectopic cilia are extremely rare congenital anomalies in which eyelash follicles appear in an abnormal place on the eyelid, most typically on the lateral quadrant of the anterior surface of the upper eyelid. In the majority of cases, simple surgical excision of ectopic cilia is indicated because of its cosmetic aspect. There is usually no associated medical co-morbidity with this anomaly. The authors report an unusual case of ectopic cilia associated with an orbital dermoid cyst and sinus tract. A 3-year-old boy was initially diagnosed with ectopic cilia on the left upper eyelid. There was no history of inflammation or swelling of the eyelid. An ophthalmological examination revealed only 1 mm of ptosis; no proptosis, inferior displacement, or palpable orbital mass was present. During surgical excision of the ectopic cilia, a thin sinus tract was identified, leading posteriorly to the orbit. Magnetic resonance imaging performed after the excision showed a supraorbital extraconal mass just below the roof of the left orbit. A supraorbital 2-piece craniotomy was performed with total extirpation of the dermoid cyst. The cyst was removed en bloc without damage to the extraocular muscles, but the sinus tract could no longer be identified. Follow-up MRI was performed 6 months after surgery and showed no evidence of recurrence. A follow-up ophthalmological examination showed no signs of inferior displacement or proptosis. To the best of the authors’ knowledge, this case is the first reported instance of ectopic cilia associated with a dermoid cyst and sinus tract in which no typical clinical signs and symptoms of possible orbital pathology were present. This case highlights the value of radiological examination in all cases of ectopic cilia prior to surgical excision.


2017 ◽  
Vol 86 (9-10) ◽  
Author(s):  
Igor Šivec Trampuž ◽  
Dragica Kosec

Idiopathic orbital inflammatory disease, previously referred to as orbital pseudotumor, is a nonneoplastic, non-infective disease. The inflammatory process can involve one or more orbital sof tissues; extraocular muscles, tear gland, scleralorepiscleral tissue or orbital fat. Orbital myositis typically presents itself with a sudden onset of restricted ocular motility, diplopia, congestive proptosis, eyelid ptosis, periocular swelling and conjunctival hyperemia. A thorough workup is essential for ruling out other entities.This report presents a case of a 47-year old man with a sudden onset of right upper eyelid swelling and diplopia. Until then he had no health problems. Afer initial ophthalmologic workup and ultrasound an orbital mass was suspected. Te MRI and a biopsy of the mass confirmed an isolated superior oblique muscle myositis. We treated him with systemic steroids. A good response and fast regression were observed. In two years of follow-up visits no recurrence was noted.Isolated superior oblique muscle myositis is extremely rare. This is one of eleven cases documented between 1988 and 2015. This case report presents a 47-year old man with a sudden onset of right upper eyelid swelling and diplopia. Until then he had no health problems. After initial ophthalmologic workup and ultrasound an orbital mass was suspected. The MRI and a biopsy of the mass confirmed an isolated superior oblique muscle myositis. We treated him with systemic steroids. A good response and fast regression was observed. In two years of follow-up visits no recurrence was noted.Isolated superior oblique muscle myositis is extremely rare. This is one of eleven documented cases between 1988 and 2015.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Yun Hsia ◽  
Huang-Chun Lien ◽  
I-Jong Wang ◽  
Shu-Lang Liao ◽  
Yi-Hsuan Wei

Abstract Background Epibulbar complex choristoma, a rare congenital epibulbar tumor, has many diverse forms. Reviewing the literature, it can present clinically as either a circumferential or isolated epibulbar mass, limbal tumor, lateral canthal mass, aggregate of ectopic cilia in the upper eyelid, eyelid mass mimicking chalazion, or lacrimal caruncle mass. The management depends on the extent of involvement, the risk of amblyopia, and cosmetic concerns. Here, we report an atypical presentation of epibulbar complex choristoma with simultaneous eyelid involvement. Case presentation A 1-month-old full-term boy was brought to our clinic with congenital epibulbar mass of the right eye with simultaneous eyelid involvement. Dilated fundus examination was unremarkable. Survey for linear nevus sebaceous Jadassohn was negative. Due to concerns of possible amblyopia and cosmetics, lamellar keratectomy, sclerotomy, and conjunctivoplasty were performed to remove the epibulbar lesion. The eyelid defect was reconstructed with 6–0 Vicryl sutures. Histopathological examination reported complex choristoma. Upon three-year follow-up, low astigmatism and favorable cosmetics results were achieved. Conclusions Congenital complex choristoma can present clinically as an epibulbar mass with eyelid involvement. The management depends on the extent of involvement, the risk of amblyopia, and cosmetic concerns. The method of eyelid reconstruction should be tailored according to the residual eyelid defect.


2017 ◽  
Vol 89 (2) ◽  
pp. 62-65 ◽  
Author(s):  
Arkadiusz Spychała ◽  
Piotr Nowaczyk ◽  
Aleksandra Budnicka ◽  
Ewa Antoniewicz ◽  
Dawid Murawa

The article presents a case report of a patient with an intramural gastric hematoma. Diagnostic examinations were suggestive of a suspected gastrointestinal stromal tumor Normal image was observed in gastroscopic examination while abdominal CT scan revealed a nodular lesion along the greater curvature of the stomach extending from the posterior wall and adjoining the pancreas and the spleen. The patient was qualified for surgical treatment. Laparotomy was performed followed by total gastric resection and Roux-en-Y reconstruction of the gastrointestinal tract. Post-operative histopathological examination revealed the presence of an extensive hematoma penetrating the perigastric fat tissue along with numerous hemosiderinophages and segmental indicators of formation of inflammatory granulation tissue suggestive of a chronic nature of the lesion. Immunohistochemical GIST assays (CD117, DOG-1, CD34, CD31, SMA, S-100, CKAE1/AE3, Ki-67) were negative. No complications were observed in the post-operative course. Patient is subject to continued follow-up and observation. Follow-up gastroscopy and abdominal CT scan performed 6 months after the surgery revealed an unremarkable image.


2011 ◽  
pp. 100-104
Author(s):  
Thi Thu Nguyen ◽  
Viet Hien Vo ◽  
Thi Em Do

The study use intralesional triamcinolone acetonide injection proceduce for chalazion treatment.1. Objectives: To evaluate results of intralesional triamcinolone acetonide injection for chalazion treatment. 2. Method: This noncomparative prospective interventional trial included 72 chalazions of 61 patients. 3. Results: 61 patients (72 chalazions) with 19 males (31.1%) và 42 females (68.9%), the mean age was 24 ± 9,78 years. 31.1% patients was the first time chalazion and 68.9% patients was more than one times chalazion including 78.6% patients was recurrent at the first position and 21.4% patients occur at new position. 72 chalazions with 16 (22.2%) chalazions was treated before and 56 (77.8%) chalazions wasn’t done that. 72 chalazions with 49 chalazions (68.1%) are local in upper eyelid and 23 chalazions (31.9%) are local in lower eyelid. The mean of chalazion diameter is 6.99 ± 3.03mm. Intralesional triamcinolone acetonide is injected to treat 72 chalazions with 16 (22.2%) chalazions are injected through the route of skin and 56 (77.8%) chalazions are injected through the route of conjunctiva. After 2 weeks follow-up, the success rate was 93.1% and 6.9% failed. 4. Conclusion: intralesional triamcinolone acetonide injection for chalazion treatment is really effective. Key words: chalazion, intralesional triamcinolone acetonide.


2021 ◽  
Vol 108 (Supplement_3) ◽  
Author(s):  
G Martínez Izquierdo ◽  
A R Arnaiz Pérez ◽  
E Escolano Fernández ◽  
M Merayo Álvarez ◽  
B Carrasco Aguilera ◽  
...  

Abstract INTRODUCTION Renal cell carcinoma (RCC) represents 3% of overall malignant neoplasms in adults. However, its aetiology has not been clearly established. Although surgery represents the cornerstone in treatment, recurrence postoperative rates are around 20-30%, what implies prognostic factors search must be mandatory in order to help to plan de follow-up and the different adjuvant therapy possibilities available in case they were necessary. MATERIAL AND METHODS A retrospective observational study was carried out in 110 patients who underwent radical nephrectomy between 2004 and 2018, with the aim of identifying possible prognostic factors of recurrence of RCC after these surgeries. Preoperative data (epidemiological, comorbidities and laboratory tests), surgical, pathological and variables related to follow-up were taken into account. A univariate and multivariate analysis were performed, using chi-square test and logistic regression, respectively. RESULTS The median follow-up time was 53.5 months (SD = 35.8), time in which 19 patients had a recurrence of RCC after radical nephrectomy (17.2%). Histopathological items such as the surgical piece size, the nodal and microvascular invasion, the renal sinus invasion and the presence of necrosis in the surgical piece were associated with RCC recurrence in the univariate analysis, while only the presence of necrosis in the surgical piece showed a significant result in the multivariate analysis (p = 0.004). CONCLUSIONS Histopathological analysis, highlighting the presence of necrosis in the histological sample, was proved to be the main risk factor of RCC recurrence.


2021 ◽  
Vol 54 (01) ◽  
pp. 058-062
Author(s):  
Pawan Agarwal ◽  
Dhananjaya Sharma ◽  
Vikesh Agrawal ◽  
Swati Tiwari ◽  
Rajeev Kukrele

AbstractBackground The purpose of this study was to evaluate the functional outcomes of a modified technique of double rectangle pattern for correction of severe ptosis.Methods This is a retrospective study over a period of 8 years including patients who underwent correction of ptosis by double rectangle using autologous fascia lata sling. Surgical outcomes were assessed postoperatively by distance from the corneal light reflex to the upper eyelid margin (MRD1) and levator function.Results Twenty-six eyelids were operated in 20 patients. There were 9 males and 11 females, with age ranging from 4 to 35 years. Preoperatively, all patients had poor MRD1 and poor levator function. Postoperative MRD1 was good in 13 patients (17 eyelids), fair in 5 (7eyelids), and poor in 2 patients (2 eyelids). Postoperative levator function was excellent in 12 patients (15 eyelids), good in 6 (9 eyelids), and fair in 2 patients (2 eyelids). At a mean follow-up of 12 months, adequate correction was achieved in 24 eyelids, and 2 eyelids had undercorrection.Conclusion Frontalis sling with a double rectangle is simple and more efficient, as it provides a straight line of pull to the eyelid for correction of severe ptosis.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zhicheng Zhang ◽  
Xiaowei Huang ◽  
Qian Chen ◽  
Demin Li ◽  
Qi Zhou ◽  
...  

Abstract Background Small intestine duplication cysts (SIDCs) are rare congenital anatomical abnormalities of the digestive tract and a rare cause of hematochezia. Case presentation We describe an adult female presented with recurrent hematochezia. The routine gastric endoscope and colonic endoscope showed no positive findings. Abdominal CT scan indicated intussusception due to the "doughnut" sign, but the patient had no typical symptoms. Two subsequent capsule endoscopes revealed a protruding lesion with bleeding in the distal ileum. Surgical resection was performed and revealed a case of SIDC measuring 6 * 2 cm located inside the ileum cavity. The patient remained symptom-free throughout a 7-year follow-up period. Conclusion SIDCs located inside the enteric cavity can easily be misdiagnosed as intussusception by routine radiologic examinations.


2021 ◽  
pp. 112067212110071
Author(s):  
Vijitha S Vempuluru ◽  
Monalisha Pattnaik ◽  
Neha Ghose ◽  
Swathi Kaliki

Purpose: To describe the risk factors, clinical presentation, management, and outcomes of patients with bilateral ocular surface squamous neoplasia (OSSN). Methods: Retrospective case series. Results: Of the 25 patients with bilateral OSSN, the mean age at diagnosis of OSSN was 31 years (median, 24 years; range, 2–60 years). Risk factors for bilateral OSSN included xeroderma pigmentosum ( n = 15, 60%), human immunodeficiency virus infection ( n = 3, 12%), conjunctival xerosis ( n = 1, 4%), and topical steroid use ( n = 1, 4%). There were no identifiable ocular or systemic risk factors in 7 (28%) patients. Presentation was synchronous in 14 (56%) and metachronous in 11 (44%) patients. Tumor morphology was bilaterally similar in 12 (48%) patients. Histopathological examination ( n = 36) revealed conjunctival intraepithelial neoplasia (CIN) grade 1 in 4 (8%); grade 2 in 7 (14%); carcinoma in situ in 5 (10%), and invasive carcinoma in 20 (40%). Primary management of OSSN ( n = 49) included excisional biopsy ( n = 31, 62%), topical immunotherapy (IFN α2B) ( n = 11; 22%), topical Mitomycin C (MMC) ( n = 3, 6%), enucleation ( n = 1, 2%), orbital exenteration ( n = 2, 4%), and plaque brachytherapy (PBT) ( n = 1, 2%). One patient was lost to follow-up after detection of tumor in the second eye. Recurrent tumors were noted in 16 (32%) eyes and binocular globe salvage was achieved in 16 (64%) patients at a mean follow up of 41 months (median 30 months; range, 1–164 months). Conclusion: OSSN occurrence can be synchronous or metachronous. Meticulous examination of the fellow eye is important for an early diagnosis of OSSN.


2021 ◽  
Vol 10 (10) ◽  
pp. 2086
Author(s):  
Luís Mota ◽  
Ricardo Jorge Dinis-Oliveira

Argyria encompasses the different cosmetic alterations that can develop if enough silver particles deposit in a specific tissue, typically in the skin, ranging from localized dark-blue macules to a generalized slate-gray/bluish tinge following systemic absorption. This work aims to fully review the state of the art regarding pathophysiology, diagnosis, treatment, and relevant clinical and forensic features of argyria. Argyria has been diagnosed in a wide range of ages, both sexes and varied ethnicities, with no known individual predisposing factors. Ultraviolet radiation with subsequence increases of melanin production aggravates the discoloration due to a reduction in the silver deposits. Physical examination and silver exposure in the anamnesis can be highly suggestive of the diagnosis, but a histopathological analysis with Energy-Dispersive X-ray Spectroscopy is required to unequivocally determine the discoloration etiology. Safe and effective treatment has only been accomplished with laser techniques, though only a few cases have been reported and with limited follow-up time. In conclusion, argyria typically has an occupational or iatrogenic etiology. It should be suspected when a patient presents with typical skin or eye lesions. A seemingly viable treatment modality, with laser technology, is finally within the horizon.


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