Proptosis In Children Research Articles

Proptosis in children caused by sinonasal pathology: A retrospective study

Proptosis in children caused by sinonasal pathology: A retrospective study

PROPTOSIS IN ACUTE MYELOID LEUKEMIA: AN UNDER RECOGNIZED PRESENTATION OF HEMATOLOGICAL MALIGNANCY

OBJECTIVE: To determine the frequency of proptosis and associations of proptosis with demographic, clinical and hematological characteristics with acute myeloid leukemia (AML) in children. METHODS: This descriptive study was conducted at Combined Military Hospital, Rawalpindi, Pakistan from January 2018 to July 2020. Patients of AML between 1-12 years of age who presented with and without orbital granulocytic sarcomas (OGS), selected by non-probability convenience sampling technique were evaluated for proptosis. Patients were classified into different subtypes of AML according to French-American-British (FAB) classification. Cytogenetic studies by karyotyping were done to identify different genetic abnormalities associated with AML. Kaplan- Meier survival analysis was used to analyze the overall survival of AML patients. RESULTS: Out of 230 patients diagnosed with AML, 34 (14.78%) patients presented with proptosis. Mean age of presentation was 6.80±3.69 years, with male-female ratio of 2.1:1. Proptosis was unilateral in 19 (55%) and bilateral in 15 (45%) patients. Ten (29.40 %) patients presented primarily with proptosis while 24 (71%) patients presented with proptosis and systemic features of leukemia. Overall AML-M2 was found in 102 (44.3%) cases and other types were observed in 128 (55.6%) cases. Most common FAB AML subtype associated with proptosis was AML-M2 (n=26; 76%). Median duration of survival in AML patients with OGS was 867 days and as compared to 353 days in AML patients without OGS. CONCLUSION: Proptosis is a frequent finding in children with AML. AML-M2 is associated with proptosis in children with AML. Survival in patients with OGS was better than patients without OGS.

Optic nerve glioma – a case study

AbstractPurposeTo evaluate a 5 year old boy with painless progressive enlargement of the left eye since the past 1 year. He also complained of a loss of vision in the left eye.MethodsStudy Design: Case Study.Duration of Study: 1 year.The patient was evaluated over 1 year and investigations were carried out as deemed necessary.InvestigationsSlit lamp biomicroscopy with 90 D Volk lens was done for anterior and posterior segment examination. Gonioscopy, Applanation Tonometry, Automated Perimetry and Indirect Ophthalmoscopy were done. CT and MRI of the orbit was also carried out.ResultsPresenting visual acuity was absence of light perception in the left eye and the proptosis was measured of 25 mm which was in the downward and outward direction. A relative afferent pupillary defect was noticed in the left eye and extraocular movements were restricted superiorly and medially. Funduscopy of the left eye revealed a pale disc, blurring of the disc margins and presence of optociliary shunt vessels. CT scan of the left eye showed a fusiform optic nerve swelling. MRI showed a fairly large fusiform swelling in left optic nerve, pressure effect on ocular bulb with proptosis, no extension of the lesion in optic canal. This was consistent with the diagnosis of optic nerve glioma.ConclusionOptic nerve glioma is an important cause of unilateral proptosis in children. Early diagnosis is mandatory and management should involve an integrated approach of neurosurgeons, plastic surgeons, ophthalmologists and radiologists.

Primary sinonasal meningioma: An uncommon cause of proptosis in children

Extracranial primary sinonasal meningioma is of a very uncommon occurrence. It can give rise to myriad of nasal symptoms, but proptosis as a result of the same is relatively lesser known. The definitive diagnosis is made only on histological examination. The importance of complete surgical removal is indubitable. Our case report citing the rarity of this tumor involving primarily the sinonasal cavities, leaving the brain undisturbed adds to the already small number of cases reported globally.

English

AIMS: To analyse the various causes for proptosis in Children. SETTINGS AND DESIGN: Prospective analytical study. METHODS AND MATERIAL: A prospective analysis of 50 cases of proptosis in children less than 15 years. Detailed Ocular and Systemic history, examination and relevant investigations were done in necessary cases. Also other related specialties opinion obtained whenever indicated for diagnosis and treatment. Statistical analysis used: Descriptive analysis. RESULTS: Out of 50 cases 32 were axial and 18 were eccentric proptosis. Incidence of proptosis in male was 60% and female 40%. Etiology of proptosis due to inflammation 19 cases, neoplasm 15 cases and others 8. CONCLUSIONS: Orbital cellulitis is the single most common cause for proptosis in children. Among malignancy secondaries are common than the primary tumors in the orbit. Orbital X- ray, B-Scan, CT Scan and MRI were helpful in diagnosis, treatment and follow up.

Changes of ocular signs in children with thyroid diseases after intake of bee bread

The objective of the study was to investigate visual acuity, refractive status and eye proptosis in children with thyroid diseases before and after intake of bee bread. There were done investigations of visual acuity, refraction and eye proptosis in 34 children with thyroid diseases at Eye Clinic of Lithuanian University of Health Sciences Medical Academy (age range from 6 to 17 years). 18 patients used bee bread and there was performed low frequency pulsed electromagnetic field therapy. For 16 subjects electromagnetotherapy alone was performed. The values were statistically analyzed with the pretreatment values after three week course of treatment. In the present study we observed an improvement of visual acuity in children with thyroid diseases who used bee bread and there was performed magnetotherapy in comparison with those who were treated magnetotherapy alone. In our study exophthalmometer values and refraction power were not significant changed in both groups. Data analysis indicated increase in visual acuity for subjects who used bee bread and pulsed electromagnetic field therapy to subjects who did not used bee bread. The data we have found suggest that bee bread and magnetotherapy had an influence on visual acuity in children with thyroid diseases.DOI: http://dx.doi.org/10.5755/j01.ct.61.3.2660

Assessment of Visual Acuity, Refraction Changes, and Proptosis in Different Ages of Patients with Thyroid Diseases

Objective. The aim of the study was to assess visual acuity, refractive status, and eye proptosis in children and young adults with Graves' disease. Material and Methods. We have done investigations of visual acuity, refraction, and eye proptosis in 16 children, 14 teenagers, and 49 adults with Graves' disease at Eye Clinic of Lithuanian University of Health Sciences Medical Academy. Data were compared with 14 children, 14 teenagers, and 35 adults of similar age without the same diseases (control group). Results. In the present study we observed a significant decrease of visual acuity in teenagers (0.73 ± 0.18, P = 0.001) and adults (0.7 ± 0.16, P = 0.001) with Graves' disease. Myopia was ascertained more frequent in teenagers (42.8 percent) and adults (44.9 percent) with Graves' disease. In our study exophthalmometer values were higher in children (15.94 ± 1.98 mm, P = 0.003), teenagers (17.28 ± 2.99 mm, P = 0.01), and adults (18.05 ± 2.91 mm, P = 0.001) in comparison with the controls. Conclusions. The data we have found suggest that Graves' disease in children, teenagers, and adults has influence on vision acuity, refraction, and eye proptosis.

Periorbital (preseptal) cellulitis in children

Infections of the eye can be periorbital (preseptal) or orbital in origin. The management of children with periorbital cellulitis varies with the clinical presentation. Some children have trivial or self-limited disorders, while others can have sight- or life-threatening problems. Thorough physical examination helps the clinician to differentiate among the causes of periorbital cellulitis and between periorbital and orbital cellulitis. The differential diagnosis of periorbital cellulitis includes conjunctivitis, hordeolum, dacryocystitis, dacryoadenitis, bacteremic cellulitis, traumatic cellulitis and inflammatory edema secondary to sinusitis. Orbital cellulitis, although very much less common than periorbital cellulitis, is the most common cause of unilateral proptosis in children.

Letters to the Editor

30 August 2007 Dear Editor, ORBITAL MYOSITIS FOLLOWING STREPTOCOCCAL PHARYNGITIS Idiopathic orbital myositis is an inflammatory process involving the extra ocular muscles. It not only occurs most frequently in young to middle-aged adults with a 2:1 female predominance, but also occurs in children. The cause is unknown, however, it has been considered that an immunopathogenic mechanism is likely.1 There have been two previously reported cases in a 36-year-old woman2 and a 13-year-old boy where orbital myositis has followed streptococcal pharyngitis.3 We now report a third case. A 9-year-old girl presented with a 40-h history of pain on movement of the right eye and several hours of swelling and purplish discolouration of the right upper eyelid. She had diplopia on lateral gaze. An initial diagnosis of periorbital cellulitis was made and she was treated with 3 days of intravenous cefotaxime and then discharged on oral cephalosporin. She re-presented 3 days after discharge with photophobia and continuing pain on eye movement. On examination, there was slight proptosis of the right eye. The right upper eye lid was swollen and there was diplopia on extreme lateral gaze. Her visual acuity was normal. Six weeks prior to the presentation, she had had an episode of severe tonsillitis with a right-sided tonsillar abscess which had been treated in hospital with intravenous penicillin for 5 days. Investigations at the second presentation showed a white cell count of 18.6 × 109/L, 14.1 × 09/L neutrophils, ESR 47, ASOT 560 iu/mL (normal < 200), anti-DNAse B 160 (normal < 20). Creatinine phosphokinase and thyroid function tests were normal. A computerized tomography (CT) scan of the orbits showed a uniform thickening of the right medial rectus muscle which enhanced after contrast administration (Fig. 1). The paranasal sinuses were normal. Computerized tomography scan showing enlargement of R medial rectus muscle. Treatment was commenced with oral prednisolone 1 mg/kg and within 48 h of starting prednisolone she was afebrile and the eyelid oedema had resolved. The steroid dose was gradually reduced over 8 weeks. On review at 4 weeks, she had no ocular symptoms. The clinical findings are typical of orbital myositis with painful eye movement, diplopia, mild proptosis and some periorbital oedema. The CT scan showed the typical muscle enlargement in the medial rectus. Orbital myositis has been described following upper respiratory tract infections.2 There are two previous cases of orbital myositis following serology or culture proven streptococcal pharyngitis. The first was a 36-year-old woman who developed periorbital myositis 2 weeks after culture proven streptococcal pharyngitis.2 There was a rapid resolution of symptoms in 5 days following institution of oral prednisolone. The second was a 13-year-old boy who developed orbital myositis in the lateral and medial recti muscles following streptococcal pharyngitis. He was treated with non-steroidal anti-inflammatory drug therapy and the symptoms resolved approximately 7 weeks after presentation.3 There are a number of post-infective complications recorded following Group A streptococcal pharyngitis which are considered to have an immunopathogenic basis. In addition to rheumatic fever and glomerulonephritis, these include post-streptococcal reactive arthritis,4 paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections,5 uveitis, erythema nodosum, tenosynovitis of the superior oblique tendon sheath and polymyalgia. Post-streptococcal polymyalgia consists of muscle pain, fever, raised antistreptolysin O titres, neutrophilia, raised inflammatory markers and normal muscle enzymes.6 Myopathic abnormalities have been observed on electromyography.6 The fever and tenderness associated with post-streptococcal polymyalgia improves within a few days of treatment with prednisolone and somewhat more slowly following non-steroidal anti-inflammatory medication. This clinical course and response to medication is similar to that observed in the present case and the other two reported cases of orbital myositis following streptococcal pharyngitis. The occurrence of polymyalgia indicates that post-streptococcal inflammatory syndromes can involve skeletal muscle tissue. This case highlights that orbital myositis is a cause of painful eye movements and mild proptosis in children and provides further support for the possibility that an immune-mediated post-streptococcal inflammation of ocular muscle is one cause.

Allergic fungal sinusitis in children

Allergic fungal sinusitis (AFS) has been characterized in adults presenting with chronic sinusitis. Rare reports allude to a similar disease in children. To characterize the features of AFS in children. Children referred to otolaryngology clinics at Arkansas and LeBonheur Children's Hospitals for chronic sinusitis during a 12-year period were studied. This retrospective analysis reviews the following: clinical presentation, laboratory evaluations, radiographic and pathologic findings, and surgical intervention. Twenty patients (age range, 7-18 years; mean age, 12.5 years; median age, 16 years) met previously published criteria for AFS. Thirteen patients were male and 7 were female. Thirteen were African American and 7 were white. Presentation at diagnosis included the following: atopy (n = 20), nasal symptoms (n = 20), recurrent sinusitis (n = 18), nasal polyps (n = 18), recurrent headaches (n = 12), asthma (n = 11), proptosis (n = 10), and ocular symptoms (n = 10). All had radiographic evidence of sinusitis and allergy to fungal organisms. IgE levels were elevated in 8 of 9 patients, and 10 of 15 patients had eosinophilia. Surgical specimens demonstrated allergic mucin (n = 11), Charcot-Leyden crystals (n = 2), hyphae or fungal debris (n = 9), and fungal growth (n = 17). All patients underwent endoscopic sinus surgery, with 11 requiring multiple surgical procedures. Postoperatively, 19 patients received intranasal and oral steroids, and all had nasal saline washes. Eleven patients (9 who had undergone multiple surgical procedures) were treated with immunotherapy. Relapse was seen in 55% of patients at 1 year of follow-up. AFS presents with a higher incidence of proptosis in children when compared with adults. Typically, AFS occurs in atopic children with refractory sinus disease, requiring a high index of suspicion for evaluation and aggressive treatment.

Pleomorphic adenoma of the lacrimal gland in a nine‐year‐old child

A case of a 9-year-old child with pleomorphic adenoma of the lacrimal gland is described. This tumour is usually found in adults; cases younger than 10 years of age have proved to be extremely rare. Because of its age distribution, pleomorphic adenoma of the lacrimal gland is usually overlooked as a possible cause of unilateral proptosis in children. Also, the history of disease provided by the family may be incorrect, possibly leading to misdiagnosis by the ophthalmologist.

Bilateral orbital cellulitis due to Neisseria gonorrhoeae and Staphylococcus aureus: a previously unreported case

Cellulitis of the orbit is a common cause of proptosis in children, and also frequently arises in the elderly and the immunocompromised. The condition is characterized by infection and swelling of the soft tissues lining the eye socket, pushing the eye ball outwards and causing severe pain, redness, discharge of pus and some degree of blurred vision. There is a small risk of infection spreading to the meninges of the brain and causing meningitis. This paper reports the case of an adult in whom polymicrobial bilateral orbital cellulitis had developed due to Staphylococcus aureus and Neisseria gonorrhoeae infection. N. gonorrhoeae infections are acquired by sexual contact. Although the infection may disseminate to a variety of tissues, it usually affects the mucous membranes of the urethra in males and the endocervix and urethra in females. To the authors' knowledge this is the first report of polymicrobial bilateral orbital cellulitis due to S. aureus and N. gonorrhoeae in medical literature.

Aetiology of childhood proptosis.

To retrospectively analyse causes of childhood proptosis and their investigations and treatment. The records of children under 15 years of age presenting with proptosis to The Children's Hospital, Camperdown, Sydney, were reviewed for the period 1983-93 inclusive. Fifty-seven cases of proptosis were found. In order of frequency the causes were; orbital cellulitis 22, thyroid eye disease 8, optic nerve +/- optic chiasm glioma 8, orbital rhabdomyosarcoma 7, metastatic neuroblastoma 4, orbital neurofibroma 3, orbital haemangioma 2, metastatic Ewing's sarcoma 2 and orbital dermoid cyst 1. Treatment depended on the cause (antibiotics, antithyroid drugs, chemotherapy, radiotherapy, surgery) and was multidisciplinary. The most common cause of proptosis in children presenting to The Children's Hospital, Camperdown, Sydney, was infective orbital cellulitis. The most useful initial investigation was an orbital computed tomography scan. Treatment depended on the cause of the proptosis and was multidisciplinary.

Aetiology and management of proptosis in young Jordanians

Sixty-two cases of proptosis in children and adolescents were prospectively evaluated in a specialized practice at Irbid, North Jordan, during the period between 1990 and 1994. Inflammatory orbital conditions were seen in 27 patients (43.5%). The causes of orbital cellulitis were identified in 15 out of 25 cases. Predisposing factors and outcome were analyzed. Vasculogenic lesions were encountered in 15 cases, five of which were subperiosteal orbital haematomas. Twelve cases with structural lesions ranging from a benign cystic dermoid to craniostenosis were managed in various ways. A rare case of primary hydatid disease in the orbit was also seen. Results of surgical treatment and outcome in each condition were assessed. Eight cases (14%) were neoplastic in nature. A rare case of retinoblastoma which presented as orbital cellulitis is included. Orbitotomies were performed for excision biopsy, mass removal and decompression or exenterations of the orbit, with no serious complications. No cases of proptosis owing to thyroid ophthalmopathy or systemic diseases were seen. The relatively low frequency of secondary orbital retinoblastoma among Jordanian children is compared with that in related reports from other countries.

Acute proptosis due to Burkitt's lymphoma

Three cases of acute proptosis in children are presented. The provisional clinical diagnosis was rhabdomyosarcoma and the histopathology revealed Burkitt's lymphoma. The fast growth rate, hematologic manifestations and the urgency of diagnosis are emphasized.

8. Orbital Cellulitis

Orbital cellulitis secondary to sinusitis has been the most common cause of proptosis in children. A case report is presented which emphasizes that the signs and laboratory findings in orbital Cellulitis may be confusing, especially when modified by prior antibiotics. When orbital cellulitis is a possibility, adequate antibiotic therapy should be instituted while diagnostic studies are being performed. CT scanning is a new and useful modality for the evaluation of these patients, but cautious and experienced interpretation is essential. In patients who do not respond promptly to appropriate medical therapy, surgical intervention is indicated for drainage and biopsy.

Orbital Tumors, by John W. Henderson, M.A. (Anatomy), M.D., M.S. (Ophthalmology). Philadelphia/London/Toronto: W. B. Saunders Company, 1973, 705 pp., $40.00

The interpretation and appropriate management of orbital tumors in children is an important segment of pediatric ophthalmology. Such lesions are usually first manifested by the onset of proptosis. It is important that the practicing pediatrician be aware of the potential significance of these signs. A review of Dr. Henderson's textbook would quickly familiarize the reader with the potential tumors that can occur within the orbit and put into appropriate perspective his own concern in regard to the occurrence of proptosis in children.