Childhood Proptosis Research Articles

Acute unilateral childhood proptosis as a presenting symptom of sickle cell disease

Although rare, orbital bone marrow infarction is a serious complication in patients with sickle cell disease (SCD). Ophthalmologists should have a high index of suspicion for this disease in cases of acute childhood proptosis. Appropriate early management will prevent optic nerve dysfunction and permanent vision loss. Here, we present a case where the patient presented to the ophthalmologist with complaints of pain and proptosis, which led to the diagnosis of SCD.

Epidemiological and Diagnostic Aspects of Childhood Proptosis in Lome (Togo) from 2010 to 2020

Epidemiological and Diagnostic Aspects of Childhood Proptosis in Lome (Togo) from 2010 to 2020

SMALL ROUND CELL TUMORS PRESENTING AS ORBITAL PROPTOSIS IN CHILDREN WITH BONE MARROW INVOLVEMENT: A STUDY OF HAEMATO- MORPHOLOGICAL FEATURE.

Introduction: Childhood proptosis is an interesting clinical presentation of underlying hematological and non-hematological disorders. Malignant proptosis is most commonly caused by retinoblastoma, rhabdomyosarcoma, leukemia/lymphomas and other metastatic tumors such as neuroblastoma. Bone marrow examination is a commonly used diagnostic and staging modality in the evaluation of hematological and non- hematological disorders in pediatric population. Patients with proptosis present with subtle changes in hemogram which point towards metastatic or hematologic etiology and hence a detailed hematologic work-up is mandatory. A descriptive cross-sectional study wasAim/objective: conducted to study the clinical, hematological and pathological data of interesting pediatric malignancies presenting as orbital proptosis with or without bone marrow involvement at the time of diagnosis from July 2020 to June 2021. All cases of orbital proptosis presenting withMethods: cytopenias referred for bone marrow examination were included in the study. Primary diagnosis of was made on histopathology/ne needle aspiration cytology combined with clinico-radiological features. Bone marrow procedure was done from posterior superior iliac spine. The informed consents were taken from the patients in the vernacular language after explaining the procedures. All procedures performed in the current study were approved by Institutional ethical committee. Aspirate smears and biopsy sections were stained by May-Grunwald-Giemsa and Hematoxylin and eosin respectively as per standard protocols. Special stains Myeloperoxidase (MPO), Alcin blue, PAS (perioidic acid and Schiff) and reticulin were performed when required. Samples were also processed for molecular genetics studies in acute leukaemias. The descriptive analysis of the data was done, and the results were presented as mean, frequencies, and percentages. Results: Of total 20 cases presenting as orbital-proptosis, only 11 cases showed bone marrow involvement as acute myeloid leukemia (45%) and as a metastatic site (55%). The most common cause of bilateral orbital proptosis was metastatic malignancies followed by acute myeloid leukaemias. Anaemia and thrombocytopenia were signicantly (p<0.05) associated with marrow inltration. The present analysis showedConclusion: different types of pattern bone marrow inltration had prognostic implications. Pancytopenias/bicytopeinias were most commonly seen in association with solid malignancies. Acute leukaemias presented with leukocytosis along with reduction in red cells and megakaryocytic lineages. Some cases primary diagnosis was initially made from bone marrow examination only. The present study highlights the signicance of bone marrow examination in patients with proptosis wherein subtle changes in hemogram point toward metastatic or hematologic etiology.

Retrospective analysis of clinical profile of pediatric proptosis in a tertiary care hospital of Eastern India

Background: Pediatric proptosis can often present with puzzling clinical features and needs a systematic approach to avoid diagnostic dilemma. Aims and Objectives: To study the clinical and etiological profile, management, and outcome of pediatric proptosis. Materials and Methods: This retrospective noncomparative study involved the analysis of record files of 41 cases of childhood proptosis, who presented in the eye outpatient department and oculoplastic clinic between January 2013 and December 2016. Results: There were a total of 41 children, age varied from 6 months to 15 years. The maximum number of cases 25 (61%) were in the age group of 0–5 years. Male:female ratio was 1.77:1. Unilateral cases were found to be 31 (76%) and bilateral cases be 10 (24%). Neoplastic lesions constituted 22 (54%) of which benign lesions were 3 (14%) and malignant were 19 (86%). Among malignant, primary were 11 (58%) and secondary were 8 (42%). Inflammatory/infective lesions were 8 (20%). Conservative/medical management was given to 11 cases (27%). Surgery with pre- or post-operative chemo- or radio-therapy was the treatment of choice. Conclusion: Neoplastic lesions were found to the most common cause of proptosis in this study. Apart from detail ocular and systemic examination, final etiological diagnosis is possible in certain cases only after investigations such as peripheral smear, bone marrow examination, histopathological examinations, ultrasonography B-scan, computed tomography scan, and magnetic resonance imaging. Timely diagnosis and management can save the vision and many a times lives.

Etiopathogenesis of childhood proptosis

Aim: To study the etiology, presentation and management of proptosis in children less than 14 years of age. Materials and Methods: This is prospective interventional study conducted at the department of Oculoplasty and Orbital diseases of Sarojini Devi eye hospital, Hyderabad. Period of study was from August 2012 to July 2014. 120 eyes of 111 children presenting as proptosis to the department were included in the study. Adult cases of Proptosis were excluded from the study. All cases underwent a detailed proptosis evaluation by an experienced oculoplasty surgeon. CT scan of orbit, axial and coronal view, 2mm cut without contrast was modality of imaging in all cases. CT scan with contrast was done in selected cases. Complete blood picture and surgical profile investigations were done. Cases were posted for excision or incision biopsy under general anaesthesia. Histopathological diagnosis was obtained in required cases. Culture and sensitivity of pus was done in infective cases. Results: 111(14.83%) cases of childhood proptosis were there out of 748 cases of proptosis presented during the study period to the department. The Male to Female ratio was 55:46. 102 cases were of unilateral proptosis and 9 were bilateral. Common etiology for childhood proptosis was 28 congenital, 18 infective, 20 parasitic, 36 neoplasm, 6 secondaries and 3 trauma cases. Of 111 cases 28 had acute proptosis, 25 had Sub acute presentation and 58 cases had chronic presentation. Management of 111 cases was done as follows: 48 cases underwent surgery, 41 were managed medically, 10 cases were referred to Medical Oncologist and 12 cases of congenital crouzons disease and microphthalmos were not intervened. Conclusion: Childhood proptosis forms 14.83% of total proptosis cases presenting at department of Oculoplasty and Orbital diseases. Etiological spectrum is different when compared to adult Proptosis.(1) Keywords: Congenital, Infective, Neoplasm, Parasitic, Proptosis

A Rare Cause of Proptosis in Childhood: Langerhans Cell Histiocytosis.

A three-year-old male patient was admitted to the clinic with proptosis in his right eye. He had a history of fever with an unknown etiology. In examination, right proptosis was observed and an immobile mass was palpated at the lateral wall of the right orbita. Eye movement was unrestricted in all directions and anterior and posterior segment examination was normal in both eyes. On computed tomography, diffuse bone destruction and expansion was observed in the right orbital lateral wall and other cranial bones. Langerhans cell histiocytosis was diagnosed by bone biopsy. Malignancy is an important cause of proptosis in childhood. Pediatric patients who are admitted to clinic with proptosis should be carefully examined and Langerhans cell histiocytosis should also be considered as an etiology.

Maxillo-orbital granulocytic sarcoma in acute myeloid leukemia

Granulocytic sarcoma or chloroma, a manifestation of acute myeloid leukemia (AML) is a rare cause of childhood proptosis. A 14-year-old boy presented with progressively increasing unilateral proptosis and swelling of lower eyelid and face on the right side. Contrast enhanced computed tomographic images revealed enhancing infiltrates occupying the right orbit, maxillary antrum, and infratemporal fossa. Incisional biopsy from the orbital swelling and the bone marrow aspirate showing leukemic blast cells confirmed the diagnosis of AML. The peripheral smear was normal initially, but high total leukocytic count with immature blast cells was evident after 1-month of presentation. Chemotherapy brought about the remission of the disease. However, the delay in diagnosis because of negative peripheral blood smear examination and inconclusive fine-needle aspiration biopsy led to the loss of vision in right eye. Diagnosis of such case can be made by a combination of good clinical examination and relevant investigations. This case of maxillo-orbital granulocytic sarcoma is reported because of its rarity and to emphasize the clinical and cyto-histological features and problems concerning differential diagnosis.

Childhood proptosis: A case of missed diagnosis

Proptosis in childhood can be a diagnostic dilemma. We present a child who came with subacute onset, bilateral and asymmetric proptosis. He was diagnosed to have pseudotumor on the basis of imaging studies and started on systemic steroids. He responded partially, only to have a recurrence few weeks later. Baseline blood investigations showed a substantial drop in hemoglobin. Peripheral blood smear (PBS) showed atypical cells suggestive of leukemia. A bone marrow biopsy confirmed acute myeloid leukemia. The case illustrates an extra-ocular, systemic cause of proptosis, which was missed on initial presentation. The clue to the diagnosis was clinched on simple baseline tests such as hemoglobin and PBS. An extra-ocular cause, though uncommon, should always be considered in evaluation for every childhood proptosis to prevent delayed diagnosis.

Zweijähriger Patient mit akutem Exophthalmus

Hemorrhage into an orbital lymphangioma can cause acute proptosis. A lymphangioma must be distinguished from acute threatening causes of rapidly developing proptosis in childhood, such as orbital cellulitis or malignant tumors (rhabdomyosarcoma). Imaging (magnetic resonance imaging, ultrasound, computed tomography) and histopathological evaluation are required for the final diagnosis. Immunohistochemical staining of lymphatic vessels with podoplanin can be useful for confirmation of the diagnosis.

Childhood proptosis: Relevance of aspiration cytology in the era of newer molecular diagnostic and imaging techniques

Childhood proptosis: Relevance of aspiration cytology in the era of newer molecular diagnostic and imaging techniques

Imaging of childhood proptosis

Imaging of childhood proptosis

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.

Esthesioneuroblastoma presenting as an orbital mass in a young child

A 3-year-old boy presented with uniocular proptosis and ophthalmoplegia. Investigation revealed a mass involving the right orbit with extension into the left orbit and paranasal sinuses, and intracranial extension involving both frontal lobes. Biopsy of an enlarged cervical node and the intranasal mass revealed esthesioneuroblastoma. This tumor has been reported rarely in a child this age, and only 12 case reports document patients under 10 years of age. The presentation as an orbital mass is previously unreported and must now be considered in the differential diagnosis of proptosis in childhood. The usual clinical, radiological, and pathological features of olfactory esthesioneuroblastoma are reviewed.

Orbital tumours in African children.

The differential diagnosis of proptosis in African children poses a bewildering number of possibilities (Davies, I968). Many of these cases prove to be due to inflammation of neighbouring sinuses or to invasion of the orbit by parasites or fungi. Orbital tumours causing proptosis occur frequently, and a series of sixty cases of proptosis in childhood caused by neoplasms in the orbit is reviewed in this paper.