Proptosis in a previously well five-year-old boy

Abstract

Neuroblastoma metastasis. Ophthalmic findings may be the first presentation of a neuroblastoma which is the commonest paediatric tumour to metastasise to the orbit. The primary tumour is usually intraabdominal. Approximately 10–20% of children will have orbital metastases [1Ahmed S. Goel S. Khandwala M. et al.Neuroblastoma with orbital metastasis: ophthalmic presentation and role of the ophthalmologists.Eye. 2006; 20: 466-470Crossref PubMed Scopus (36) Google Scholar, 2Dubois S.G. Kalka Y. Lukens J.N. et al.Metastatic sites in stage IV and IVS neuroblastoma correlate with age, tumour biology, and survival.J Pediatr Hematol Oncol. 1999; 21: 181-189Crossref PubMed Scopus (308) Google Scholar] and up to 50% are bilateral [3Chung E.M. Murphey M.D. Specht C.S. et al.Pediatric orbit tumours and tumour like lesions: osseous lesions of the orbit.Radiographics. 2008; 28: 1193-1214Crossref PubMed Scopus (54) Google Scholar, 4Alfano J.E. Ophthalmological aspects of neuroblastomatosis: a study of 53 verified cases.Trans Am Acad Ophthalmol Otolaryngol. 1968; 72: 830-848PubMed Google Scholar, 5Altonbary Y. Mansour A.K. Sarhan M. et al.Proptosis is a pediatric dilemma.Ann Pediatr Child Health. 2015; 3: 1066Google Scholar]. Proptosis and ecchymosis (“raccoon eyes”) are the commonest ocular manifestations [[3]Chung E.M. Murphey M.D. Specht C.S. et al.Pediatric orbit tumours and tumour like lesions: osseous lesions of the orbit.Radiographics. 2008; 28: 1193-1214Crossref PubMed Scopus (54) Google Scholar]. The most common cause of proptosis in a child is orbital cellulitis [[5]Altonbary Y. Mansour A.K. Sarhan M. et al.Proptosis is a pediatric dilemma.Ann Pediatr Child Health. 2015; 3: 1066Google Scholar]. Histiocytosis, haemangioma or lymphangioma are further benign causes. Malignant aetiologies include rhabdomyosarcoma, Ewing’s sarcoma, lymphoma and metastases from a neuroblastoma, bone tumours or leukaemia [[5]Altonbary Y. Mansour A.K. Sarhan M. et al.Proptosis is a pediatric dilemma.Ann Pediatr Child Health. 2015; 3: 1066Google Scholar]. Typical CT or MRI features are an extra-conal soft tissue mass arising from the wall of the orbit [[8]Lonergan G.J. Schwab C.M. Suarex E.S. et al.Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: Radiologic-Pathologic Correlation.Radiographics. 2002; 22: 911-934Crossref PubMed Scopus (478) Google Scholar]. As in this patient, it can be locally aggressive extending into the infratemporal fossa structures or intra-cranially, with bony destruction of the roof and wall of the orbit and resultant bony spiculation [3Chung E.M. Murphey M.D. Specht C.S. et al.Pediatric orbit tumours and tumour like lesions: osseous lesions of the orbit.Radiographics. 2008; 28: 1193-1214Crossref PubMed Scopus (54) Google Scholar, 6Wells R.G. Sty J.R. Gonnering R.S. Imaging of the pediatric eye and orbit.Radiographics. 1989; 9: 1023-1044Crossref PubMed Scopus (14) Google Scholar, 7Lallemand D.P. Brasch R.C. Char D.H. et al.Orbital tumours in children: characterisation by computed tomography.Radiology. 1984; 151: 85-88Crossref PubMed Scopus (8) Google Scholar]. The mass may be unilateral or bilateral, unlike a rhabdomyosarcoma which is typically unilateral [[3]Chung E.M. Murphey M.D. Specht C.S. et al.Pediatric orbit tumours and tumour like lesions: osseous lesions of the orbit.Radiographics. 2008; 28: 1193-1214Crossref PubMed Scopus (54) Google Scholar]. Furthermore, rhabdomyosarcoma may involve anterior pre-septal orbital structures but a neuroblastoma will usually not [[3]Chung E.M. Murphey M.D. Specht C.S. et al.Pediatric orbit tumours and tumour like lesions: osseous lesions of the orbit.Radiographics. 2008; 28: 1193-1214Crossref PubMed Scopus (54) Google Scholar]. Neuroblastomas can cause dural and leptomeningeal metastases, with involvement of the skull causing diastasis of affected sutures [3Chung E.M. Murphey M.D. Specht C.S. et al.Pediatric orbit tumours and tumour like lesions: osseous lesions of the orbit.Radiographics. 2008; 28: 1193-1214Crossref PubMed Scopus (54) Google Scholar, 8Lonergan G.J. Schwab C.M. Suarex E.S. et al.Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: Radiologic-Pathologic Correlation.Radiographics. 2002; 22: 911-934Crossref PubMed Scopus (478) Google Scholar]. This child had clinical, radiological and pathological findings consistent with metastatic neuroblastoma and an MRI abdomen (Fig. 2) revealed a left adrenal primary site. Proptosis in a previously well five-year-old boyJournal of Clinical NeuroscienceVol. 34PreviewA five-year-old boy presented with a one-week history of left-sided proptosis and retro orbital pain, with no prior medical or surgical history. On examination, there was left-sided proptosis with no visual field defect or other neurological abnormality. Full blood count, electrolytes and inflammatory markers were unremarkable. Full-Text PDF