Orbital lesions in pediatrics.

Transcription

1 Orbital lesions in pediatrics. Poster No.: C-1912 Congress: ECR 2012 Type: Educational Exhibit Authors: J. H. Yoo, J. V. HUNTER ; Seoul/KR, HOUSTON, TX/US Keywords: Head and neck, CT, MR, Computer Applications-Detection, diagnosis, Pathology DOI: /ecr2012/C Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 36

2 Learning objectives To understand the wide variety of lesions that can affect the pediatric orbit. To recognize specific radiological appearances of pediatric orbital diseases according to their specific location. To review the differential points of the various pediatric orbital disease according to their pathologic origin and their location. Background The orbital lesions are not umcommon and are variable in disease spectrum. The purpose of this review is to illustrate the range of pathology in the orbit by reporting the spectrum of the disease diagnosed at a tertiary hospital during recent years. We will review and provide illustrative examples of both congenital and acquired lesions ; congenital anomaly such as optic nerve hypoplasia and aplasia, micropthalmia, coloboma, benign lesions such as dermoid, epidermoid, lipoma, meningioma, optic glioma, neurofibroma, Langerhans' cell histiocytosis, vascular lesion such as hemangioma, venolymphatic malformation, PHACE syndrome, choroid angioma in the Sturge-Weber syndrome, malignant tumor such as retinoblastoma, rhabdomyosarcoma, neuroblastoma, leukemia and lymphoma, orbital injury such as foreign body, blow-out trauma, eye globe rupture, infection and inflammation such as cellulitis, abscess, congenital infection such as toxoplasmosis, autoimmune disease such as Sjogren disease. Imaging findings OR Procedure details The characteristic patterns of imaging with CT and MRI will be displayed in a spectrum of lesions that can affect the pediatric orbit and will be discussed the differential points. 1. Orbital trauma is not uncommon in the pediatrics. - Foreign bodies such as pencil injury and BB gun injury (Fig. 1, 2), trauma with orbital blow-out trauma with or without EOM muscle herniation (fig. 3). - Orbital AVF, Eye globe laceration or rupture or orbital hemorrhage (Fig. 4-6). 2. Infection and inflammation of the orbit shows variable findings of diseases involving focal or diffuse cellulitis with/without destroy orbital wall, with/without inflammatory Page 2 of 36

3 phlegmon or abscess, disease from the sinusitis or extending to sinus or intracranium (Fig.7,8,9 ). - Inflammatory pseudotumor (Fig.10) and optic neuritis (Fig.11), thyroid ophthalmopathy (Fig. 12, 13), Immune related fungal infection, and congenital infection such as toxoplasmosis (Fig. 14), and autoimmune disease such as Sjogren disease also can be seen. 3. Benign and malignant orbital tumor : - Many benign masses such as dermoid and epidermoid in the typical region (FIG. 15), lipoma, meningioma (Fig. 16), optic glioma (Fig. 17), neurofibroma, Langerhans' cell histiocytosis (Fig. 18). - And vascular origin mass such as hemangioma (Fig ), venolymphatic malformation (Fig. 21), vascular mass related syndrome such as PHACE syndrome and choroid angioma in the Sturge-Weber syndrome (Fig. 22). - Frequent pediatric malignant orbital tumors are retinoblastoma(fig ), rhabdomyosarcoma (Fig.26), neuroblastoma (Fig. 27). - Hematologic orbital malignancies are also seen in leukemia, chroloma in AML, and orbital lymphoma (Fig ), 4. Congenital anomaly are bilateral small globes in LOWE (oculocerebrorenal syndrome), optic hypoplasia in Chiary II malformation, hemifacial microsomia, micropthalmia with absent optic nerve. Others includes drusen eye, and coloboma. Images for this section: Page 3 of 36

4 Fig. 1: Pencil penetrating injury Page 4 of 36

5 Fig. 2: Penetrating injury by BB gun with radiopaque foreign body. Page 5 of 36

6 Fig. 3: Orbital blow out fracture with orbital fat heriation. Page 6 of 36

7 Fig. 4: Globe rupture Page 7 of 36

8 Fig. 5: Choroidal hemorrhage Page 8 of 36

9 Fig. 6 Page 9 of 36

10 Fig. 7: Orbital and periorbital cellulitis. Page 10 of 36

11 Fig. 8: Subperiosteal abscess along the beneath superior orbital wall Page 11 of 36

12 Fig. 9: Dacryocystitis. Page 12 of 36

13 Fig. 10: Inflammatory pseudotumor. For the DDx. pseudotumor vs. thyroid eye disease, pseudotumor not only the muscles, but also the tendons are involved. Page 13 of 36

14 Fig. 11: Optic neuritis. Optic nerve swellng and enhancement in acute stage, High signal intensity on T2 image suggestive demyelination in chronic stage. Page 14 of 36

15 Fig. 12: Thyroid ophthalmopathy. Autoimmune ds % pt. with hyperthyroidism, specific EOM enlargement(i>m>s>l>obl; I'm slow!) low density within m belly (belly involve with spindle shape) orbital fat increase, venous engorgement, Optic n stretching and compression. Page 15 of 36

16 Fig. 13: Progression of thyroid ophthalmopathy with optic nerve compression Page 16 of 36

17 Fig. 14: Congenital infection Page 17 of 36

18 Fig. 15: Variable sites of orbital dermoids. Well-defined cystic mass as an anterior extraconal mass. Superolateral and anterior frontozygomatic suture are most common sites (65-75%). fatty content containing in 40-50%. Osseous remodaling 85%, fluid or mixed contents. Page 18 of 36

19 Fig. 16: Optic glioma. 3 typesoptic nerve glioma - associated with NF1 or not associated with NF1, and typical adult form with poor Px. Page 19 of 36

20 Fig. 17: Optic nerve sheath meningioma. typical tram-track sign of optic nerve sheath meningioma due to tubular thickening of sheath. Page 20 of 36

21 Fig. 18: Langerhans cell histiocytosis. Well-defined bone destruction without periosteal reaction amd superolateral aspect of orbit are typical finding. Page 21 of 36

22 Fig. 19: Hemangioma. Page 22 of 36

23 Fig. 20: 'Alarming Hemangioma.The term 'alarming hemangiomas' was coined by Enjolras and coworkers to outline threatening lesions, e. g. those that compromise the airway, impair vision, cause heart failure, or induce a thrombocytopenic coagulopathy. A 1-month-old girl with left orbital hemangioma. a. Pre-Tx CT scan. b. Eight-month postcontinuous interferon Tx. Marked decrease of the lesion is obvious. Page 23 of 36

24 Fig. 21: Orbital lymphatic malformation Page 24 of 36

25 Fig. 22: 12 yr old male. Reinal hemangioblastoma in von-hippel Lindau syndrome. Page 25 of 36

26 Fig. 23: Retinoblastoma. One of most common 1st year tumor, 10-30% familial, 90-95% having alcification along the posterio aspect of orbit. Page 26 of 36

27 Fig. 24: Bilateral retinoblastoma. 2-35% pt. of retinoblastoma, all are familial hereditary. Page 27 of 36

28 Fig. 25: Bilateral retinoblastoma Page 28 of 36

29 Fig. 26: Rhabdomyosarcoma. large extraconal soft tissue mass, dysplacing structures, not frequent bone destruction Page 29 of 36

30 Fig. 27: Neuroblastoma. Differential point from the LCH is bulky mass and sunburst periosteal reaction on the bone-setting image. Page 30 of 36

31 Fig. 28: Leukemic infiltration of orbit Page 31 of 36

32 Fig. 29: Lymphoproliferative disorder Page 32 of 36

33 Fig. 30: Orbital lymphoma. Homogeneous solid mass with destruction of orbital wall. Page 33 of 36

34 Fig. 31: Recurrent leukemia. Extraconal well defined mass with well enhancement. Page 34 of 36

35 Fig. 32: Chroloma AML. Page 35 of 36

36 Conclusion The orbiital lesions are not uncommon and the disease spectrums are variable in pediatrics. This is a didactive presentation with CT and MR imaging of orbital lesions in children with illustrative examples of the range of the pathologies seen in pediatric age group. Personal Information J.H.Yoo. Radioogy department, Ewha Womans University School of Medicine, Seoul, Korea J.V.Hunter. diagnostic imaging, Texas Childrens Hospital, Houston, Tx. USA. References 1. Lope LA, Hutcheson KA, Khademian ZP. Magnetic resonance imagin in the analysis of pediatric orbital tumors : utility of diffusion-weighted imaging. J AAPOS 2010 Jun 143); Won Sang Jung, MD1, Kook Jin Ahn, MD1, Mi Ra Park, MD2, Ji Young Kim, MD1, Jae Jeong Choi, MD1, Bum Soo Kim, MD1, Seong Tai Hahn, MD1, The Radiological Spectrum of Orbital Pathologies that Involve the Lacrimal Gland and the Lacrimal Fossa, Korean J Radiol 2007;8: Harsh Kandpal, sushma Vashisht, Raju Sharma, Ashu Seith, Imaging spectrum of pediatric orbital pathology: a pictorial review. Indian J Ophthalmol 2006;54: Page 36 of 36