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Vol 13 ppt

  1. 1. Volume 13Adamantinoma----------------Case 214-218 & 1094-1098Chordoma----------------------Case 219-224 & 1099-1106Histiocytoses Eosinophillic granuloma--Case 417-444 Hand-Schiller-Christian --Case 445-446 Letter Siwe disease--------Case 447-450 Sinus histiocytosis---------Case 450.1 Rosai-Dorfman’s disease
  2. 2. Adamantinoma
  3. 3. Adamantinoma The adamantinoma of bone is an extremely rare primary sarcomaof bone, accounting for about .3% of all malignant tumors of bone,and in 90% of the cases it will be seen in the diaphyseal portion ofthe tibia, especially in the anterior cortex. It occurs equally in malesand females, typically in the second and third decade of life. Thetissue of origin still remains a mystery but immunohistochemicalstains suggest an epithelial origin that might account for why theyare more common just beneath the skin in the anterior cortex of thetibia. Radiographically, the adamantinoma takes on the appearanceof a fibrous dysplastic lesion or perhaps osteofibrous dysplasia ofthe tibia. The lesion appears benign with a lytic core surrounded bydense, reactive fibro-osseous bone that frequently dilates the anteriorcortex and may be multiloculated in appearance. The tumor is quiteslow growing and is usually painful, whereas in fibrous dysplasiaand osteofibrous dysplasia, the patients remain asymptomatic. If a
  4. 4. so-called fibrous dysplastic lesion continues to grow past maturity,a physician should suspect adamantinoma, especially if the lesion ispainful, and look for the characteristic nests and cords of epithelial-looking cells surrounded by fairly benign-appearing fibro-osseoustissue on histological specimens. It is very rare for this tumor tometastasize to different sites but occasionally it will metastasize toregional lymph nodes and the lung. Treatment for this low grade lesion is purely surgical, consistingof a wide local resection, frequently a segmental resection of themid portion of the tibia, and reconstruction with a large boneallograft over an intramedullary nail. There have been a few rarecases in the literature where adamantinoma has arisen out of a pre-existing osteofibrous dysplastic lesion.
  5. 5. CLASSICCase #21425 year male withadamantinoma tibia
  6. 6. Lateral view
  7. 7. Wide resection specimenopened on back table
  8. 8. Photomic
  9. 9. Surgical specimen after3 mins in autoclave
  10. 10. Autoclaved tibiareplaced over IMnail and rotated 180degrees
  11. 11. Cancellous autogenousiliac chips placedbetween tibia androughed up fibular
  12. 12. 1 year later showingunion of prox tib-fibstep ladder synostosis
  13. 13. 3 years post op with solidunion to autoclaved bone
  14. 14. 7 years post op withsymptoms of ankle painTurns HIV positive
  15. 15. One year later withosteopenia aboutpainful and swollenankle
  16. 16. Sagittal T-1 MRIshows recurrent tumorabove ankle
  17. 17. Cut amputation specimenshowing excellentosseointegration betweenfibula & autoclaved tibia tumor
  18. 18. autoclaved tibiareactive live bone Photomic at tibia-fibula interphase
  19. 19. Case #21522 year femaleadamatinomadistal tibia
  20. 20. Photomic
  21. 21. Another photomic
  22. 22. Wide resectiondistal tibia biopsy site
  23. 23. Autoclaved specimencemented over IM nailincluding total ankle
  24. 24. Composite reconstructioncompleted includingtotal ankle
  25. 25. Post op lateral x-ray
  26. 26. AP x-ray showingfibular recurrenceresulting in BK amp2 years later
  27. 27. Case #21611 year female withadamantinoma arisingfrom osteofibrous dysplasiatibia
  28. 28. AP view
  29. 29. Bone scan
  30. 30. Low power photomic with osteofibrous dysplasia to right and adamantinoma to left
  31. 31. Photomic with cords and nests of epithelioid cells
  32. 32. Higher power of cords and nests of epithelioid cells
  33. 33. 4 years followingsegmental resectionand allograft reconover IM nail
  34. 34. Case #21725 year femaleadamantinoma tibia
  35. 35. Close up AP view
  36. 36. Low power photomic
  37. 37. Case #21858 year femaleadamantinomaproximal tibia
  38. 38. Lateral view
  39. 39. Bone scan
  40. 40. Coronal GadContrast MRI
  41. 41. Sagittal STIR MRI
  42. 42. Axial T-2 MRI
  43. 43. Another axial T-2 MRI showing cystic fluid-fluid levels
  44. 44. Photomic
  45. 45. Another photomic
  46. 46. Case #109415 year female withadamantinoma arisingfrom osteofibrous dysplasia
  47. 47. AP view several years latershowing slow progression andfibular involvement as well
  48. 48. Lateral view showingpath fracture
  49. 49. Photomic showing combined fibro-osseous and adamantinoma histology
  50. 50. Photomic showing mostly adamantinoma histology
  51. 51. Another photomic
  52. 52. 6 months followingcurettement and cancellousallograft placement
  53. 53. 9 months later
  54. 54. AP view same time
  55. 55. 3.5 years later showinggood healing and noprogression of disease
  56. 56. Lateral view at same time
  57. 57. Case #109558 year female withadamantinoma tibia
  58. 58. Sagittal T-1 MRI
  59. 59. Sagittal T-2 MRI
  60. 60. Case #109618 year male withadamantinoma tibia
  61. 61. Bone scan
  62. 62. Sagittal T-2 MRI
  63. 63. Axial T-2 MRI
  64. 64. Case #1096.1 Pseudo adamantinoma 78 year male with chronic osteomyelitis tibia since age 2 years with recent fungating lesion anterior tibia
  65. 65. Case #109714 year male withadamantinoma distaltibia and fibula
  66. 66. Coronal T-1 MRI
  67. 67. Photomic
  68. 68. Case #109816 year male withadamantinoma mandible
  69. 69. AP x-ray
  70. 70. Oblique view
  71. 71. Surgical exposure
  72. 72. Resected specimen
  73. 73. Photomic
  74. 74. Another photomic
  75. 75. Chordoma
  76. 76. Chordoma The chordoma is a very rare malignant tumor of bone thataccounts for only 4% of all malignant bone tumors. It arises fromthe primitive notochord of the axial skeleton and is most commonlyseen in the lower portion of the sacrum, accounting for 50% of thechordomas. 37% arise in the spheno-occipital area and a smallnumber occur in the cervical and lumbar spine. The more commonsacral lesions are seen in an older age group between the ages of40 and 80 years, compared to the spheno-occipital chordomas thatoccur in a younger adult age group. The later carry a much worseprognosis because of the location at the base of the skull. Thechordoma is clinically similar to a mucinous type of chondrosarcoma. In the sacral area, chordomas are usually attached to the anteriorportion of the distal three segments of the sacrum and grow in theretroperitoneal space, pressing up against the rectum whereeventually they will present with clinical symptoms related toconstipation and can be picked up on a rectal examination. Because
  77. 77. the tumor is very slow growing, it rarely causes significant painsymptoms. The radiographic findings are frequently not very obvious,even with large tumors that are better evaluated by soft tissuetechnique, such as CT scan or, better yet, MRI. Microscopically, thechordoma has a mucinous appearance similar to a low grade myxoidchondrosarcoma, but the diagnostic feature is the “signet ring”appearance of the physaliferous cells that have a peripheral nucleus,a large cytoplasmic inclusion of physaliferous mucinous material thatcan look a bit like a liposarcoma. Treatment for the chordoma consists primarily of a wide surgicalresection, which sometimes is very difficult, especially with lesionsextending into the upper sacral segments where the nerve rootsbecome a problem and may result in significant neurogenic boweland bladder complications. Even with surgical treatment, the localrecurrence rate is very high so that post op radiation therapy isrecommended. It has cut the local recurrence rate to about 30%compared to 65% without RT. Recurrences can occur locally up to
  78. 78. fifteen years after the original surgery. Pulmonary metastases areextremely rare and systemic chemotherapy is not indicated for thistumor.
  79. 79. CLASSIC Case #219 45 year male with chordoma sacrum
  80. 80. Lateral view
  81. 81. Sagittal T-1 MRI
  82. 82. Coronal T-1 MRI
  83. 83. Axial T-1 MRI
  84. 84. Axial T-2 MRI
  85. 85. Resected specimen cut in path lab
  86. 86. Photomic showing physaliferous cells
  87. 87. Case #220 Sagittal T-1 MRI 74 year male with chordoma sacrum
  88. 88. Sagittal T-2 MRI
  89. 89. Case #220.1 CT scan Sacral chordoma 48 year male with 4 mo history of sacral pain
  90. 90. Bone scan
  91. 91. Axial MRI T-1 T-2
  92. 92. Sag T-2
  93. 93. Cor T-2 Gad
  94. 94. Post op x-ray
  95. 95. Case #221 82 year male with chordoma lower sacrum
  96. 96. Lateral view
  97. 97. Sagittal T-1 MRI
  98. 98. Sagittal T-2 MRI tumor
  99. 99. tumorAxial T-1 MRI
  100. 100. Case #222 CT scan 65 year female with chordoma sacrum and buttock
  101. 101. tumorAxial T-2 MRI
  102. 102. Axial T-2 MRI at higher level
  103. 103. femur tumorSagittal T-2 MRI
  104. 104. Photomic
  105. 105. Case #222.1 T-2 MRI Sacral chordoma 65 yr male
  106. 106. Rebar and cement reconstruction
  107. 107. Case #223 49 year male with recurrent chordoma sacrum
  108. 108. tumorCT scan
  109. 109. Sagittal T-2 MRI tumor
  110. 110. tumorAxial T-2 MRI
  111. 111. Wide resection entire CD rodsacrum and recon with L-5CD rods and Steinmanpins prior to cementation Steinman pins
  112. 112. CD rodsAfter cementation cement sacrum
  113. 113. Post op x-ray cement
  114. 114. L-4 L-5Lateral view cement cement coccyx
  115. 115. Case #224 Sagittal T-1 MRI L-3 S-1 55 year male with chordoma lumbar spine
  116. 116. Resection L-4, L-5 and part of S-1
  117. 117. Resected vertebrae
  118. 118. Photomic
  119. 119. sacrumAnterior recon with large bone allograft and plate
  120. 120. allograft CD rodsPost op lateral x-ray
  121. 121. Case #1099 53 year female with chordoma sacrum
  122. 122. Bone scan
  123. 123. Oblique bone scan
  124. 124. tumorCT scan one year later
  125. 125. Sagittal T-2 MRI
  126. 126. Sagittal T-2 MRI tumor
  127. 127. tumorAxial T-2 MRI
  128. 128. Photomic
  129. 129. Case #1100 CT scan 61 year male with chordoma distal sacrum
  130. 130. Bone scan bladder
  131. 131. Sagittal T-1 MRI tumor
  132. 132. Sagittal T-2 MRI tumor
  133. 133. Axial T-1 MRI
  134. 134. Photomic
  135. 135. Case #1101 Axial T-1 MRI 60 year female with chordoma sacrum
  136. 136. Axial T-2 MRI
  137. 137. Sagittal T-2 MRI
  138. 138. Case #1102 Axial and coronal CT scan 37 year male with chordoma in body of L-4
  139. 139. CT scan myelogram with block at L-4
  140. 140. Myelogram showing blockat the L-4 level
  141. 141. Oblique myelogram views
  142. 142. Photomic
  143. 143. Post op x-ray followingremoval of L-4 bodyand recon with fibularstrut from L-3 to L-5
  144. 144. Lateral view of fibularstrut reconstruction
  145. 145. 8 mos later withcollapse of L-3 on L-5
  146. 146. 10 more monthsand further collapseof L-3 on L-5
  147. 147. Sagittal T-1 MRI showing collapse and kinked dural sac
  148. 148. X-ray following restorationof collapsed vertebral spacewith iliac strut graft infront and CD rods behind
  149. 149. AP view
  150. 150. Case #110334 year male withchordoma L-5
  151. 151. tumorCT scan at L-5 shows chondroid like tumor arising from the postero-lateral elements of L-5
  152. 152. tumorAnother CT cut
  153. 153. Another
  154. 154. tumorAxial T-1 MRI
  155. 155. Axial T-2 MRI
  156. 156. Another axial T-2 MRI
  157. 157. Sagittal T-2 MRI
  158. 158. Another sagittal T-2 cut
  159. 159. Photomic
  160. 160. Case #110465 year male with achordoma of L-2Myelographic studyshowing completeblock at L-2 level
  161. 161. Surgical exposure of paraspinous mass at L-2 level
  162. 162. Post op x-ray showingrecon with cementand pins in tumor defect
  163. 163. Lateral view
  164. 164. Case #1104.1 Chordoma L-149 year male with backpain for 6 mos and recentparaparesis
  165. 165. Axial CT L-1 level
  166. 166. Axial T-1 T-2 Gad
  167. 167. Sag T-1 T-2
  168. 168. PO x-ray
  169. 169. PO Cor CT Sag
  170. 170. Case #110554 year male withchordoma C-spine
  171. 171. Case #1106 42 year female with chordoma base of skull
  172. 172. Autopsy specimenshowing lobulatedchondroid lookingmass in post fossa
  173. 173. Macro section
  174. 174. Photomic
  175. 175. Photomic
  176. 176. Histiocytoses Of Bone
  177. 177. EosinophilicGranuloma
  178. 178. Eosinophilic Granuloma (Langerhans Histiocytosis) The so-called benign histiocytoses, sometimes referred to as histiocytosis X, include various disease conditions such as eosino- phillic granuloma, Hand-Schuller-Christian disease and Letterer- Siwe disease. Eosinophillic granuloma is the most benign of the histiocytic disorders, followed next by Hand-Schuller-Christian disease that presents with an intermediate diffuse process of both bone and soft tissue that can be fatal. Letterer-Siwe disease is the most aggressive and fatal form of the histiocytoses, presenting like leukemia with a very poor prognosis for survival. Eosinophillic granuloma, now referred to as Langerhans histio- cytosis, is a benign histiocytic disorder that frequently presents in children between the ages of 5 and 15 years with a clinical picture that can masquerade as a malignant neoplasm such as Ewing’s sarcoma. It occurs twice as often in males than females. It is usually a monostotic disorder of the skeletal system, however, in
  179. 179. 10% of cases it will be seen in two or three separate sites. Theetiology of this histiocytic process is still unknown but some havepostulated a viral etiology. Patients present with inflammatory pain,more severe at night, that may be associated with a low grade feveror elevated sed rate. The most common location is in the skull,followed next by the ribs, pelvis, maxilla, vertebral body, clavicleand scapula, in that order. Besides flat bone involvement, it iscommonly seen in the diaphyses of long bones where it canmasquerade as Ewing’s sarcoma, but can also occur in metaphysealbone and is found least commonly in epiphyseal bone. In youngchildren, the condition can be extremely permeative and destructivein nature, taking on the appearance of Ewing’s sarcoma, metastaticneuroblastoma, or acute osteomyelitis. On x-ray, eosinophillic granuloma has an onion-skin appearancesimilar to a Ewing’s sarcoma. In an older age group, the conditiontends to be more focal and more granulomatous in appearance withless permeative change. Microscopically, there are large, pale-
  180. 180. staining histiocytes speckled with small, bright-staining eosinophilsand an occasional giant cell. Eosinophillic granuloma tends toinvolute spontaneously without treatment and therefore symptomatictreatment should be conservative--simple curettement for diagnosticpurposes and perhaps cortical steroid injections can be beneficial toinhibit the inflammatory process. In more difficult parts of the body,such as the spine or pelvis, very low grade radiation therapy can beconsidered, realizing that this could convert the process to a malignantsarcoma at a later date. In more aggressive forms with multi-focalinvolvement, especially if there is soft tissue involvement of the skin,lymph nodes or lung, a low dosage chemotherapy program can beconsidered. Sometimes the low grade eosinophillic granulomahistiocytosis can upgrade to a more aggressive and dangerous formsuch as Hand-Schiller-Christian disease or even Letterer-Siwe disease.With spinal lesions, spinal cord compression can result in paraparesisrequiring laminectomy decompression. However, kyphotic deform-ities in younger patients tend to correct spontaneously without surgery.
  181. 181. CLASSICCase #41717 year maleEG distal femur
  182. 182. Lateral view
  183. 183. Coronal T-1 MRI
  184. 184. Sagittal T-2 MRI
  185. 185. Coronal STIR MRI
  186. 186. eosinophil histiocyte Biopsy photomic
  187. 187. Case #417.1 6 yr male with pain in left thigh for 6 months
  188. 188. CT scan
  189. 189. Axial T-1 T-2 Gad
  190. 190. Cor Gad Sag Gad
  191. 191. Case #417.2 10/08 11/08 12/08 6 year male with pain in thigh for 1 mo.
  192. 192. 11/08 1/09Cor T-1 T-2 T-1 T-2
  193. 193. Case #4182 year maleEG mid femur
  194. 194. Case #4198 year maleEG mid femur
  195. 195. Case #420 3 year female path fracture EG femur
  196. 196. Lateral view
  197. 197. Case #4213 year maleEG mid femur
  198. 198. Lateral view
  199. 199. Case #4227 year female withEG femur
  200. 200. Case # 423 14 year male with EG clavicle
  201. 201. Case #424 16 year male with EG mid clavicle
  202. 202. Macro section of resection specimen
  203. 203. Photomic
  204. 204. Case #425 6 year male with EG proximal humerus
  205. 205. Case # 42612 year male withEG mid humerus
  206. 206. Arteriogram
  207. 207. Bone Scan
  208. 208. Case #4272.5 year maleEG distal humerus
  209. 209. Lateral view
  210. 210. Case #428 2.5 female with EG proximal ulna
  211. 211. AP view
  212. 212. CT scan showing reactive involucrum formation
  213. 213. More proximal CT cut
  214. 214. edemaAxial T-2 MRI
  215. 215. Case #429 23 year female with EG right ilium
  216. 216. Bone scan
  217. 217. CT scan
  218. 218. Axial STIR MRI
  219. 219. Case #429.1 51 year female with 6 mos pelvic pain second to EG
  220. 220. Bone scan
  221. 221. Axial T-2 MRI
  222. 222. Case #429.2 EG pelvis 39 year male with dull aching pain left hip for 9 mos.
  223. 223. Axial CT scan
  224. 224. Cor and Sag CT
  225. 225. Axial T-1 T-2T-1 T-2
  226. 226. Cor T-1 Sag T-2
  227. 227. Case #43012 year maleEG ilium
  228. 228. Case #431 20 year male with EG scapula
  229. 229. CT scan
  230. 230. Coronal T-2 MRI
  231. 231. Axial T-2 MRI
  232. 232. Case #4324 year maleEG mid fibula
  233. 233. Case #433 12 year male with EG posterior rib
  234. 234. Case #434 x-ray resection specimen 24 year male with EG skull
  235. 235. CT scan of EG skull
  236. 236. Photomic
  237. 237. Case #4354 year femaleEG right mandible
  238. 238. Oral photo of submucosal mass
  239. 239. Mandibular view
  240. 240. Full mouth dental x-ray view
  241. 241. Axial CT scan
  242. 242. Photomic
  243. 243. Case #4363 year malevertebra plana fromEG thoracic vertebra
  244. 244. Sagittal CT scan
  245. 245. Sagittal T-1 MRI
  246. 246. Sagittal T-2 MRI
  247. 247. Sagittal gad contrast MRI
  248. 248. Case #438 7 year male with EG T-11
  249. 249. Sagittal gad contrast MRI
  250. 250. Sagittal T-2 MRI showing protrusion into vertebral canal
  251. 251. Case #439 new collapse19 year male with EG collapse new collapseat 3 different levels but at olddifferent times oldThe lower levels show heightrestoration as a spontaneoushealing process seen in children old old
  252. 252. Case # 4407 year female withhealing collapsedlumbar vertebra
  253. 253. Case # 44115 year male withEG lumbar vertebra
  254. 254. Case #4425 year femaleEG C-3
  255. 255. Case #44310 year femaleEG C-7
  256. 256. Case #444 skull defect8 year maleEG skull withEG dermatitis
  257. 257. Severe EG dermatitis back
  258. 258. Case #444.1 Multifocal osteomyelitis EG pseudotumor5 year old male with painin heel and knee for 6 mos
  259. 259. Sag T-1 T-2 Gad
  260. 260. Cor T-1 Sag T-2 Axial T-2
  261. 261. Case #444.2 EG 2 year old male with painful foot for 2 months
  262. 262. Sag T-1 T-2 Gad
  263. 263. Axial T-1 T-2 Gad
  264. 264. Hand-Schiller-Christian Disease
  265. 265. Hand-Schiller-Christian Disease Hand-Schiller-Christian disease is the intermediate form ofhistiocytosis-X that involves predominantly children, two-thirds ofthe cases being younger than five years of age. The classic triadfor this syndrome is diabetes insipitus, exophthalmos, and singlegeographic lesions involving mostly the skull and pelvic bones.The initial lesions appear like eosinophillic granuloma and, in fact,eosinophillic granuloma can progress into a Hand-Schiller-Christiantype syndrome as the disease advances. It is common to have softtissue involvement of lymph nodes, liver, spleen, lung, brain andkidney as well as skin changes that can be seen in eosinophillicgranuloma. Histologically, the same histiocytic cells as are seenwith eosinophillic granuloma are present, along with eosinophils. Inthe later stages, foam cells and cholesterol deposits are typical.as the disease progresses and more and more soft tissue organs areaffected, the prognosis worsens with an overall fatally rate of
  266. 266. 10-30%. Treatment consists of local surgical treatment plus systemictreatment consisting of therapeutic protocols similar to those used inleukemic patients.
  267. 267. CLASSIC Case # 445 5 year male with HSC disease skull
  268. 268. reactive bonegranulomaPhotomic from edge of skull lesion
  269. 269. Photomic showing foam cells
  270. 270. Higher power of foam cells
  271. 271. Photomic showing cholesterol deposits
  272. 272. Case # 446 6 year female with HSC disease skull, spine and pelvis
  273. 273. Pelvis
  274. 274. Spinal lesions
  275. 275. Biopsy photomic
  276. 276. Photomic showing cholesterol deposits
  277. 277. Letterer-Siwe Disease
  278. 278. Letterer-Siwe Disease Letterer-Siwe disease is the least common of the histiocyosescomprising about 10% of all histiocytic disorders. It is a pro-gressive, acute syndrome in children under three years of age,involving multiple visceral organ systems, such as the spleen,lymph nodes and skin, associated with purpura, bleeding gums,and multiple lesions similar to those seen in Hand-Schiller-Christian disease. The skull and pelvis are frequently involved.The skeletel lesions tend to be more diffuse than with the otherhistiocytoses and take on a picture similar to that of leukemia ordiffuse lymphoma. These patients usually die of bacterial infectionswithin one or two years of their acute clinical onset because of bonemarrow suppression. Histologically, the lesions look very similarto eosinophillic granuloma or Hand-Schuller-Christian disease,although it is unusual to see foam cells in this form of histiocytosis.Treatment consists of chemotherapeutic agents similar to thoseused in leukemia.
  279. 279. CLASSIC Case #447 3 year female with LS disease skull
  280. 280. AP view
  281. 281. Chest x-ray with diffuse involvement ribs, scapulae & humeri
  282. 282. Diffuse involvement pelvis and hips
  283. 283. Bilateral humeral involvement
  284. 284. Photomic showing histiocytes and eosinophils
  285. 285. giant cell histiocytepolyPhotomic showing giant cell, polys & histiocytes
  286. 286. Case # 448 15 month male with LS skull
  287. 287. T-12 collapse
  288. 288. Femoral disease
  289. 289. Photomic
  290. 290. Case #449 3 year male with LS skull
  291. 291. Femoral disease
  292. 292. Case #450 2 year old female with LS skull
  293. 293. Sinus Histiocytosis Rosai-Dorfman’s Disease
  294. 294. Sinus Histiocytosis Rosai-Dorfman’s Disease Sinus histiocytosis is a rare and new variant of the histiocytoseswhich is characterized by enlarged lymph nodes in the head andneck area in 80% of cases along with bony involvement in 40%of cases. It is an aggressive form of the histiocytoses that is seen inteen agers and young adults. Symptoms may include fever, weightloss and malaise. The bony lesions may be solitary or multifocaland suggest inflammatory disease such as chronic osteomyelitis orEG. The pathology shows mononuclear or multinuclear giant cellswith lymphs in the cytoplasm of the giant cells. Other inflammatorycells such as plasma cells, lymphocytes and foamy histiocytes maybe seen. 10% of those with bone lesions die of the disease from extra-skeletal involvement of the lungs and kidneys.
  295. 295. Case #450.1 Rosai-Dorfman’s disease 52 year old female with knee pain for 1 year
  296. 296. Sag T-1 T-2 Gad
  297. 297. Axial T-1 T-2 Gad
  298. 298. Cor T-1 T-2 Gad
  299. 299. Post op X-ray

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