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2. 8 week old girl, vomiting, starting in the 6th week of life. Spewing and exhausted vomiting (not bilious) almost after every meal.  

4. Ultrasound 1: Pylorus transversal, thickened muscularis (5-6 mm). The ring of muscularis does not frequently appear (like also in this picture) circular and of low echo density.

5. Non-innate hypertrophy of the sphincter muscle (longitudinal muscle is practically not afflicted), which bulges into the antrum. Pathologically, if the diameter of the pylorus is over 14 mm as well as a length of 16mm (where the length is not the only thing that is important), a pyloric stenosis is noted. The thickness of the muscularis propria must be surpass 4mm in an 8 week old baby; in younger children (4 weeks), the limit of 3 mm is set.The illness afflicts, according to literature, one in 500 babies (in our observation, this number is high) and is often familial (6.9% postive family histories, concordance in identical twins), the ratio of boys to girls is 4:1.

6. HPS • The pathogenesis is unclear. An underdeveloped plexus myentericus, hypoganglionoses as well as a problem in NO-synthetase have been discussed as causes.The diagnosis is made, aside from the clinical symptoms, by ultrasound. Depiction of the pylorus horizontally and longitudinally is obligatory (using at least a 7.5 MHz transponder). Next to the above-mentioned criteria, a longer examination time is important, so that one can document the insufficient passage of fluid and air through the pylorus. Noted as indirect signs are: full stomach for more than 2 hours after meals with increased peristalsis (in restless children, hard to differentiate).

7. Case 2: Osteopetrosis • Fourteen week old female where blindness was noticed. Ophthalmologist found an atrophic optic nerve. Further examination showed the following:- nanism- anemia- hepatomegaly  

8. Osteopetrosis: Marble Bone disease

10. Marble Bone disease: Infantile malignant type

11. Choledochal cyst • 10 years old male patient complains of on and off right upper abdominal pain since last 3-4 months.  

12. Choledochal cyst • Congenital dilatation of the biliary tract, so called choledochal cyst, is classified based on spectrum of morphologic changes in the bile ducts. The most common classification scheme of Alonso-Lej includes type I, which is dilatation of the CBD; type II, which is diverticulum of the CBD; and type III, which is a rare choledochocele. • Todani and his colleagues further added to this classification type IV-A, representing multiple cysts of the IHBDs and EHBDs; type IV-B, representing multiple cysts of EHBDs; and type V, representing multiple cysts of the IHBDs , or Caroli disease.The choledochal cyst is not a true cyst of the biliary tract but rather some variation of duct dilatation. Etiology is unknown but supposedly multifactorial.

13. Choledochal cyst • On CT choledochal cyst can have varying appearance depending on the extent of ductal involvement and the degree of dilatation. There may only be mild EHBD dilatation or a large water density mass in porta hepatis or adjacent to the head of the pancreas.Reportedly, 60% of choledochal cyst will have associated congenital IHBD dilatation..

14. Congenitally dilated IHBD often have a lobulated cystic appearance with an abrupt transition zone at the junction with the normal ducts.Acquired biliary dilatation usually doesn't have the lobulated cystic appearance and the dilated ducts usually taper gradually towards the periphery of the liver. • Direct communication of the cystic duct to the dilated EHBDs will aid making the diagnosis of choledochal cyst.Ultrasonography aids to differentiate with other pathology. • DISIDA scan may be required. CT and MR cholangiography are diagnostic. • This is a case of type I disease with tiny calculi both within gall bladder and choledochal cyst.  

16. Thalassemia Intermedia • 9 year old girl, paleness, hepatosplenomegaly •  Autosomal hereditary hemoglobin defect with a resulting hypocromatic, microcytic anemia. Through hemolysis is an iron overload common. Extensive medullary and extramedullary bleeding Autosomal hereditary hemoglobin defect with a resulting hypocromatic, microcytic anemia. Through hemolysis is an iron overload common. Extensive medullary and extramedullary bleeding

17. X-Ray 3: Hand: Trabelces are affected in the metacarpals. Widening of the bone marrow and thinning of the cortical zone through the hypertophied blood-producing marrow.. Further typical changes of the increased erythropoeisis is seen in the ribs, facial skeleton and in the vertebral bodies are possible not shown.In the MRI (T2): Signal reduction in bone marrow, in the liver and in the spleen (iron deposition

19. Macrocephaly with widend diploe, thinned tabula externa and subperiostal spiculae. Holes in the tablua externa are possible.

21. Case study 1 • 5 10/12 year-old boy. Treatment for ALL. Findings after a two-time hemopoietic stem cell transplant. • Now, double vision, discrete exophthalmus on the right side, incomplete oculomotor nerve paralysis on the right side.  

22. Comment on each of the following slides

23. MRI 1: T2-transversal with fat saturation: Diffuse, signal intense mass in the retrobulbar space to the right around the lateral rectus muscle. Expansion to the tip of the orbital siphon. The meningeal structures are not able to be distinguished for certain from the mass in the area of the optic nerve foramen and the optic nerve.

24. MRI 2: T1-SE- transversal before contrast application : Mass with low signals in the right retrobulbar space.

25. view MRI 3MRI 3: T1-SE transversal with fat saturation after contrast: Mass in the right retrobulbar space, expansion to the tip of the orbital siphon.The meningeal structures are not able to be distinguished for certain from the mass in the area of the optic nerve foramen and the optic nerve.

26. What is the diagnosis? • Lymphoblastic leukemia, the most common form of leukemia in children.   Retrobulbar prolapseImage: head and neckLikely etiology: neoplastic Pathophysiology/pathomorphology

27. Which DD would be also possible with the radiological findings? • In this case, with known underlying illness, this was an obvious finding. Intraorbital tumor of other cause.Orbital pseudotumor - often only slight contrast media uptake.  

28. What is the Course / Prognosis? • 4 types of ALL - depending on cell/surface marker - are able to be categorized.-B-cell ALL (70%)-T-cell-ALL (20%)-B-/T-cell-ALL (2%)-0-cell-ALL (missing surface marker).  

29. Case study 2 • 9/12 year-old boy. Reoccurring respiratory infections. In the X-ray (sent to us from another clinic), segmental, basal opacities on the left side.

30. MRI 1: T2-HASTE coronal: conically configurated, ca. 4x2cm large structure in the left dorsobasal lung, In the T2-weighted image, an obvious signal intensity increase is seen

31. view MRI 2MRI 2: T1-FLASH coronal with contrast: strong, homogenic, aortic synchronized enhancement of the sequestration after application of contrast. No aerated areas within this structure.

32. view MRI 3MRI 3: MRA (MIP-Projections): Proof of the systemic arterial vasculature from the aotra and the venous drainage in the hemiazygos vein.

33. What is the likely diagnosis • Region-organ: Thorax - Lung • Congenital • The presented case is the classical example of a lung sequester. Here, probably an extralobular sequester

34. Pathomorphology or Pathophysiology of this disease • Pulmonary sequester = "Rokitansky-lobe"Congenital malformation of the lung.A mass of non-aerated pulmonary tissue that is not connected to the normal bronchial tree. It receives its own blood supply from a systemic artery.In 65% of the cases in the left lobe, otherwise in the right, posterior lower lobular segment.Two forms:Intralobular (75-85%) - without own pleura, drainage into the pulmonary veins.Extralobular (15-25%) - with own pleura, drainage into the vena cava/azygos vein.

35. What other investigation can be done for diagnosis confirmation? • Imaging including endoscopy  

36. Which DD would be also possible with the radiological findings? • In obvious sequester-vascular supply: none.  

37. Give etiology and management? • Congenital deformity.Often secondary hematogenic infection with delayed healing and sustained inflammatory symptoms due to missing aeration (no self-cleaning).Mostly, surgical resection. Sometimes also prophylactically due to the danger of infection.  

38. Case study 3 • 11 year old boy; for 2 weeks, persisting, mainly dry cough. Subfebrile temperatures to moderate fever of 39°C.  

39. X-Ray 1: Thoracic image on the day of introduction. Transparency is decreased in the right middle and lower lobes. In the p.a. image, it is not able to be determined in which lobe it is located (Interstitial findings - ground glass).

40. X-Ray 2: Thoracic image after 14 days of erythromycin treatment: Normal thoracic findings.

41. Describe the radiological findings • Interstitial pneumonia = reaction of the lung due to various noxious agents, predominantly in interstitium, but also later in the alveolar space. X-ray: interstitial (reticular) opacity through thickening of the interlobular septae, mostly striped, peri-bronchovascular, perihilar and subpleural opacities. Disseminated, discrete nodules.= reticular, striped and nodular lung opacities, diffuse milky murkiness (but also alveolar infiltration with discrete to obvious infiltrates with invasion of the alveolar sparce possible). Increased intrathoracic lymph nodes are often seen in viral pneumonias. Pleural effusions and scarring are seldom (differential diagnosis: bacterial superinfection).  

42. What is the diagnosis and pathomorphology-pathohysiology? • Thorax- lung • Inflammatory-Infectious • Most possibe diagnosis is Mycoplasma Pneumonia • Inflammation of the lung due to infection with mycoplasma. Predominantly inflammatory reaction of the lung interstitium.

43. What is the D.D.? • Pathomorphological display and clinical symptoms of pneumonias are often similar, even when caused by different agents. The radiological display usually does not direct us to the agent.The different interstitial-alveolar opacities give a hint of which agents may come in question:- interstitial: Viruses, "atypical" agents (mycoplasma, chlamydia, rickettsia), fungi- alveolar: Bacteria (pneumococci, legionella, klebsiella).Even allergic, physical and chemical noxious agents can cause a pneumonia.

44. Case 4 • 5 month-old infant, only slightly delayed statomotoric development since the 3rd month of life.Clinical signs are often initially seen in adulthood due to a problem with CSF circulation (intracranial pressure signs, epileptic episodes).  

45. MRI 1: Findings of a (in this case unusually large) CSF-isointense tumorous, cystic cavity, here temporally on the right side. Gyration and cortex of the brain is intact (in contrast to an infarction). Shown is a coronal slice, T2-weighted.

46. view MRI 2MRI 2: Transversal slice, T1-weighted

47. view MRI 3MRI 3: Coronal slice, T2-weighted after surgical, internal rerouting of the arachnoidal cyst. The brain has completely expanded, no defect is detectable. Particularly common is the temporal localisation, but basal and in interhemispheric cisterns have also been seen. Follow-up after surgical intervention

48. What is the diagnosis- pathomorphology and pathophysiology? • Head- brain and nerves • Congenital in origin possibly arachnoid cyst • Spaces filled with fluid due to duplication or splitting of the arachnoid, (Gap formation between the arachnoid and the pia mater), congenital cause, sometimes dysplasia of the surrounding brain regions possible (i.e. temporal lobe).  

49. What is the D.D. • Other an arachnoid cyst: • Infarction - (respective of the cortical surface by the arachnoidal cyst).  

50. Course-prognosis? • To clarify the question of a communication with the subarachnoidal space, an intrathecal injection of contrast media is necessary. Surgical indication is given by an obvious tumor and absence of communication with the subarachnoid space.