VGT Voorjaar 2010Deel 2

Iris ten Katen

Foetale lobulatie van de nier is echografisch zichtbaar als insnoering van de cortex ter hoogte van een pyramide.

Onjuist

The fetal kidneys are subdivided into lobes that may be separated by grooves.

This lobulation usually diminishes by the end of the fetal period. Sometimes these lobulations persist into adulthood

May be misdiagnosed as a renal tumor or a scarred kidney

Lobulation is characterized on sonography by the presence of renal surface indentations that overlie the space between the pyramids

Scars related to renal infarcts are typically situated between pyramids.

Indien een totale verdubbeling van het verzamelsysteem van de nier aanwezig is, is er vaker reflux in het onderpoolssysteem dan in het bovenpoolssysteem.

Juist

This rule applies to children with complete duplication of the urinary tract

The upper pole uereter inserts medially and caudal to the ipsilateral normal ureteric orifice and is often associated with a ureterocele

♀:♂ 5:1

Een vertebra plana is een manifestatie van Langerhans cel histiocytose.

Juist

Proliferatation of a histiocyte (Langerhans cell) = macrofaag

In most cases self-limiting Different types:

◦ Letterer-Siwe disease – children < 6 months, poor prognosis

◦ Hand-Suller-Christian disease – slowly progressive, children 3-6 years

◦ Eosinophilic granuloma (75%) – limited to the bone, children 6-10 years and young adults. Good prognosis. Limited to skeletal involvement: anterior skull, ribs,

femur, pelvis, spine, humerus. Pathogonomic: vertebra plana

Vertebra plana represents marked flattening of a vertebral body with relatively preserved intervertebral disk space.Eosinophilic granuloma is the most common cause; other causes include: multiple myeloma, metastatic disease, Ewing sarcoma, lymphoma, leukemia, Gaucher disease, TB, aneurysmal bone cyst, infection etc.

Onder de leeftijd van 5 jaar komt een Ewing sarcoom vrijwel niet voor.

Juist

Vaker bij ♂ 2:1 Meest voorkomende bottumor in eerste

decade, in tweede decade na osteosarcoom op 2e plaats

Zelden voor de leeftijd van 5 jaar (piek 5-15)

30-40% presenteert zich met pijn , koorts, leukocytose.

Vaak lange pijpbeenderen, daarna WK en ribben

Poorly defined margins, lytic lesion Onion skin appearance, multiple layers of

periosteal new bone appear Hair on end appearance, in which thin bone strands

extend at right angles to the parent bone Large soft tissue component Frequent bone metastases (15-25%

gemetastaseerd bij presentatie) early detection with scintigraphy

5-years survival approaches 50% DD: non Hodgkin-lymphoma, osteosarcoma,

osteomyelitis, metastatic neuroblastoma

- Axial fat-suppressed proton-density MR image shows abnormal high signal intensity in the medullary canal of the fibula, as well as in the large soft tissue mass surrounding the fibula.

Als beide heupen door de ziekte van Perthes zijn aangedaan verlopen de afwijkingen synchroon

Onjuist

Idiopathic avascular necrosis of the femoral head

Most common cause of hip pain in the young child (4-8 years old)

Bilateral (but not symmetric!) involvement in <20%

Often delayed maturation (>2SD)

Fam history in < 10% Cause unclear, ischemic /

trauma? ♂:♀ → 5:1

CR: subchondral fractures (frog-leg lateral view) → can progress to fragmentation and flattening of femoral head

In 75% slight lateral displacement of fem head

DD bilateral avascular necrosis: epiphyseal dysplasia, Gaucher disease, sickle cell disease

DD unilateral: SCFE (slipped capital femoral epiphysis), chronic dislocation of the hip

Advanced LeggCalvePerthes' disease with flattening and fragmentation of the femoral epiphysis and focal lucencies within the metaphysis.

Benigne macrocrania en idiopathische externe hydropcephalus zijn twee termen voor dezelfde afwijking.

Hierbij is er een kleine perifere liquorruimte

Onjuist

Seen in children between 6 months and 2 years The head circumference is above the 97th

percentile After the age of 2 years the head size normalizes The cause is not known It is usually characterized by prominent

subarachnoid fluid with minimal or no ventricular system dilation

Normal subarachnoid space measures < 3.3 mm

Bij ongeveer een kwart van de patienten met een myelomeningocele is een Chiari 2-malformatie aanwezig.

Onjuist

A myelomeningocele is the most serious form of spina bifida

Type I involves the extension of the cerebellar tonsils into the foramen magnum, without involving the brain stem. Most common. Asymptomatic

Type II (classic CM), involves the extension of both cerebellar and brain stem tissue into the foramen magnum.  Also, the cerebellar vermis may be only partially complete or absent.  Type II is usually accompanied by a myelomeningocele

The brain and spinal cord were examined with MR imaging in 30 myelomeningocele patients. All patients had Chiari malformations, 28 of type II and two of type I

De afstand tussen de dens en de achterzijde van de voorste atlasboog mag bij kinderen maximaal 5 mm zijn.

Juist

Distance between the anterior surface of the dens and the posterior surface of the tubercle of C1 is usually 3 mm or less in adults and 5 mm or less in children◦ Synonyms: predentate space, predental space,

atlantodental distance The distance may increase slightly on

flexion in children but is usually unchanged between flexion and extension in adults

‣ Down syndrome (due to laxity of the transverse ligament)‣ Grisel syndrome (atlantoaxial subluxation associated with

inflammation of adjacent soft tissues of the neck)‣ RA (From laxity of the ligaments and destruction of the

articular cartilage)‣ Osteogenesis imperfecta‣ Neurofibromatosis‣ Other arthridities (psoriasis, lupus)

Jongen van 16 jaar. Pijn in rechterlies na voetballen

Het beeld past bij een traumatische avulsie van de spina iliaca anterior inferior.

Juist

 This injury usually occurs with an extension moment to the hip joint, with the knee flexed, and it is commonly seen in sports that involve kicking.

AIIS avulsion fractures should be a diagnostic consideration in patients with pain in the groin who are involved in activities requiring high-level forces of flexion of the hip.

Jongen van 3 maanden met assymetrisch gelaat.

Het beeld past bij een scaphocephalie.

Onjuist

= premature fusion of the sutures

Saggital suture dolichocephaly

Unilateral fusion of coronal sutureHarlequin shaped orbit

Fusion of metopic sutures

Bilateral fusion of coronal sutures

Cloverleaf skull (fusion of sag, cor and lambdoid sutures)