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This Blog contains practically relevant interesting cases with relevant references, links and images.
Type | Presentation | Other notes |
---|---|---|
I | Is generally asymptomatic during childhood, but often manifests with headaches and cerebellar symptoms. Herniation of cerebellar tonsils. | The most common form. |
II | Usually accompanied by a myelomeningocele leading to partial or complete paralysis below the spinal defect. Abnormal development of the cerebellar vermisand medulla oblongata occur, and they both descend into the foramen magnum. Hydrocephalus is frequently present. | |
III | Causes severe neurological defects. It is associated with an encephalocele. | |
IV | Characterized by a lack of cerebellar development. |
Complications of horseshoe kidney include the following:
Shiny corner sign, also known as a Romanus lesion, is an early spinal finding in ankylosing spondylitis. These represent small erosions at the superior and inferior endplates (corners on lateral radiograph) of the vertebral bodies, with surrounding reactive sclerosis. Eventually the vertebral bodies become squared.
On MRI the 'shiny corners' appear as areas of increased T1 signal due to focal fatty marrow as a result of chronic inflammation. MRI however can detect changes earlier than x-ray manifestations, when there is initially increased T2 signal on STIR and decreased signal on T1WI. At this stage plain films appear normal.
CCAM is a congenital anomaly of the terminal brochioles which results in a dysplastic, multicystic mass. These cysts usually communicate with the bronchial tree. At birth, these cysts are fluid-filled. Ultimately, communication with the airway allows for the fluid-filled mass to become air-filled. Whether fluid-filled or air-filled, the cysts become increasing larger, exert mass effect, compress mediastinal structures and lung parenchyma.
Infants less than one year old usually present with respiratory distress. Usually, the abnormality is confined to one lobe. There are three types of CCAM:
Treatment: Surgical lobectomy is curative with Type I. There is increased morbidity and mortality with Type II and II because the lesions are often larger at presentation.
References:
A posterior urethral valve is an abnormal congenital obstructing membrane that is located within the posterior male urethra; this valve is the most common cause of bladder outlet obstruction in male children. A posterior urethral valve occurs in approximately 1 in 5000 live male births.
There are 3 types of posterior urethral valves.The type I valve is a bicuspid valve that radiates distally from the posterior edge of the verumontanum to the anterior proximal membranous urethra. It account for 95% of all valves. The type II valve is no longer considered an obstructing valve; rather, it is thought to be a sequela of voiding dysfunction. It was described as a hypertrophic band of superficial muscle that runs along the posterolateral wall of the urethra from the ureteral orifice to the verumontanum. Therefore, these valves can be differentiated from type I and type III valves by their location proximal to the verumontanum. The type III valve is a circumferential membrane or diaphragm that is located at the membranous urethra, which is thought to result from the incomplete regression of the urogenital membrane during embryogenesis. There is a central aperture, and the central portions of the ring may prolapse into the more distal urethra during voiding, which results in a wind-sock appearance on VCUG. Type III valves account for almost 5% of all valves.
VCUG (Voiding cystourethrography) is considered the diagnostic study of choice for the evaluation of posterior urethral valves. The bladder is typically thickened with trabeculae and may exhibit vesicoureteral reflux or, less commonly, diverticula. The bladder neck is typically hypertrophic, leading to a lucent ring or collar. On voiding, the posterior urethra is dilated (ie, shield shaped), and valve leaflets may be seen as lucencies, giving the appearance of a spinning top. If leaflets are not visible, a commonly associated finding of posterior urethral bulging distally over the bulbar urethra may be noted. The anterior urethra is typically underfilled, and voiding is incomplete.
IVP is not routinely used in children because the contrast agent is poorly concentrated and visualized in newborn kidneys, particularly if renal function is diminished. Elevated serum creatinine levels may preclude the use of intravenous (IV) contrast material. IVP can show an absent kidney in the case of renal dysplasia or delayed renal function with persistent high intraluminal pressures. Hydroureteronephrosis may be seen. Delayed images may show bladder or urethral pathology, but the lower urinary tract is better visualized with VCUG.
CT scans with IV contrast enhancement may reveal dysplastic and/or dilated kidneys with delayed renal function and excretion, hydroureter, dilated bladder with wall thickening, trabeculation, and diverticula. A dilated posterior urethra might be seen, although leaflets may be easily missed. Elevated serum creatinine levels generally preclude use of IV contrast material. MRI findings are similar to those of CT scanning except that enhancement with IV gadolinium-based contrast agents may allow functional as well as anatomic assessment. Nuclear cystography has no role in the diagnosis of posterior urethral valves because of the poor anatomic detail, but this modality can depict the presence of vesicoureteral reflux. However, grading of such reflux is not as accurate as with contrast VCUG.
Differential diagnosis of cecal wall thickening associated with neutropenic colitis includes lymphomatous or leukemic intramural deposits and hemorrhage. Lymphomas and leukemia often occur simultaneously, and both diseases may involve the bowel wall. Intramural hemorrhage and lymphomatous or leukemic deposits present as focal eccentric rather than diffuse bowel wall thickening. An abscess related to appendicitis and neutropenic colitis may exhibit similar clinical presentation. Appendicitis is a rare and deadly complication of acute leukemia and has been linked to drug toxicity. On CT, a periappendiceal abscess usually appears as a low-density mass with sharp or irregular margin. An enhancing rim or intrinsic air bubbles may be observed. Cecal wall thickening, however, is not an expected CT finding in appendicitis.