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43 Cards in this Set

  • Front
  • Back

Incidence of MDS in general population?


For mother with one MDS?


For pt with MDS?

1:1000


1:20-50


1:40

Prevention?

400 micrograms of folic acid in all child bearing women decreases incidence 50%

What is the difference between meningocele, myelomeningocele, lipomyelomeningocele?


What is most common?

Meningocele occurs when just the meninges but no neural elements extend beyond the confines of the vertebral canal


Myelomeningocele, neural tissue, either nerve roots or portions of the spinal cord, has evaginated with the meningocele


3. lipomyelomeningocele denotes that fatty tissue has developed with the cord structures and both are extending with the protruding sac.


Myelomeningocele accounts for more than 90% of all open spinal dysraphic states

Level of spine involved from most to least common?

What is the associated abnormality if CSF is leaking?

In 85% of affected children there is an associated Arnold-Chiari malformation, in which the cerebellar tonsils have herniated down through the foramen magnum, obstructing the fourth ventricle and preventing the cerebrospinal fluid from entering the subarachnoid space sur- rounding the brain and spinal cord.

Is the neurologic lesion predictable based on spinal level?

Variable, depending on what neural elements, if any, have everted with the meningocele sac. The bony vertebral level often provides little or no clue to the exact neurologic level or lesion produced

Features of UMN lesion?

Overactive detrusor, exaggerated sacral reflexes, absence of voluntary control over sphincter function, DSD, and no EMG evi- dence of denervation potentials in the sphincter.


Bladder tends to be trabeculated with a closed bladder neck on VCUG or US

Features of LMN lesion?

Acontractile detrusor and partial or complete denervation of the external urethral sphincter with diminished or absent sacral reflexes may be seen.


Bladder is usually small and smooth walled with an open bladder neck

Neonatal assessment? Normal bladder capacity in neonate?

Normal is 10 to 15 mL; therefore, a residual urine volume of less than 5 mL is acceptable. Urinalysis and culture, serum creatinine determination, and careful neurologic examination of the lower extremities.


VCUG and renal US

What percentage of neonates has abnormal GU tract on imaging?

15-20%

What are the 3 types of lower urinary tract findings on UDS?

Synergic (26%)


Dyssynergic with and without poor detrusor compliance (37%)


Complete denervation (36%)

What is one treatment choice for filling pressures >40 cm H2O?

CIC alone or in combination with anticholinergic agents, when detrusor filling pressures exceed 40 cm H2O and voiding pressures are higher than 80 to 100 cm H2O,

How should these children be followed?

Incidence of VUR in MDS?

3-5%

3 indications for vesicostomy?

(1) Severe reflux that CIC and anticholinergic medication fail to improve upper urinary tract drainage


(2) whose parents cannot adapt to the catheterization program


(3) poor candidates for augmentation cystoplasty

Indications for anti reflux surgery?

1. Recurrent symptomatic (febrile) urinary infection while receiving adequate antibiotic therapy and appropriate catheterization tech- niques


2. persistent hydroureteronephrosis despite effective emptying of the bladder and lowering of intravesical pressure


3. severe reflux with an anatomic abnormality at the UVJ


4. reflux that persists into puberty


5. Reflux in any child undergoing surgery to increase bladder outlet resistance.

What metabolic derangements are seen with gastric augmentation?

Hyponatremic hypochloremic metabolic alkalosis

Who gets enterocystoplasty?

Persistently non-compliant or overactive detrusor

Who gets a continent urinary diversion?

Intractable urethral incompetence despite bladder outlet surgery or to make it easier to cath pts who can't cath via native urethra

What UDS parameters does botox improve?

Dcreased maximum detrusor pressure (33% to 57%)


Increased maximum cystometric capacity (34% to 165%)


Improved compliance (121% to 183%)

Why is bowel dysfunction unpredictable?

External anal sphincter is innervated by the same (or similar) nerves that modulate the external urethral sphincter, whereas the internal anal sphincter is under the influence of more proximal nerves from the sympathetic nervous system. In addition, the internal anal muscle reflexively relaxes in response to anal distention

Tx for fecal incontinence?

Diet, medications, and manual evacuation fail to achieve predictable bowel emptying without soiling, MACE, cecostomy are options

8 occult spinal dysraphisms?

What percentage have skin abnormality? What are they?


What do you see in the legs?

90%


Hairy tuft, asymmetric gluteal cleft, lipoma, dermocutaneous vascular malformation


High arched foot or feet; hammer toes or claw toes; a discrepancy in muscle size, shortness, and decreased strength in one leg compared with the other, typically at the ankle; and/or a gait abnormality

What percentage have abnormal UDS? What are the common findings?



What is seen if dx after 3yo for UDS abnoramlities?

1/3< 18mo


UMN lesions- overactive detrusor/hyperactive spinal reflexes



92% have abnormality


UMN and LMN combination

What are 3 reasons why lipomas invariably cause UMN?

(1) compression on the cauda equina or sacral nerve roots by an expanding lipoma or lipomeningocele


(2) tension on the spinal cord from tethering secondary to differential growth rates in the bony vertebrae and neural elements while the lower end of the cord is held in place by the lipmoa or by a thickened filum terminale


(3) fixation of the split lumbosacral cord by an intra- vertebral bony spicule or fibrous band

What percentage of abnormal UDS normalize in infants and older children after detethering?

50% of infants with abnormal UDS go normal post-op


30% improve


10% become worse with time.


In older children there is a less dramatic change after surgery: 27% becoming normal, 27% improving, 27% stabilizing, but 19% get worse with time

Define sacral agenesis. What is a potential causative factor?

Absence of part or all of two or more lower vertebral bodies. The cause of this condition is still uncertain, but teratogenic factors may play a role, because insulin-dependent diabetic mothers have a 1% chance of giving birth to a child with this disorder

What is the bimodal presentation distribution for SA?



What may be the only sign?

More than three fourths of the disorders being detected in early infancy and the remainder discovered between 4 and 5 years of age



The only clue, besides a high index of suspicion, is flattened buttocks and a low, short gluteal clef

What do you see on UDS for SA?

Equal number of individuals manifest either an upper or a lower motor neuron lesion (35% vs. 40%, respectively); 25% have no sign of denervation

What are the treatment principles of neurogenic bladders?

Incidence of imperforate anus


M:F ratio?

1:4000-5000


1.5:1 M:F

What is the Wingspread Classification of Anorectal Malformations?



36% high, 14% intermediate, 47% low, and 1% a cloacal lesion.

What are all the subclasses in the imperforate anus Wingspread classificiation?

Most common associated urinary tract abnormalities?


Urinary tract abnormalities have been noted in 26% to 52% of affected children, with:


renal agenesis (primarily left-sided) and vesicoureteral reflux as the most common associated findings

Which IA pts get UDS? (3)


1. Bony abnormality of the spine


2. spinal cord defect


3. telltale signs of dysfunction on VCUG or renal ultrasonography


IA UDS findings?


Most common - UMN lesion with an overactive detrusor and/or DSD, but a lower motor neuron lesion with an acontractile detrusor and a denervated sphincter may be seen as well

Incidence of Cerebral Palsy?

1.5:1000

What are the classic findings on UDS?


Forty- nine (86%) had the expected picture of a partial upper motor neuron type of dysfunction, with exaggerated sacral reflexes, an overactive detrusor and/or DSD

What is the UDS findings in T level trauma?

Overactive detrusor with DSD, high voiding pressures, eventual hydronephrosis, and vesicoureteral reflux.

What's different about traumatic lesions above T6?

Autonomic dysreflexia, need alpha blockers for UDS

Tx of traumatic SCI?


How to differentiate spinal shock on UDS?

Indwelling, then CIC as soon as able.


After 4 to 6 weeks, if there is no improvement in lower urinary tract function, urodynamic studies are conducted to determine whether the condition is the result of spinal shock or an actual nerve root or spinal cord injury. An acontractile detrusor is not uncommon under these circumstances. On the other hand, EMG recording of the sphincter often reveals normal motor units without fibrillation potentials but absent sacral reflexes and a nonrelaxing sphincter with bladder filling, a sign that transient spinal shock has occurred



Goal is keep pressures below < 40cm h20

Long term management of SCI?

UDS within 2 to 3 months after the injury, again 6 to 9 months later, and possibly at 2 years after the trauma to determine the stability of lower urinary tract function, the need for continued CIC, and whether adjuvant drug or surgical therapy should be added.


Renal US early and VCUG if signs of BOO are present on UDS or if recurrent UTI.


Radionuclide cystography is indicated if the patient has repeated UTI or develops hydronephrosis. Because stone formation can be insidious, periodic imaging of the kidneys and bladder is necessary. Early identification and proper management may prevent the signs and effects of bladder outlet obstruction before they become