Treatment
If suspected antenatally, a consultation with a paediatric surgeon/ paediatric urologist maybe indicated to evaluate the risk and consider treatment options.Treatment is by endoscopic valve ablation. Fetal surgery is a high risk procedure reserved for cases with severe oligohydramnios, to try to limit the associated lung underdevelopment, or pulmonary hypoplasia, that is seen at birth in these patients. The risks of fetal surgery are significant and include limb entrapment, abdominal injury, and fetal or maternal death. Specific procedures for in utero intervention include infusions of amniotic fluid, serial bladder aspiration, and creating a connection between the amniotic sac and the fetal bladder, or vesicoamniotic shunt.There are three specific endoscopic treatments of posterior urethral valves:
Vesicostomy followed by valve ablation - a stoma, or hole, is made in the urinary bladder, also known as low diversion, after which the valve is ablated and the stoma is closed. Pyelostomy followed by valve ablation -  stoma is made in the pelvis of the kidney as a slightly high diversion, after which the valve is ablated and the stoma is closed
Primary (transurethral) valve ablation - the valve is removed through the urethra without creation of a stomaThe standard treatment is primary (transurethral) ablation of the valves. Urinary diversion is used in selected cases, and its benefit is disputed.Following surgery, the follow-up in patients with posterior urethral valve syndrome is long term, and often requires a multidisciplinary effort between paediatric surgeons/ paediatric urologists, paediatric nephrologists, pulmonologists, neonatologists, radiologists and the family of the patient.
Spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME), sometimes called Jankovic–Rivera syndrome, is a very rare neurodegenerative disease whose symptoms include slowly progressive muscle (atrophy), predominantly affecting proximal muscles, combined with denervation and myoclonic seizures. Only 12 known human families are described in scientific literature to have SMA-PME.SMA-PME is associated with a missense mutation (c.125C→T) or deletion in exon 2 of the ASAH1 gene and is inherited in an autosomal recessive manner. SMA-PME is closely related to a lysosomal disorder disease called Farber lipogranulomatosis. As with many genetic disorders, there is no known cure for SMA-PME. The condition was first described in 1979 by American researchers Joseph Jankovic and Victor M. Rivera. ASAH1 gene
The ASAH1 gene codes for acid ceramidase, an enzyme found in lysosomes. The lysosome breaks down acid ceramidase; the fatty acid component  is then used to produce myelin. Myelin is an insulating coating around the neurons in the body which helps to contain bioelectrical signals along a nerve cells axon and increase transmission rate. In patients with SMA-PME, the ceramidase function decreases to 33.33% effective. The lack of myelin resulting from the lack of acid ceramidase breakdown leads to nerve cell dysfunction. See also
Spinal muscular atrophies
Progressive myoclonus epilepsy

References
Further reading
National Institutes of Health (December 2013). "Genetics Home Reference: Spinal muscular atrophy with progressive myoclonic epilepsy". == External links ==