What Is Lower Urinary Tract Obstruction
A lower urinary tract obstruction is a rare fetal condition that occurs when there is a blockage in the urinary tract of a developing fetus. The urinary tract:The urinary tract consists of the organs that produce and store urine:
The urinary bladder
As a fetus grows in the womb, urine made by the kidneys flows through the ureters into the bladder. Urine is stored in the bladder until it gets full. When the bladder is full, it pushes the urine out of the body through a tube called the urethra. The kidneys and ureters are called the upper urinary tract and the bladder and urethra are called the lower urinary tract. LUTO occurs when the flow of urine is blocked from exiting the body at the level of the lower urinary tract. When LUTO occurs, all parts of the urinary tract that lie above the obstruction may become swollen with urine that cannot drain. Over time this blockage can lead to permanent kidney damage. When urine can no longer be drained, the fluid around the fetus decreases. This can lead to lung damage because the fetus must move amniotic fluid in and out of the lungs in order for them to develop properly. Therefore, LUTO can lead to damage in more than one organ system.
Comfort From A Distance
Chance is now 11 months old. Hes had some urinary tract infections that have landed him in the hospital, but hes doing well otherwise. Hes a happy baby, says his mom.
The staff and care we have received and continue to receive at CHOP is like no other and worth the two-hour drive, says Tahilees. Its the same team that followed him from his first days in the N/IICU. They know him best. They got to see him from day one. They schedule our appointments so we can see both teams on the same day, get back to me to answer questions, and follow up to see how hes doing after clinic visits.
A few months after birth, CHOP urologist, Aseem Shukla, MD, implanted a catheter, a thin, sterile tube, into Chances bladder. Dr. Shukla plans to gradually increase the size of the catheter tube in the hopes that it will expand Chances urethra and create a passageway wide enough for urine to pass so he can close the vesicostomy andChance can urinate naturally.
Chance is such a strong and brave boy, says Tahilees. Even before he was born, he showed me strength and determination in myself that I didnt know I had.
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What Is Fetal Echocardiography
Fetal echocardiography is performed at our center by a pediatric cardiologist . This non-invasive, high-resolution ultrasound procedure looks specifically at how the babyâs heart is structured and functioning while in the womb. This test is important because babies with birth defects are at increased risk of heart abnormalities. A fetal echocardiogram is also a necessary part of the evaluation process when prenatal surgery is being considered.
Fetal Surgery For Lower Urinary Tract Obstruction: Chance’s Story
A few days after Tahilees excitedly told her 12-year-old daughter and close family that she was pregnant, she got devastating news. A first trimester screening at 11 weeks showed the fetus had a blockage in its urethra. Read about the family’s journey from Brooklyn to find hope at CHOP.
This rare and life-threatening birth defect, known as lower urinary tract obstruction , was preventing urine from being released into the amniotic fluid space around the fetus. As a result, the bladder was enlarged and amniotic fluid levels around the fetus were lower than normal .
I was terrified and heartbroken, says Tahilees. Low amniotic fluid levels can result in underdevelopment of the lungs , a life-threatening condition. The obstruction can also cause back pressure on the kidneys, which can lead to kidney damage. It was devastating to think that I would have to tell my daughter she wasnt going to be a big sister.
The maternal-fetal medicine specialist at the hospital near Tahilees home in Brooklyn, NY, wanted to wait a few weeks to see if the blockage resolved on its own. If it didnt, the MFM said the pregnancy might require intervention before birth.
I went home and immediately began researching LUTO, says Tahilees. A lot of the medical journal articles and blog posts didnt offer much hope. I felt overwhelmed.
How Does The Packard Childrens Hospital Team Typically Care For Fetal Lower Urinary Tract Obstruction
Our comprehensive team diagnoses fetal lower urinary tract obstruction through same-day imaging, multidisciplinary counseling, and diagnostic procedures as early as the first trimester. A thorough in utero evaluation guides prognosis and treatment options specific to each case, including consideration for fetal vesicoamniotic shunt placement.
If the evaluation shows that the mother and fetus are candidates for in utero therapy, we are able to perform this innovative shunt treatment for fetuses with lower urinary tract obstruction.
- During a fetal vesicoamniotic shunt placement, a small pigtail shunt is inserted into the fetal bladder with the other end placed in the amniotic cavity.
- The shunt diverts the urine from the fetal bladder to the amniotic cavity surrounding the fetus. The shunt is then removed after the baby is born.
Criteria for vesicoamniotic shunt placement:
- Normal fetal karyotype or microarray
- Isolated anomaly
- Bladder refilling following vesicocentesis and favorable bladder/renal analytes
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Examples Of Urinary Tract Obstructions
Left: Bladder obstruction: the bladder is very distended, and there is no amniotic fluid around the fetus.Right: Urinary obstruction: the kidney is very distended , and loops of very dilated ureter are visible.
Lifespan, Rhode Island’s first health system, was founded in 1994 by Rhode Island Hospital and the Miriam Hospital. A comprehensive, integrated, academic health system with The Warren Alpert Medical School of Brown University, Lifespan’s present partners also include Rhode Island Hospital’s pediatric division, Hasbro Children’s Hospital Bradley Hospital Newport Hospital Gateway Healthcare Lifespan Physician Group and Coastal Medical.
Diagnosis Of Bladder Outlet Obstruction
Ultrasound images are used to assess the size of the bladder, and changes in the appearance of the kidneys. It also is used to check fluid variations around the baby.
Bladder outlet obstruction can be seen in babies with trisomy 13, 18, and 21. A chromosome test is recommended if there is a suspicion of an underlying problem. A fetal echocardiogram is recommended if a heart defect is suspected.
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Lower Urinary Tract Obstruction Treatment
If a LUTO seems to be isolated, fetal surgical treatment may help decrease the amount of lung and kidney damage that can occur during pregnancy. The goal of fetal treatment is to provide constant drainage of urine from the body into the amniotic fluid. This prevents urine buildup and helps to normalize amniotic fluid volume. The type of treatment used depends on where in the urinary tract the blockage occurs. Types of treatments include:
Vesicocentesis: A needle is placed into the fetal bladder to remove the urine. Multiple procedures may be needed if urine repeatedly builds up in the bladder. One in ten fetuses may require only a single vesicocentesis to resolve LUTO.
Vesicoamniotic shunt: A small, plastic tube called a shunt is inserted into the bladder to allow the flow of urine from the bladder to the outside of the fetus. The shunt remains in the bladder until the baby is born.
Fetal cystoscopy: A small, surgical camera called a cystoscope can be inserted into the fetuss urethra and bladder to remove any blockage in the flow of urine.
Not all attempts at fetal treatment are successful. Sometimes the position of the fetus makes it difficult to detect on ultrasound, making it impossible to perform a fetal therapy procedure. Even if a fetal treatment successfully decreases the amount of urine trapped in the baby, complications can still occur. The urine can build up again over time.
Fetal Intervention: Cystoscopic Laser Ablation Of Puvs
Fetal cystoscopic laser ablation of PUVs, consisting of the identification and fulguration of the PUV with a diode laser through fetal cystoscopy under local anaesthesia, has been proposed as an alternative to VAS. A 2011 systematic review of existing literature reporting on fetal cystoscopy in LUTO retrieved four papers describing 63 fetuses treated with either fetal cystoscopy or VAS placement, or expectant management. The review concluded that fetal cystoscopy laser valve ablation improved perinatal survival compared with no treatment, but no differences in perinatal survival were observed between fetal cystoscopy and VAS placement . In addition, urological fistulas have been reported in ~10% of fetuses undergoing cystoscopic laser ablation, probably owing to the difficulty in directing the laser energy in alignment with the urethral canal in some instances, depending mostly on fetal and placental position. Technological improvement and development of new devices that will enable improved flexibility in visualizing the urethral outlet and directing the laser energy might reduce the occurrence of this type of complication.
Fetuses with prenatal LUTO must be referred to a tertiary obstetric centre with multidisciplinary expertise in prenatal and postnatal management of obstructive uropathies
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Will My Baby Need Long
Yes. Because of all the potential health issues associated with LUTO, your baby will require long-term follow-up care. We will work with you to develop a detailed care plan for your baby. The plan will be implemented by a comprehensive team of specialists, including a pediatrician and a pediatric kidney specialist.
Eli’s Story: Traveling To Texas For A Lifesaving Fetal Surgery
On Oct. 31, baby Eli will be five months old, but his journey getting here was nothing short of a miracle. It all started during Lacey Prejean’s 12-week pregnancy appointment.
For more information or to schedule an appointment,
call Texas Childrens Fetal Center at 832-822-2229 or 1-877-FetalRx toll-free.
Our phones are answered 24/7. Immediate appointments are often available.
Boy Beats The Odds To Survive Rare Fetal Urinary Condition
Before he was even born, Kaleb Perry faced tough odds of survival. Just 12 weeks into the pregnancy, an ultrasound showed that a blockage in the urethra was trapping urine inside his body, a rare condition called fetal lower urinary tract obstruction .
With comprehensive treatment from a multidisciplinary team at Lucile Packard Children’s Hospital Stanford, however, Kaleb is now a smiling, dancing 3-year-old.
Initially, Kaleb’s mother, Mandy, was told that Kaleb likely wouldn’t survive the pregnancy, so she consulted with Yair Blumenfeld, MD, who directs fetal therapy at Packard Children’s for a second opinion.
” said, ‘If your baby is willing to fight, we will fight for him.’ I get emotional just thinking about it,” Mandy explained in a story on Packard Children’s blog.
LUTO occurs in about 1 in 3,000 pregnancies, but the cause remains unknown. The biggest threat posed by LUTO is low amniotic fluid levels, which can lead to problems with lung development.
As the article explains:
Babies can have a partial blockage, or a complete blockage, like Kaleb. To give him the best chance for developing healthy lungs, his doctors inserted a shunt to release the urine from the bladder into the amniotic fluid. Sometimes a shunt can become dislodged and needs to be replaced when a baby moves. Kaleb was a very active baby and so Mandy underwent multiple surgeries.
But Kaleb wasn’t out of the woods yet.
What Happens After Fetal Treatment For Luto
A baby who has received fetal treatment for LUTO will need to be delivered in a hospital with a neonatal intensive care unit . After delivery, the NICU can do more testing to figure out the cause of the LUTO. Depending on the exact diagnosis, surgery may be needed to create a permanent way for urine to leave the babys body. Therapies like dialysis and kidney transplant may also be needed. How sick a baby will be after birth depends on how severe the lung and kidney damage are and the cause of the LUTO.
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What Causes Lower Urinary Tract Obstruction
Sometimes LUTO is caused by a problem with a fetuss chromosomes or by a genetic disorder. If so, additional medical problems or organ abnormalities may be present. Sometimes in male fetuses a fold of tissue from the bladder blocks the hole that allows drainage of the bladder into the urethra. In other instances, parts of the urethra may be too narrow or completely blocked . LUTO without any other underlying conditions is called “isolated” LUTO. While ultrasounds and amniocentesis can help determine the cause of LUTO, testing after birth will give the most accurate analysis of a babys condition.
What Happens After My Evaluation Is Complete
After we have gathered all the anatomic and diagnostic information from the tests, our full team will meet with you to discuss the results and whether treatment is feasible. The treatment option that will be best for your baby will depend on several factors, including the cause, location, and severity of the urinary tract blockage. Your childâs medical team will design a treatment plan tailored to your childâs specific needs.
Outcome And Postnatal Followup
As discussed above LUTO is a condition with high perinatal mortality due mainly to pulmonary hypoplasia however, longterm outcomes are also important. After birth, the neonate must be at least assessed for renal function via plasma creatinine and renal ultrasound. These investigations should be repeated at 6weeks of age and 1year at this later stage, if possible, tubular function should be assessed using renal electrolytes. It is important to also monitor blood pressure, height and weight. At 5years of age, there should be an assessment of disability and continence by questionnaire administered to the parent.18 It is known that, as well as longterm complications of endstage renal failure, these children are at risk of bladder dysfunction , poor growth and male infertility if uraemic.19,20,21 It is thus important when counselling parents to ensure that they are aware of the still considerable uncertainty as to the development of renal failure, but that these children are expected to have normal cognitive abilities and achieve acceptable continence with medical and surgical care, with similar qualityoflife scores to a healthy child.19,21
What Is Urinary Tract Obstruction
The urinary tract consists of two kidneys , two ureters , the bladder, and the urethra . The urine should flow from the kidney, through the ureter, to the bladder, and out of the fetus through the urethra to the amniotic fluid. There are many causes of urinary tract obstruction in the fetus. Most are caused by a narrowing at some point in the urinary tract. This narrowing can slow down or stop the flow of urine, and this in turn can interfere with the development of both the kidneys and the lungs.
Amniotic fluid is crucial in the development of the fetal lungs. If there is not enough amniotic fluid, the lungs of the fetus do not grow. As a result, fetal urinary tract obstruction can produce pulmonary hypoplasia and renal dysplasia . A low amniotic fluid level, or no amniotic fluid, can signal a blockage at some point in the urinary tract to the flow of urine.
Postnatal Care For Babies Born With Luto
After your baby is born, pediatric urologists and nephrologists coordinate imaging studies of kidney and bladder function. After thorough evaluation of your child’s condition, the team will counsel your family on what to expect in both the short- and long-term.
Postnatal treatment options depend on the type of obstruction. For posterior urethral valves , endoscopic resection is a minimally invasive technique performed within the first several days of life, along with the removal of the shunt. Urologic surgeons attach specialized surgical instruments to a tool with a light and camera and insert it into the urethra to remove the tissue causing the obstruction.
In more complicated cases, a vesicostomy an opening below the belly button that allows the bladder to drain directly into a diaper diverts urine until the baby is healthy enough to undergo valve resection or urethral reconstruction. Learn more about how PUVs are treated by CHOP’s pediatric urologists.
Support Groups & Other Resources
- The Fetal Hope Foundation provides support and information to parents, funds research, and increases awareness about TTTS, TRAP Sequence, Amniotic Band Syndrome, and Urinary Tract Obstruction
- Researchers, volunteers, educators, outreach workers and advocates working together to give all babies a fighting chance
- Birth Defect Research for Children a parent networking service that connects families who have children with the same birth defects
- Kids Health doctor-approved health information about children from before birth through adolescence
- CDC – Birth Defects Dept. of Health & Human Services, Centers for Disease Control and Prevention
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Antenatal Diagnosis And Assessment
The detection of fetal LUTO using ultrasound has good accuracy,9,10 partly because the anomalies of the renal tract and kidneys are also associated with secondary findings, such as oligohydramnios. Assessment of the fetal genitourinary tract forms a part of all routine screening ultrasound examinations , and when abnormalities are detected this should lead to a detailed assessment focusing on amniotic fluid volume, renal size, parenchyma, collecting system and bladder size. Ultrasonography may help in the differentiation of obstructive and nonobstructive causes of megacystis, with the association of increased echogenicity and oligohydramnios in the presence of bladder distension being predictive of an obstructive aetiology in about 87% of cases10 .). However, it is of limited value differentiating PUV from other causes of LUTO.10,11
Figure 1An ultrasound image of a fetal bladder in a baby with lower urinary tract obstruction. The dilatation of the proximal urethra gives the bladder a classic keyhole appearance.
Assessing fetal renal function in early pregnancy has been used to select fetuses before performing in utero treatment. There are many published studies evaluating the use of fetal urine metabolites in evaluating fetal renal function, but no consensus seems to have been reached as to the overall efficacy.