AUA2021 Reflections: Panel Discussion on Transitional Care in the Spina Bifida Patient

By: Konrad M. Szymanski, MD, MPH; John Stoffel, MD; Stephanie Kielb, MD; Rose Khavari, MD; Hadley Wood, MD | Posted on: 01 Nov 2021

Advances in medical and surgical therapy allow most children with spina bifida (SB) to survive to adulthood. Lifelong urological care is required, but about 60% of patients do not establish adult care.1 About 80% of pediatric urologists recommend that patients with prior complex surgical reconstruction be followed by urologists with specific interest, training and experience in transitional urology. For the Plenary Session, we presented a case-based discussion of a 26-year-old female with SB and a history of multiple lower urinary tract reconstructions in childhood presenting to reestablish urological care, having been discharged from her pediatric SB clinic 8 years prior. We aimed to focus on scenarios every urologist might encounter, emphasizing the knowledge gap in our typical discussions (obstetrics), drawing on experience and (when available) evidence.

Baseline Evaluation and Surveillance

Adults with SB utilize ambulatory care less than children.2 Monitoring should focus on safety (upper tracts, infections, soft tissue protection) and quality of life-related issues (satisfaction with bladder management, time and resources), aiming for patients’ health and happiness. Symptoms and quality of life should be tracked, preferably with validated instruments (Neurogenic Bladder Symptom Score3 and QUAlity of Life Assessment in Spina bifida for Adults,4 respectively).

Testing recommendations include annual renal/bladder ultrasound, serum creatine and, after urinary reconstruction, B12 levels. Cystoscopy and upper tract imaging after bladder augmentation are recommended after clinical changes in upper or lower urinary tract status, gross hematuria, recurrent symptomatic urinary tract infections (UTIs), increasing incontinence or pelvic pain.5

Prenatal and Sexual Counseling

There is a lack of sexual counseling in SB patients. Only 38% of male and 54% of female SB patients receive SB-specific sexuality counseling from physicians. About 95% have inadequate knowledge about SB-specific sexual health. Only 39% discuss sexuality issues with a doctor. Among SB patients and parents who have not spoken with physicians, virtually everyone would have discussed sexuality issues if their physicians had initiated the conversation. SB patients are interested in sex and fertility, with the same desires for relationships and fertility as their healthy counterparts.6 In 1 study, 29% of women had unwanted sexual contact.6 Disability, particularly cognitive, is a risk factor for sexual abuse.

Fertility in females with SB is equivalent to the general population.7 Development of neural tube defects is linked to folic acid metabolism. Risk of transmission to offspring is 1%–8% where 1 parent has SB (male or female), increasing to 15% if both are affected. As neural tube defect occurs in the first 3–4 weeks of gestation, supplementation with folic acid at a high dose of 4–5 mg/day (compared to 0.4 mg/day) is recommended by the Centers for Disease Control and Prevention and the American College of Obstetricians and Gynecologists, beginning a minimum of 3 months before conception through 12 weeks of gestation. Folic acid should be increased alone, not increasing the dosage of prenatal vitamins. Accurate renal function evaluation should be undertaken and risks of pregnancy discussed (worsening renal insufficiency, hypertension, preterm labor, growth retardation).8

Role of General Urologist during Pregnancy in SB

Women with SB have higher rates of complications in pregnancy, especially after lower urinary tract reconstruction, including upper tract changes, catheterization difficulties, new incontinence (17%–39%) and UTIs, with up to 46% needing a nephrostomy tube.9 Close urological monitoring during and after pregnancy is needed. Between 7% and 100% of women experience problems catheterizing bowel and bladder channels.9,10

Women with SB have a higher risk of symptomatic UTIs/pyelonephritis. Pyelonephritis can lead to preterm labor, fetal wasting and low birth weight. To reduce UTI/pyelonephritis risks, some advocate antibiotic prophylaxis, whereas others culture the urine routinely and treat cystitis or asymptomatic bacteriuria to avoid antibiotic resistance.

There is a concern for generating appropriate Valsalva with uterine contractions in SB, prolonging labor, leading to urinary fistulas or emergent cesarean delivery (CD). There are few data available for successful vaginal delivery in this group. If a woman with low lumbar, ambulatory SB with reconstruction desires a vaginal birth after careful discussion with the obstetrician and assessment of pelvic strength, scheduled vaginal delivery should be considered, with the urology team being ready for potential CD.

Teamwork and pre-delivery planning are critical.9 CD may be challenging given previous reconstruction. A urologist familiar with reconstruction needs to be present and to participate. Despite an expert urologist being present in all CD deliveries in 1 series, 40.5% of patients had complications.9

(Emergency) Cesarean Section

The goal of delivery is a healthy baby and a healthy mother. Approximately 95% of safe delivery management begins before the scheduled CD. This includes communication with the maternal-fetal medicine team, decision about delivery environment (labor and delivery vs operating room), instrument and disposable availability (catheters, wires, suture), personnel who will be present (urology technician/circulator), appropriate pre-delivery imaging if needed and a discussion about approach (abdominal incision, uterine incision).

Delivery considerations include maternal position (monitors may alter exposure), urine culture, antibiotic management, anesthetic (epidural may not be high enough for long midline if needed), the fact that the mesentery often pushes to the side with particular care required if mesenteries involved are bilateral, Mitrofanoff management (often redundant due to uterine enlargement), suprapubic tube management (if placed during pregnancy), drains and particular care with ureters (especially if previously replanted). It is important to check for enterotomies and run the bowel prior to closure.

In conclusion, adults with SB need yearly, scheduled evaluations to monitor for renal/bladder safety. New symptoms (like recurrent UTIs or hematuria) should prompt more thorough and frequent evaluations. Adolescents and adults are sexually active and express desire for children at the same rate as the general population, but many have little to no education in this area. Folic acid supplementation (4 mg/day) is recommended. Higher rates of complications during pregnancy and delivery in women with SB demand regular involvement of a urologist from very early pregnancy to well after delivery. Antibiotic prophylaxis or vigilant screening for UTIs/asymptomatic bacteriuria should be considered. Given the higher rate of premature labor, an elective/scheduled CD should also be considered, and a high-risk obstetrician and urologist with experience in reconstruction should be available. Healthy delivery starts before the delivery date, with emphasis on the importance of communication as well as protecting previously reconstructed anatomy.

Disclosures: Konrad Szymanski: research grants (NIH-NIDDK-K23 DK113227, NIH-NIDDK-R21NS111383), Rose Khavari: research grants (NIH-NIDDK- R03DK126994, NIH-NIDDK- K23DK118209), Hadley Wood: site principal investigator (Boston Scientific).

  1. Szymanski KM, Cain MP, Hardacker TJ et al: How successful is the transition to adult urology care in spina bifida? A single center 7-year experience. J Pediatr Urol 2017; 13: 40e1.
  2. Shepard CL, Doerge EJ, Eickmeyer AB et al: Ambulatory care use among patients with spina bifida: change in care from childhood to adulthood. J Urol 2018; 199: 1050.
  3. Welk B, Morrow S, Madarasz W et al: The validity and reliability of the neurogenic bladder symptom score. J Urol 2014; 192: 452.
  4. Szymanski KM, Misseri R, Whittam B et al: QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A): development and international validation of a novel health-related quality of life instrument. Qual Life Res 2015; 24: 2355.
  5. Joseph DB, Baum MA, Tanaka ST et al: Urologic guidelines for the care and management of people with spina bifida. J Pediatr Rehabil Med 2020; 13: 479.
  6. Verhoef M, Barf HA, Vroege JA et al: Sex education, relationships, and sexuality in young adults with spina bifida. Arch Phys Med Rehabil 2005; 86: 979.
  7. Jackson AB and Mott PK: Reproductive health care for women with spina bifida. ScientificWorldJournal 2007; 7: 1875.
  8. Jones DC and Hayslett JP: Outcome of pregnancy in women with moderate or severe renal insufficiency. N Engl J Med 1996; 335: 226.
  9. Roth JD, Casey JT, Whittam BM et al: Complications and outcomes of pregnancy and cesarean delivery in women with neuropathic bladder and lower urinary tract reconstruction. Urology 2018; 114: 236.
  10. Greenwell TJ, Venn SN, Creighton S et al: Pregnancy after lower urinary tract reconstruction for congenital abnormalities. BJU Int 2003; 92: 773.
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