We investigated vitamin B12 deficiency following ileocystoplasty in children.
Patients who underwent ileocystoplasty between December 1993 and September 2006 were included and B12 levels were retrospectively analysed. Patients with a serum B12 of less than 150 pg/ml were considered deficient. The distance of the ileal segment from the ileocaecal valve was recorded.
There were 105 patients in the series; 61 were male. Mean age at surgery was 7.7 years (SD = 3.9). The mean interval from surgery to most recent B12 level was 50 months (SD = 30). None of the patients were on B12 supplementation. Two patients were B12 deficient, both more than 7 years after surgery; 44% of patients with levels available 7 years after surgery had a B12 below 300 pg/ml. There was a significant negative correlation between B12 level and length of follow up (Spearman's rank, P < 0.01). Twenty patients with an ileal segment sparing 60 cm from the ileocaecal valve had a higher mean B12 (524 vs 419, SEM 60 vs 28). This was not statistically significant.
We demonstrate a reduction in serum B12 level with time following ileocystoplasty. These patients should have their B12 levels measured in the long term.
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... The vitamin B 12 intrinsic factor complex binds to the vitamin B 12 receptor protein within the ileal epithelial cells. 56,57 Vitamin B 12 deficiency has been reported to occur after any urological reconstruction where a segment of ileum has been used; that is, ileal augmentations, ileal neobladders, ileocecal reservoirs and ileal conduits. 56 Classically, low serum vitamin B 12 levels will begin to appear 3-5 years post-surgical reconstruction; however, because of the high levels of vitamin B 12 stored in the liver and skeletal muscles, it might take up to 15 years to manifest. ...
... 56 Classically, low serum vitamin B 12 levels will begin to appear 3-5 years post-surgical reconstruction; however, because of the high levels of vitamin B 12 stored in the liver and skeletal muscles, it might take up to 15 years to manifest. 56,57 In our experience, we have found that 8% (12/153) of patients where the ileum or the ileal-cecal junction was used for urological reconstruction will develop low vitamin B 12 levels by the 15th post-augment year. Approximately three-quarters (9/12) of our patients are able to correct the defect by increasing the ingestion of oral vitamin B 12 or multivitamins. ...
... If not, parenteral treatment will become necessary. 56,57 It is noteworthy that failure to identify or correct this vitamin deficiency might be associated with peripheral neuropathy, dementia, accelerated atherosclerosis as a result of elevated levels of homocysteine and megaloblastic anemia. 58,59 Small bowel obstruction ...
The present review provides clinical insights and makes recommendations regarding patient management garnered by the long-term follow up of patients undergoing enteric bladder augmentation for the management of congenital anomalies. A prospectively maintained database on 385 patients that have experienced an enteric bladder augmentation, using either the ileum or colon, was reviewed. Evaluations included methods used to prevent bladder calculi formation and recurrence, the incidence and etiology of renal calculi development, the incidence and treatment of vitamin B12 deficiency, and the complications and need for surgical revisions for continent catheterizable stomas. A significantly increased risk for continent catheterizable stomal complications occurred after Monti-Yang tube formation, 70% (21/30 patients), compared with appendicovesicostomy, 41% (27/66 patients), P = 0.008. Both procedures had significantly more complications than continent catheterizable stomas using tapered ileum with a reinforced ileal-cecal valve, 21% (13/63 patients), P < 0.0001 and P < 0.013, respectively. Approximately 50% of the patient population developed a body mass index ≥30 during adulthood. The onset of obesity resulted in significantly more complications developing in patients with a Monti-Yang tube (87%; 13/15 patients) or appendicovesicostomy (55%; 18/33 patients) compared with a tapered ileum with a reinforced ileal-cecal valve (27%, 8/30 patients), P < 0.00015 and P < 0.025, respectively, with a median follow-up interval of 16 years, range 10-25 years. Long-term follow-up evaluations on patients undergoing an enteric bladder augmentation are necessary to prevent the long-term sequela of this procedure. The key to improving patient prognosis is the nutritional management of the patient as they mature, especially if a continent abdominal stoma is going to be carried out.
... Several clinicians employ annual B12 evaluation starting approximately 5 years postoperatively [8••, 33,34]. Due to the rising risk of B12 deficiency, long-term follow-up and prompt replacement are required [35,36]. Replacement therapies can be oral or parenteral and require lifelong follow-up and repletion. ...
Purpose of Review
Bladder augmentation and associated reconstructive procedures are a surgical option for patients with urologic congenital anomalies and neuropathic bladder with the goal of improving continence and reducing the risk of upper tract deterioration. Despite these benefits, augmentation also portends several short- and long-term potential complications. The goal of this chapter is to discuss these complications and review the current literature on this topic.
Metabolic abnormalities, including acid-base disorders and their sequelae, occur in 5–15% of patients with bladder augmentation and are typically seen within 5–10 years from intervention. Chronic bacteriuria and recurrent urinary tract infections often play a role in renal dysfunction and contribute to 70% of urinary tract stone disease. Bladder perforation is reported at rates between 3 and 13%, with associated mortality rates ranging from 1% to as high as 25%. Other more common surgical complications include catheterizable channel issues: stricture, false passage, stenosis, stomal prolapse, channel redundancy, and channel incontinence. Channel complications should be thought of as suprafascial vs. subfascial in order to help guide treatment options. Following augmentation cystoplasty, incidence of bladder cancer ranges from 0 to 5.5%; unfortunately, the vast majority present with locally advanced or node-positive disease. Therefore, yearly screening with cytology and cystoscopy is not recommended and symptom-based screening protocols have been proposed.
Given the complexity and wide variety of complications possible after bladder augmentation, it is critical that these patients are closely followed into adulthood by a urologist, but also receive tailored multi-disciplinary care based on their specific medical and surgical needs.
... It has been reported that up to 44% of patients submitted to bladder augmentation with ileal tissue will require B12 replacement. Oral B12 treatment has not been shown to be an effective replacement therapy [20,21]. ...
Many of the common congenital pathologies surgically treated early in childhood may have long-term clinical and surgical complications when they come to adulthood. Surgically treated obstructive congenital pathologies will need follow-up for the resolution of obstruction and improvement of the upper tract. Of the children treated for vesicoureteric reflux, especially those with bilateral high grade, 5-20% may have a risk of hypertension and renal failure in the long term. Although bladder augmentation with an ileal segment in children is a procedure that can provide good results in terms of preservation of the upper urinary tract and renal function, and achieving continence, there are many long-term clinical and surgical complications. Many of the reconstructive procedures employed on the genitalia may have functional and cosmetic problems, which may require additional surgery in the long term. The rate of additional surgery is as high as 25% within 2-5 yr of follow-up after hypospadias surgery. Therefore, there is an obvious need of long-term follow-up for these children and urologists with expertise to recognize these complications and treat them. Many of the common congenital pathologies surgically treated early in childhood may have long-term clinical and surgical complications when they come to adulthood. Therefore, there is an obvious need of long-term follow-up for these children and urologists with expertise to recognize these complications and treat them.
To describe a surgical alternative option in select patients with neurogenic bladder and a history of Malone antegrade continence enema (MACE) who now require revision augmentation of the bladder, and/or creation of a new continent catherizable urinary channel (CCC).
Herein, we describe a novel surgical approach for patients who have had prior MACE creation who subsequently require surgical revision and creation of a new CCC. Rather than the traditional approach of creating a new CCC utilizing bowel, we perform a cecocystoplasty and leave the previously created MACE intact. The prior MACE channel becomes repurposed as the new Mitrofanoff, which we have termed the MACEtrofanoff channel. Concomitant cecostomy tube placement for bowel management can be performed at the time of surgery.
We have successfully performed this procedure in two patients with good outcomes to date. This technique does not require the sacrifice of the prior appendix channel, nor require the formation of a new stoma and channel. Patients are able to adapt easily to clean intermittent catheterization through a channel they are already familiar with for their prior antegrade enemas.
In select patients, surgeons should consider the MACEtrofanoff procedure to avoid the added morbidity of further bowel mobilization.
Following spinal cord injury, many patients can experience neurogenic lower urinary tract dysfunction (nLUTD), which can result in a poorly compliant bladder and/or unpredictable urinary emptying. When medical and intravesical options fail to manage nLUTD, augmentation cystoplasty can be considered to create a urinary reservoir that can store larger volume of urine at low pressures. Continent catheterizable channels can be considered to facilitate ease of catheterization to drain the bladder. Multiple options exist in managing those with intrinsic sphincteric deficiency including bulking agents, bladder neck sling, bulbar urethral sling, artificial urinary sphincter (AUS), and bladder neck closure (BNC). In certain individuals, urinary diversion is a desired treatment option to manage nLUTD. This chapter will explore these various surgeries and their indications, contraindications, surgical approaches and techniques, outcomes, and complications.
The cutting age application of robotic techniques is arguably intracorporeal pediatric bladder augmentation. While high volume centers are beginning to gain experience in intracorporeal bladder surgery in adults for oncologic applications, pediatric surgeons must apply these techniques in extremely small working spaces and in patients with complex anatomy or surgical history. Long-term results have yet to be reported, but with 5–8 year follow-up, the technique appears to have similar results to traditional open techniques. Larger, multi-center studies are required to confirm safety and effectiveness prior to widespread adoption of this complex technique. Bladder augmentation is often performed with catheterizable channels, bladder neck reconstructions and antegrade colonic enema surgery; as such a brief discussion of these topics is also pertinent to this discussion.
Chronic allograft dysfunction is a significant clinical problem and strategies to reduce or prevent it would have significant health implications. Chronic allograft dysfunction is the cumulative burden of injury from several stresses affecting the allograft throughout its life. Donor factors, implantation stresses and post-transplant factors are important. Post-transplant factors have received much attention and it is becoming clear that antibody mediated rejection is an important cause of late allograft loss, followed by polyoma virus nephropathy and de novo and recurrent glomerular disease. Antibody mediated rejection can follow on from acute cellular rejection although most cases of T-cell mediated rejection respond well to treatment, and whilst there may be some damage, this should not progress. Antibody mediated rejection due to donor specific antibodies is becoming clearly linked to non-adherence or under immunosuppression. Microcirculatory inflammation seems to be the best predictor of antibody mediated rejection; C4d staining, whilst specific is not a sensitive marker of antibody mediated rejection. By the time of failure most allografts have evidence of donor specific antibodies. In contrast, late allograft dysfunction associated with T cell mediated rejection, calcineurin inhibitor toxicity or chronic fibrosis on biopsy are much less likely to be associated with late allograft loss. Molecular and microarray studies allow a greater understanding of the processes involved.
The Spina Bifida Association reports that 7 of every 10,000 children born in the United States have myelomeningocele (MMC) and that there are more than 166,000 people currently living with MMC . Today, survival into adulthood is reported as high 85 % (at age 34 years, 94 % of those without shunts, and 75 % of those with shunted hydrocephalus are alive) . With decreases in birth prevalence of MMC after the introduction of prenatal diagnosis and mandatory folic acid fortification of grain products in the United States, there may be more adults currently living with MMC in the United States than children [23, 32, 59, 107, 136, 140, 161]. Despite a large number of people living with MMC, there are no randomized controlled trials regarding urologic care in this group, and there is a paucity of literature describing long-term urologic outcomes in adults with MMC .
The physiological and biochemical events which occur in the child undergoing surgical interventions are multifaceted, inter-relational and complex. Many serve to facilitate the recovery and survival of the child undergoing a surgical stress. However, when unchecked, some can result in patient morbidity and increased mortality. The advent of minimally-invasive surgical practice has further added to the interest that such techniques may blunt the potential adverse response in the child to traditional surgical stresses. Much scientific endeavor has been spent in investigating these, as yet not fully understood, responses in the human child. This chapter explores the knowledge available concerning the child's responses to the trauma of surgery and attempts to provide practical insight into the management of these physiological challenges for the clinician.
Among 141 consecutive patients with neuro-psychiatric abnormalities due to cobalamin deficiency, we found that 40 (28 percent) had no anemia or macrocytosis. The hematocrit was normal in 34, the mean cell volume was normal in 25, and both tests were normal in 19. Characteristic features in such patients included paresthesia, sensory loss, ataxia, dementia, and psychiatric disorders; longstanding neurologic symptoms without anemia; normal white-cell and platelet counts and serum bilirubin and lactate dehydrogenase levels; and markedly elevated serum concentrations of methylmalonic acid and total homocysteine. Serum cobalamin levels were above 150 pmol per liter (200 pg per milliliter) in 2 patients, between 75 and 150 pmol per liter (100 and 200 pg per milliliter) in 16, and below 75 pmol per liter (100 pg per milliliter) in only 22. Except for one patient who died during the first week of treatment, every patient in this group benefited from cobalamin therapy. Responses included improvement in neuropsychiatric abnormalities (39 of 39), improvement (often within the normal range) in one or more hematologic findings (36 of 39), and a decrease of more than 50 percent in levels of serum methylmalonic acid, total homocysteine, or both (31 of 31). We conclude that neuropsychiatric disorders due to cobalamin deficiency occur commonly in the absence of anemia or an elevated mean cell volume and that measurements of serum methylmalonic acid and total homocysteine both before and after treatment are useful in the diagnosis of these patients.
We summarize important metabolic consequences and long-term complications associated with enterocystoplasty with particular emphasis on the pediatric patient with genitourinary abnormalities.
Materials and methods:
A directed MEDLINE literature review for metabolic and long-term complications following enterocystoplasty was performed. Information gained through the published literature and from our database was reviewed and summarized to provide the reader with a thorough review of the subject.
Bowel is not a perfect tissue for substitution or augmentation and its use to treat functionally and structurally compromised bladders is associated with several metabolic consequences and long-term complications. Metabolic acidosis is the most common metabolic abnormality seen. The rates and severity of these complications vary, although they may have a profound impact on patient quality of life after enterocystoplasty.
The metabolic consequences and long-term complications associated with enterocystoplasty are important clinical features of this intervention. Careful consideration should be given to them prior to pursuing enterocystoplasty.
We previously identified vitamin B(12) deficiency as a potential long-term consequence in pediatric patients with prior ileocystoplasty despite adequate preservation of terminal ileum. Vitamin B(12) deficiency can result in hematological and neurological deficits, of which some are irreversible. Deficiency discovered after ileocystoplasty is purportedly due to B(12) malabsorption since the principal absorption site is ileum. B(12) deficiency due to malabsorption is typically treated with intramuscular injection to ensure adequate treatment. We determined whether oral vitamin B(12) supplementation could increase serum vitamin B(12) in patients with deficiency who underwent ileocystoplasty.
During followup after ileocystoplasty we identified patients with low (200 pg/dl or less) or low normal (200 to 300 pg/dl) vitamin B(12). Oral vitamin B(12) was begun at 250 μg. Serum B(12) was assessed at 1, 2 and 3-month intervals after beginning therapy.
A total of 128 patients with a mean followup of 83 months after ileocystoplasty had vitamin B(12) levels available for review. Of these patients 36 (28%) had a level of 300 pg/dl or less with a level of 200 pg/dl or less in 16 (13%). After oral vitamin B(12) treatment serum levels increased from a mean 235 to 506 pg/dl (114%) upon initial measurement (p <0.001). Subsequent measurements continued to increase from the first posttreatment level (p <0.05). No adverse effects were noted during a mean 4-month followup.
To our knowledge this is the first study to show that oral vitamin B(12) effectively increases serum levels in pediatric patients with prior ileocystoplasty.
Vitamin B12 is absorbed exclusively in the terminal ileum the resection of which may produce malabsorption of B12. The present study aimed to determine whether the length or specific segment of the intestine used in bladder reconstruction affects the overall incidence of B12 malabsorption. It was also aimed at the reasonable way of following these patients.
Seventy patients who underwent urinary reconstruction between 1988 and 1997 were studied: 28 had undergone orthotopic reconstruction, 14 had ileal conduit diversion, 8 had continent diversion and 20 had undergone enterocystoplasty. The median follow up was 65 months (10-137 months). Indications for operation included carcinoma of the bladder, neurogenic bladder dysfunction, idiopathic detrusor instability and interstitial cystitis. Schilling tests were performed, and serum B12 level and haematological indices were measured.
Patients were classified depending on whether the ileocaecal junction was used. Group 1 included ileal conduit and entero-cystoplasty where 15-20 cm of ileum, 15-20 cm from ileocaecal junction was used. Group 2 consisted of orthotopic reconstruction and continent diversions where the ileocaecal segment was used. Group 3 comprised patients in whom long ileal segments (50-60 cm) had been used for reconstruction. Five patients in group 1 and one in group 2 had low B12 levels, but none had developed neuropathy or megaloblastic anaemia. One patient in group 1, and six patients in group 2 had low Schilling tests indicating intestinal malabsorption of B12. No patient in group 3 had a low B12 or an abnormal Schilling test.
This study showed that use of ileocaecal segments results in intestinal malabsorption of B12. The length of ileum alone does not seem to be the determinant factor in causing B12 deficiency or B12 malabsorption.
The incidence of stones in patients with enterocystoplasty is reported as 12-52.5%. Most patients will have multiple physical factors such as immobility, need for self catheterisation and poor urine drainage, so that it is not certain that an intestinal reservoir is the cause of stones on its own. There is little or no evidence that mucus is an aetiological factor. Foreign bodies in the reservoir, such as staples, increase the risk of stone formation from 13% to 43%. Stones require surgical removal. Minimally invasive techniques may be used for small stones. A low velocity disintegrator is required so that fragments are not propelled into the intestinal mucosa. Stones are infective in origin in 86% of cases, but 14% are sterile. Metabolic screen shows that 80% of patients have risk factors for at least three different types of stone. All patients have raised pH (mean 6.93) and hypocitraturia. Raised serum and urinary calcium, hyperoxaluria and hyperuricosuria are found in up to 33% of patients.
The interposition of bowel in continuity with the urinary tract has allowed for the preservation of renal function and continence in children with bladder exstrophy, as well as neurogenic and valve bladders. Although bladder augmentation with ileum or colon has been shown to be safe, the long-term effects of metabolic acidosis in addition to abnormalities in linear growth and bone metabolism remain largely unknown. We reviewed the literature to critically examine linear growth in children who have had bladder augmentation with a particular emphasis on the correlation between acid-base status, bone mineralization and growth. The majority of studies suggest that linear growth is not affected by bladder augmentation. In the short-term, children post-augmentation have varying degrees of metabolic acidosis which, overtime, appears to resolve with no affect on linear growth. In a single study, bladder augmentation led to significant bone demineralization almost a decade after surgery, however, even in these children no decrease in linear growth was noted. No alterations in bone density levels were seen with short-term follow-up.
Cancer following augmentation cystoplasty is a recognized risk factor. The procedure has only gained popularity in pediatric urology within the last 25 years, limiting the population being studied by statistical power and the lack of long-term followup. The majority of reported cases of post-augmentation malignancy have occurred in adults with multiple risk factors. Currently the most common indication for augmentation cystoplasty in children and adolescents is neuropathic bladder. We review 3 cases of transitional cell carcinoma (TCC) following augmentation cystoplasty in this unique population with no additional risk factors for bladder cancer.
Materials and methods:
We reviewed our clinical database of children and adolescents who underwent bladder augmentation since 1978 to evaluate the incidence of cancer. This study represents a captured population within a single institutional practice. There were 483 cases entered into the database, and particular attention was paid to 260 augmentations with at least 10 years of followup. We reviewed medical history, clinical outcomes, cancer risk factors, augmentation type and pathology of the 3 patients who presented with TCC after augmentation cystoplasty.
Three patients presented with grade 2 to 3 TCC following bladder augmentation, all of whom underwent exploratory laparotomy and eventually died of metastatic disease. No patient had a history of smoking exposure greater than 10 packs per year or other known risk factors for bladder cancer. Two patients had an ileocecal augmentation and 1 had a cecal augmentation for neuropathic bladder. Patient age at augmentation was 8, 20 and 24 years, and age at diagnosis of TCC was 29, 37 and 44 years, respectively. Mean time from augmentation to TCC was 19 years. Assuming a 10-year lag period before the risk of cancer, in at least 1.2% of bladder augmentation cases in our database cancer has developed.
This study supports the hypothesis that bladder augmentation appears to be an independent risk factor for TCC, with a lag time of less than 20 years. We recommend endoscopic surveillance of all patients with a history of bladder augmentation beginning 10 years after initial surgery.