Is a vesicoamniotic shunt intrinsically bad? Shunting a normal fetal bladder

Introduction

We previously demonstrated that in utero vesicoamniotic shunting of obstructive uropathy in fetal lambs produces a shrunken noncompliant bladder. We hypothesized that the normal fetal bladder filling and emptying cycle in fetal life is critical to the development of normal bladder function.

Materials and Methods

We placed vesicoamniotic shunts in 4 normal fetal lambs at 74 days' gestation. The fetuses were delivered at term (145 days), and bladder volume and compliance were measured and compared with those measurements in 3 normal term fetuses. The lambs were then killed and the renal tracts and bladders removed submitted to histologic examination.

Results

All shunted lambs survived to term. Three normal control lambs were delivered at term. The mean bladder volume in shunted lambs was 4 ± 2.8 mL (n = 4) compared with 60 ± 17 mL (n = 3) in control lambs (P < .05). Bladders in the shunted lambs had very poor compliance compared with normal lambs' bladders. Histologic examination of the shunted bladders showed increased fibrosis and distortion of the muscle layers compared with control bladders.

Conclusion

Even in the absence of obstruction, preventing normal bladder filling and emptying in fetal life produces fibrotic bladders with poor compliance.     

Effects of early vesicostomy in obstructive uropathy on bladder development

Purpose

Creation of a vesico-amniotic shunt for obstructive uropathy removes the normal fetal urination cycle. It is unclear how this affects bladder function at term. The authors measured the bladder volume and reviewed the bladder histology after fetal vesicostomy.

Methods

The authors created an obstructive uropathy in fetal lambs at 60 days’ gestation by ligating the urethra and urachus. Vesicostomy (female) or urethrostomy (male) were performed 21 days after obstruction to release the obstruction. The fetuses were killed at term (145 days).

Results

Thirteen fetuses were shunted. Seven fetuses miscarried after shunting. Six survived, and 3 had a successful shunt with a very small bladder (5 to 7 mL). Two had incomplete shunts that failed some time after shunting. These both had huge bladders (399 mL). In one, the obstruction was unsuccessful. Histologic examination showed that the obstruction caused bladder muscle hypertrophy. Shunted lambs had severe fibrosis of the bladder wall and very poor bladder compliance.

Conclusions

Shunt operations after obstructive uropathy may salvage the kidney but fail to preserve bladder function. The fetus needs a normal urination cycle for normal bladder development. This requirement exists even when the obstruction is successfully bypassed.     

Pressure-limited vesico-amniotic shunt tube for fetal obstructive uropathy

Introduction

In utero shunting (vesico-amniotic shunt) of obstructive uropathy in fetal lambs produces a shrunken, noncompliant bladder. We hypothesized that using a ventriculo-peritoneal shunt for the vesico-amniotic shunt may preserve the filling/emptying cycle and thus normal bladder development.

Materials and Methods

We created obstructive uropathy in 60-day gestation fetal lambs, ligating the urethra and urachus. Vesico-amniotic shunting was performed 21 days later using the valve end of a ventriculo-peritoneal shunt (valve shunt) or silastic tubing (nonvalve shunt). They were delivered at term (145 days), and the bladder volume was measured and compared to normal term fetuses. The lambs were sacrificed, and the kidneys and bladder removed for histology.

Results

Twenty-seven lambs were shunted. Of 14 valve shunts, 8 were effective. Of 13 nonvalve shunts, 11 were effective. The mean bladder volume was 57 ± 41 mL with a valve shunt and 8.8 ± 4.7 mL with a nonvalve shunt (P < .05) (normal term lambs, 65 ± 18 mL, n = 5). Histology of the shunted bladders showed increased fibrosis in the submucosal and muscle layers. This was less obvious in lambs with a valve shunt.

Conclusion

A pressure controlled shunt for fetal obstructive uropathy improves bladder volume but does not prevent bladder wall fibrosis.     

Bladder development following bladder outlet obstruction in fetal lambs: optimal timing of fetal therapy

Background

Lower urinary tract obstruction causes both renal failure and bladder dysfunction after birth. This study examined the early bladder wall changes after creating an obstructive uropathy focusing on bladder wall thickness and muscle integrity.

Methods

We created obstructive uropathy in fetal lambs at 60 days' gestation, ligating the urethra and urachus. The fetuses (n = 28) were delivered at 48 hours and 3, 4, 5, 7, and 14 days after obstruction and at term (145 days' gestation). Sham-operated lambs were used as controls (n = 20). Histology samples were stained using α-smooth muscle actin) immunohistochemistry and also hematoxylin-eosin, Masson trichrome, and colloidal Fe stain.

Results

The bladder wall initially expanded and stretched. By day 4, the bladder wall became thicker. Histologically, the bladder in obstructed lambs demonstrated a prominent submucosal fibrotic change by 7 days. The mean bladder wall thickness at 14 days after obstruction was thicker than controls, and fibrosis was prominent.

Conclusion

The initial changes in the bladder wall were expansion of the muscle component followed by fibrosis. The bladder wall thickness dramatically increased 4 to 7 days after obstruction. We conclude that shunting operations to preserve bladder function may be needed earlier than expected.     

The early effects of urinary tract obstruction on glomerulogenesis

Purpose

Our obstructive uropathy model in fetal lambs showed that renal cystic changes appeared 3 weeks after obstruction. In this study, the authors investigated the changes resulting from complete urinary tract obstruction in the first 7 days after obstruction.

Methods

An obstructive uropathy was created in fetal lambs at 60 days’ gestation by ligating the urethra and urachus. They were delivered 48 hours, 3 days, 5 days, and 7 days later by cesarian section. The kidneys were removed and processed for histologic examination.

Results

Eighteen fetuses were operated on and 15 (4 at 48 hours, 4 at 3 days, 2 at 5 days, and 5 at 7 days; 83%) survived. Macroscopically, bladder dilatation and slightly dilated ureters were identified from 48 hours. Microscopically, dilatation of proximal tubules started from 48 hours after obstruction and increased by 7 days. Glomerular cysts in the nephrogenic zone also were identified from 48 hours. Dysplastic changes were not found.

Conclusions

The first areas in the developing kidney that suffer damage after obstructive uropathy are the proximal tubule and the nephrogenic zone. This change started 48 hours after obstruction. Shunting procedures need to be performed considerably earlier than previously thought.     

Prenatal urinary matrix metalloproteinase profiling as a potential diagnostic tool in fetal obstructive uropathy

Background/Purpose

The diagnostic evaluation, patient stratification, and prenatal counseling for congenital obstructive uropathy remain sub-optimal. Matrix metalloproteinase (MMP) expression profiles are emerging as a valuable diagnostic tool in assorted disease processes. We sought to determine whether congenital obstructive uropathy impacts MMP expression in fetal urine.

Methods

Fetal lambs (n = 25) were divided in two groups: group I (n = 12) underwent a sham operation and group II (n = 13) underwent creation of a complete urinary tract obstruction. Gelatin zymography panels for 4 MMP species were performed on fetal urine in both groups at comparable times post-operatively. Statistical analysis was by the Fisher's exact test (P < .05).

Results

Overall fetal survival was 80% (20/25). A variety of significant differences in MMP expression between the two groups were identified. The following profiles were present only in obstructed animals: any MMP other than MMP-2 (P = .029), including any MMP other than 63 kDa and 65 kDa (P = .009); 2 or more MMPs excluding MMP-2s (0.029); and 3 or more MMPs (P = .029).

Conclusions

Limited matrix metalloproteinase expression is present in the urine of normal ovine fetuses. Fetal obstructive uropathy impacts urinary MMP expression in various distinguishable patterns. Prenatal urinary MMP profiling may become a practical and valuable diagnostic tool in the evaluation of congenital obstructive uropathy.     

Serial amnioinfusions prevent fetal pulmonary hypoplasia in a large animal model of oligohydramnios

Background/Purpose

Severe neonatal pulmonary hypoplasia incurs mortality rates approaching 71% to 95%. We sought to determine the utility of serial amnioinfusions through a subcutaneously implanted intraamniotic catheter to prevent pulmonary hypoplasia in fetal obstructive uropathy.

Methods

Fetal lambs (n = 32) were divided into 3 groups. Group I (n = 12) underwent a sham operation, group II (n = 15) underwent a complete urinary tract obstruction via ligation of the urachus and urethra with a subcutaneous tunneled intraamniotic port-a-cath without amnioinfusions, and group III (n = 5) underwent a creation of a complete urinary tract obstruction with a port-a-cath as described in group II with serial amnioinfusions. Lung tissue was analyzed by lung volume to body weight ratios and stereology. Statistical analysis was performed by analysis of variance and Bonferroni comparisons (P < .05).

Results

Obstructed fetuses grossly had smaller lungs than treated and control animals. Lung volume to body weight ratios were statistically significant between groups. Airspace fractions were comparable between groups I and III (average = 0.53 and 0.55, respectively), although both were significantly greater than group II (average = 0.48) (P = .049).

Conclusions

Serial amnioinfusions through an intraamniotic port-a-cath prevented pulmonary hypoplasia in an ovine model of complete obstructive uropathy. The use of an easily accessible device for amnioinfusions may be a viable option to treat oligohydramnios.     

Can a pressure-limited vesico-amniotic shunt tube preserve normal bladder function?

Introduction

We have previously shown that a vesico-amniotic shunt (V-A shunt) produces fibrotic bladders with poor compliance in normal fetal lambs. We hypothesized that using a ventriculo-peritoneal shunt (V-P shunt) as a V-A shunt in normal bladders may preserve the filling/emptying cycle and normal bladder development.

Materials and methods

The V-A shunting in normal fetal lambs was performed at 74 days of gestation using a V-P shunt (group A) and a free-draining shunt tube (group B). Sham-operated lambs were used as controls (group C). They were all delivered at term (145 days), and the pressure-volume curve, bladder volume, and histologic features of the bladder wall were compared.

Result

The mean bladder volume in group B (n = 5), 5 ± 2.4 mL, was significantly smaller (P < .01) than that in group A (n = 6), 53 ± 14 mL, and group C (n = 10), 57.3 ± 12 mL. The bladder wall thickness in group A was 338 + 94.2 μm; group B, 741 ± 128 μm; and group C, 374 ± 120 μm. Group B bladders had very poor compliance with thick bladder wall (P < .01). Histologically, group B bladders showed prominent submucosal fibrotic change, but group A bladders were similar to controls.

Conclusion

This study shows that a pressure-limited shunt tube for V-A shunting preserves the normal fetal bladder development.     

Correlation between prenatal urinary matrix metalloproteinase activity and the degree of kidney damage in a large animal model of congenital obstructive uropathy

Background/Purpose

We aimed to determine whether the profile of matrix metalloproteinase (MMP) activity in fetal urine correlates with the degree of kidney damage in the setting of congenital obstructive uropathy.

Methods

Fetal lambs underwent either a sham operation or creation of a complete urinary tract obstruction. Necropsies were performed before term, when urinary MMP profiling was performed by zymography; and kidney damage was assessed histologically by multiple semiquantitative analyses and histomorphometric measurements.

Results

There was a significant correlation between inner medullary thickness and MMP-9 (P = .005) and 63-kd MMP-2 (P = .019) activities. In like manner, the only MMPs associated with kidney fibrosis were MMP-9 and 63-kd MMP-2. Matrix metalloproteinase-9 activity was a highly significant independent predictor of the total combined kidney fibrosis score (P < .001) as well as of higher fibrosis grades in each of 6 kidney areas analyzed (all with P < .01). The activity of 63-kd MMP-2 correlated significantly with higher fibrosis in select areas.

Conclusions

In a fetal ovine model, urinary MMP activity correlates with the degree of kidney damage. The presence of MMP-9 (in particular) and that of 63-kd MMP-2 are independent predictors of severity. Prenatal urinary MMP profiling may enhance patient stratification and counseling in the setting of congenital obstructive uropathy.     

Optimal timing of prenatal treatment of obstructive uropathy in the fetal lamb

Purpose: It was still unclear how urinary tract obstruction alters normal nephrogenesis and leads to renal dysplasia. The authors created an obstructive uropathy model in fetal lambs and reviewed the pathology of the obstructed kidney to determine the optimal timing for decompression of the obstruction.Methods: Obstructive uropathy was created in fetal lambs at 60 days’ gestation by ligating the urethra and urachus. They were delivered 20 to 31 days later by cesarian section. The kidneys were processed for histologic examination.Results: Thirty-four 60-day lambs were operated on. Dysplastic changes were noted in 25 fetuses, and 24 fetuses had cysts in the nephrogenic zone. The cystic components in multicystic dysplastic kidneys (MCDK) are mainly in the proximal tubules.Conclusions: In utero urinary tract obstruction causes reduction of numbers of functioning nephrons and produces cysts in the nephrogenic zone and in the deeper cortex. These cysts and dilated proximal tubules suppress new nephron formation. Twenty days after obstruction, there were early features of dysplasia, but the nephrogenic zones still were present. Early shunting may salvage renal function.     

Prenatal ultrasonographic and magnetic resonance imaging findings of congenital cloacal anomalies associated with meconium peritonitis

Background/Purpose

Cloacal malformations are rare anomalies, occurring in females, and in which they demonstrate a single perineal orifice for urethra, vagina, and rectum. Prenatal ultrasonograms (US) of cloacal malformations sometimes show ascites, hydrocolpos, and hydronephrosis. We herein describe the characteristic prenatal US and magnetic resonance imaging (MRI) findings of the cloacal malformations associated with meconium peritonitis.

Methods

The pre- and postnatal records of 11 newborn patients with cloacal malformations, treated in our hospital from 1988 to 2004, were reviewed. All fetuses underwent prenatal US by experienced obstetricians, whereas in addition, fetal MRI was performed in 1 patient.

Results

The prenatal US and/or MRI findings showed fetal ascites, a multicystic pelvic mass, oligohydramnios, and bilateral hydronephrosis in 5 of 11 patients with cloacal malformations. In these 5 cases, postnatal examinations showed associated hydrocolpos, hydrometrocolpos, and bilateral hydronephrosis; furthermore, 4 of these 5 cases also showed meconium peritonitis at laparotomy.

Conclusions

The prenatal US and MRI findings, showing fetal ascites, multicystic pelvic mass, bilateral hydronephrosis, and oligohydraminios are highly suggestive of the cloacal malformations associated with meconium peritonitis.     

Segmental multicystic dysplastic kidney in children: clinical presentation, imaging finding, management, and outcome

Purpose

The aim of the study was to assess the presentation, imaging findings, management, and outcome of segmental multicystic dysplastic kidney (MCDK) in children.

Materials and Methods

Six patients with segmental MCDK were diagnosed and observed at our hospital. We reviewed the medical records to collect data on initial symptoms, results of imaging studies, associated urinary tract anomalies, operation, and outcome.

Results

Three patients had abnormal prenatal ultrasound and were diagnosed by further postnatal imaging; they were asymptomatic after birth. The other 3 children presented with a renal mass, recurrent urinary tract infection, or urinary incontinence. Five patients had associated ipsilateral or contralateral urinary tract abnormalities, including vesicoureteral reflux, ureterocele, duplex collecting system, ureteropelvic junction stenosis, and ectopic ureter. Lower tract reconstruction was performed in 3, and open biopsy was done in 1 child. None underwent partial or total nephrectomy, and all had involution of the cysts for a mean of 40 months.

Conclusions

Most of the cases occur in the upper pole of a duplex kidney and often involute spontaneously without significant complication. The clinical presentation and imaging findings depend on the associated anomalies and complications. Although the latter may require surgery to resolve, segmental MCDK itself rarely requires resection.     

Persistent ascites can be effectively treated by peritoneovenous shunts

Aim

The aim the study was to review our experience and determine the effectiveness of peritoneovenous shunts in the management of persistent ascites.

Methods

A retrospective review of all patients who had a peritoneovenous shunt inserted for persistent ascites in our institution over 32 years (1977-2010) was performed.

Results

Thirty-one peritoneovenous shunts were inserted into 25 patients. Records from 22 were available. Median age was 34 months (range, 1 month-12 years), with median duration of ascites of 1.75 months (range, 3 weeks-2.5 years). Sixteen had previous abdominal surgery, whereas one had previous thoracic surgery. Other underlying pathologies included hepatitis (n = 2), lymphohistiocytosis, lymphatic hypoplasia, and carcinomatosis. One was regarded as idiopathic. Previous management consisted of paracentesis (n = 15), dietary modification (n = 11), diuretics (n = 9), and total parenteral nutrition (TPN) (n = 4). One underwent fetal drainage of ascites.No intraoperative complications occurred. Eight (36%) had postoperative complications, including shunt occlusion (n = 2), pulmonary edema (n = 2), infection (n = 2), and wound leakage (n = 1). One developed a varix following shunt removal. The ascites resolved after shunting in 20 (91%) of the 22 children. Four died from their underlying pathology. In children with malignancy, the shunt allowed prompt resumption in treatment schedule.

Conclusion

This represents the largest series of children receiving peritoneovenous shunt for persistent ascites. It is a safe and effective treatment which should be considered early.     

Associated urologic anomalies in children with solitary kidney

Purpose

The aim of this study was to investigate the incidence, nature, surgical treatment, and outcome of associated urologic anomalies (AUA) in children with anatomic or functional solitary kidney (SK).

Methods

In this study, SK was defined as the kidney that is present in cases of unilateral renal agenesis (RA), or the kidney that is contralateral to a kidney that is nonfunctional secondary to either noncystic dysplastic kidney disease (NCDK), or multicystic dysplastic kidney disease (MCDK). Fifty-seven consecutive patients with SK (17 owing to RA, 10 owing to NCDK, 30 owing to MCDK) were reviewed.

Results

A total of 23 of 57 (40%) had AUA in the SK (11 of 17 [65%] of RA cases; 7 of 10 [70%] of NCDK cases; and 5 of 30 [17%] of MCDK cases). The incidence of AUA in MCDK was significantly lower than that in RA or NCDK (p < .01: Mann-Whitney U test). Surgery was performed on 20 of 23 (87%) for vesicoureteric reflux (VUR) in 13, pelviureteric junction stenosis in 3, and ureterovesical junction stenosis in 4. Surgery was not performed in 3 of 16 with VUR because the VUR that was present was of low grade. AUA-related symptoms such as urinary tract infection and abdominal mass were seen in 15 of 23; the remaining 8 were asymptomatic. Mean age at surgery was 4.8 years. There were no operative or postoperative complications, and all cases are well without impaired renal function or hypertension after a mean follow-up period of 5.3 years.

Conclusions

In patients with SK, 40% had AUA in the renal collecting system of the SK. All children with SK should undergo a screening voiding cystourethrography (VCUG) even in the absence of hydronephrosis or UTI, and early recognition and treatment are imperative to decrease the long-term risk for renal damage.     

Lung function in lambs with diaphragmatic hernia after reversible fetal tracheal occlusion

Background/Purpose

Short-duration resuscitation (≤4 hours) of lambs with diaphragmatic hernia treated in utero with tracheal occlusion have shown improved lung function compared with untreated diaphragmatic hernia. This may be a transient phenomenon in the treated diaphragmatic hernia lambs because of surfactant deficiency. Our objective was to analyze the effect of fetal tracheal occlusion with or without release of the occlusion 1 week before delivery on pulmonary function during a longer period of resuscitation (8 hours) in the diaphragmatic hernia lamb model.

Methods

Four groups were compared: diaphragmatic hernia (n = 5), diaphragmatic hernia and tracheal occlusion until delivery (n = 5), diaphragmatic hernia and tracheal occlusion with release of the occlusion 1 week before delivery (n = 5), and normal controls (n = 4).

Results

Despite persistently decreased surfactant levels, diaphragmatic hernia lambs treated with tracheal occlusion had normal-sized lungs with marked improvement in lung function and gas exchange over 8 hours when compared with untreated lambs with diaphragmatic hernia. Release of the tracheal occlusion 1 week before delivery added no benefit.

Conclusions

It appears that surfactant-independent mechanisms such as pulmonary growth and structural changes are of foremost importance in relating to improved compliance, oxygenation, and ventilation of diaphragmatic hernia lambs treated with tracheal occlusion.     

The effectiveness of ureteric reimplantation during bladder augmentation for high-grade vesicoureteric reflux in patients with neurogenic bladder: long-term outcome

Purpose

The outcome of ureteric reimplantation (UR) during bladder augmentation (BA) for high-grade vesicoureteric reflux (HVUR) in patients with neurogenic bladder was assessed to determine the effectiveness of UR. A literature review of HVUR and BA was also performed.

Methods

Twenty-two consecutive sigmoidocolocystoplasty patients were reviewed retrospectively; 10 had unilateral HVUR only, 3 had bilateral HVUR, and 9 had unilateral HVUR associated with low-grade VUR in the contralateral renal unit (RU). Preoperatively, VUR was grade V in 3 RU, grade IV in 22 RU, grade III in 5 RU, and grade II in 4 RU. Ureteric reimplantation was performed in the native bladder in 24 RU (16 patients) and in the colon cap in 10 RU (6 patients).

Results

Mean age at sigmoidocolocystoplasty/UR was 8.5 years (range, 2-15 years). Mean follow-up was 12.8 years (range, 2-22 years). Postoperatively, cystourethrography showed residual VUR (grade IV to grade III) in only 1 RU (3%); diethylenetriamine pentaacetic acid scintigraphy showed no obstruction in all RU; ^99mTc-dimercaptosuccinic acid was improved in 13 RU, unchanged in 18 RU, and worsened in 3 RU; and serum creatinine remained normal in 20 patients and worsened in 2. Urinary tract infection, universal preoperatively, was seen postoperatively in only 2 patients. In the literature, 0% to 16.7% of HVUR persisted after BA alone, and no long-term data were available.

Conclusions

Ureteric reimplantation during BA is safe and effective for treating HVUR in patients with neurogenic bladder.     

Dynamic tracheal occlusion improves lung morphometrics and function in the fetal lamb model of congenital diaphragmatic hernia

Background

Congenital diaphragmatic hernia (CDH) is associated with significant neonatal morbidity and mortality. Although prenatal complete tracheal occlusion (cTO) causes hypoplastic CDH lungs to enlarge, improved lung function has not been demonstrated. Furthermore, cTO interferes with the dynamic pressure change and fluid flow associated with fetal breathing.

Purpose

The purpose of the study was to assess a novel dynamic tracheal occlusion (dTO) device that preserves pressure changes and fluid flow.

Methods

In this pilot study, CDH was created in fetal lambs at 65 days of gestational age (GA). At 110 days GA, a cTO device (n = 3) or a dTO device (n = 4) was placed in the fetal trachea. At 135 days GA, lambs were delivered and resuscitated. Unoperated lamb co-twins (n = 5), sham thoracotomy lambs (n = 2), and untreated CDH lambs (n = 3) served as controls.

Results

Tracheal opening pressure, lung volume, lung fluid total protein, and phospholipid were significantly higher in the cTO group than in the dTO and unoperated control groups. Maximal oxygenation and lung compliance were significantly lower in the cTO group when compared with the unoperated control and dTO groups.

Conclusion

Preliminary results suggest that in the fetal lamb CDH model, dTO restores normal lung morphometrics and function, whereas cTO leads to enlarged but less functional lungs.     

The optimal dose of Adriamycin to create a viable rat model potentially applicable to congenital obstructive uropathy

Purpose

The Adriamycin rat model is an established model for different organ anomalies including congenital obstructive uropathy. In the current study, we carried out a dose-response analysis to find out the optimal dose of Adriamycin to create a viable rat model of obstructive uropathy.

Methods

Thirty time-mated Sprague-Dawley rats were divided into 5 groups including 1 control group and 4 different treatment groups. The 4 Adriamycin dosage regimens investigated in this study were 1.25, 1.5, 1.75, and 2 mg/(kg d). Experimental rats (n = 24) were injected intraperitoneally with different doses of Adriamycin on gestational days 7 to 9 (6 rats in each group). Control rats (n = 6) were injected with an equivalent volume of saline on the same days. Viable term fetuses were harvested on gestational day 21 by cesarean delivery and dissected under a dissecting microscope. Serial transverse sections from urinary tract system were obtained for histological examination.

Results

One hundred thirty-three viable fetuses were recovered from Adriamycin-treated rats, and 50 were from rats in the control group. There were no resorptions in the control group; however, 52 resorptions were recorded in Adriamycin groups. The rates of hydronephrosis and resorptions were 60% and 0%, 80.5% and 5.8%, 100% and 17.3%, and 100% and 76.9% at doses of 1.25, 1.50, 1.75, and 2 mg/(kg d), respectively. Histologic examination of the kidneys in the treated groups showed a significant decrease in renal parenchyma compared with the control group.

Conclusions

The dosage of 1.5 mg/(kg d) of Adriamycin yielded the highest number of viable hydronephrotic fetuses. At this dose, urinary abnormalities are milder; but the highest number of viable fetuses is provided, which is necessary to create a reproducible and viable animal model.     

Prenatal and postnatal urologic complications of sacrococcygeal teratomas

Purpose

Urologic complications are an underappreciated sequelae of the mass effect of sacrococcygeal teratoma (SCT) and its resection. The goal of this study was to evaluate the incidence and severity of urologic complications in patients with SCT.

Methods

A retrospective review of patients with a prenatal diagnosis of SCT and postnatal referral for SCT at a single institution during a 5-year period (2004-2009) was performed. The presence of prenatal and postnatal urologic abnormalities were collected and analyzed.

Results

Thirty patients were identified for inclusion in the study. Twenty-two patients were diagnosed prenatally, 3 patients were referred for resection of the tumor, and 5 patients were initially treated elsewhere and referred after resection for urologic complications. Of the 20 patients with subsequent follow-up included in this study, 9 had neurogenic bladder, 5 of whom also had associated renal injury. All patients with renal injury had a higher grade Altman type II/III lesion.

Conclusion

Urologic problems appear to be common in patients with SCT. Higher grade SCTs should alert the surgeon to possible urologic sequelae, particularly neurogenic bladder and renal injury. Careful urologic evaluation and management of the genitourinary tract should be included in prenatal and preoperative counseling.     

Bacillus Calmette-Guérin without maintenance therapy for high-risk non-muscle-invasive bladder cancer

Background

Bacillus Calmette-Guérin (BCG) is the standard intravesical treatment of high-risk noninvasive (Ta, T1, Tis) bladder cancer. Maintenance BCG is recommended for maximum efficacy.

Objective

We compared our results in a large cohort of high-risk bladder cancer patients who received BCG without maintenance with published results from randomized maintenance BCG trials.

Design, setting, and participants

A cohort of 1021 patients underwent restaging transurethral resection for high-risk (Ta, T1, Tis) bladder cancer.

Intervention

Patients received a 6-wk induction course of BCG therapy. Responding patients did not receive maintenance BCG. Relapsing patients were eligible for retreatment with BCG. All patients were followed for a minimum of 5 yr.

Measurements

End points were 5-yr tumor- and progression-free survival rates.

Results and limitations

Of 816 complete responders to induction BCG, 2- and 5-yr recurrence-free survival rates were 73% and 46%, respectively. The progression-free survival rate was 89%. Progression-free survival time was 56 mo (95% confidence interval, 55-58 mo). Thirty-two percent of the patients required another course of BCG therapy. We cannot exclude that maintenance BCG may benefit patients beyond 5 yr over induction BCG alone and selective BCG retreatments.

Conclusions

Our results with BCG treatment without maintenance of patients with high-risk non-muscle-invasive bladder cancer compare favorably with trials in which comparable patients received maintenance BCG.