[Urology in Germany].
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Education, Medical↗
Biomedical subjects
R Hohenfellner
Publications and source records attributed to R Hohenfellner.
[The "nutcracker syndrome" of the renal vein (superior mesenteric artery syndrome) as the cause of gastrointestinal complaints].
HISTORY AND CLINICAL FINDINGS: Since the age of 19 a now 22-year-old man had complained of intermittent abdominal pain, irregular stools and paroxysmal tachycardia. The only preceding illness had been a single episode of iron-deficiency anemia. A laparoscopy, done 8 months after the onset of symptoms, had revealed an inflamed Meckel's diverticulum which was surgically removed. After transient improvement the symptoms recurred 5 months postoperatively. On admission to clarify the cause of the symptoms he had discrete abdominal pain on pressure, but physical examination was otherwise unremarkable. INVESTIGATIONS: Routine biochemical tests and endoscopy were normal. Abdominal computed tomography was suspicious of severe narrowing of the left renal artery by a crossing superior mesenteric artery. As a result the left testicular vein and the peripelvic venous network were markedly dilated by retrograde congestion, strongly suggesting the "nutcracker syndrome" of obstruction of the left renal vein. This diagnosis was confirmed by selective renal phlebography and pressure measurement. TREATMENT AND COURSE: The vascular anomaly was corrected surgically by reimplanting the left renal vein into the inferior vena cava 3-4 cm further caudally. The patients has been completely symptom-free since then. CONCLUSIONS: The nutcracker-syndrome is a rare cause of hematuria. The coexistence of this anomaly with gastrointestinal symptoms has not been previously described, but it is likely that congestion of the splanchnic veins by obstruction of the left renal vein was at least partly responsible for them, in view of the postoperative relief.
Abdominal Pain↗
Refinements in buccal mucosal grafts urethroplasty for hypospadias repair.
We evaluated our experience with buccal mucosa in urethral reconstruction for complex hypospadias repair in a total of 62 children operated on at our institution since 1990 at a mean follow-up of 23 months. After the graft had been taken from the lower lip, with possible extension to the inner cheek, complete thinning of the graft was performed. The graft was then sutured in an onlay fashion to the urethral plate, and suprapubic urinary diversion was carried out for 3 weeks. The overall complication rate in this rather complex series of patients, including 26 "hypospadias cripples," was 13%; the complications involved 4 fistulas, 3 cases of graft necrosis, and only 1 meatal stenosis during the follow-up period. These favorable results prompted us to use buccal mucosa onlays as our current method of choice for urethral reconstruction with avoidance of tubularized repairs. The definite value of this technique, however, will become evident only after a longer follow-up period and confirmation of these favorable results.
Adolescent↗
Long-term metabolic effects in patients with urinary diversion.
The use of intestinal segments in genitourinary reconstruction could influence vitamin metabolism and affect the skeletal bone and its mineral content in the long term. In 137 patients, serum levels of the vitamins A, B1, B2, B6, B12, D, and E and of folic acid, bile acid, and ammonia as well as levels of intracorpuscular vitamin B12 and folic acid were examined and a red blood cell count was performed. The patients were divided into three groups (< or = 2 years, > 2 to < or = 4 years, and > 4 years after surgery) as well as into children and adults. In addition, bone mineral density (dual-photon absorptiometry) was measured in 25 patients. Of these, 16 patients were approximately 16.8 years s/p rectal reservoir, 6 were approximately 20.5 years s/p colonic conduit, two were 6 and 8 years s/p ileocecal pouch, and one adolescent was 5 years s/p ileal bladder augmentation. In all patients the levels of vitamins A, B1, B2, B6, D, and E and of folic acid, bile acid, and ammonia as well as the red blood cell count were within normal ranges. In children (n = 51) there was no significant drop in vitamin B12 levels after the operation. In adults (n = 86), serum vitamin B12 levels dropped significantly from 402 +/- 182 ng/l during the first 2 years after the operation to 292 +/- 204 ng/l after the 4th year (normal range 240-1,100 ng/l). No significant increase in the intracorpuscular vitamin B12 level was observed during the same period. The bone mineral density was normal in all 25 patients with different types of urinary diversion. In addition to regular examination (sonography, creatinine levels, and base excess), vitamin B12 levels should be determined at 4 years after urinary diversion. It remains unclear whether substitution is necessary. However, substitution is easy to achieve and cheaper than the regular determination of vitamin B12. No decrease in bone mineral content was seen in the long-term follow-up with early correction of the base excess (below -2.5).
Adult↗
Treatment of interstitial cystitis: comparison of subtrigonal and supratrigonal cystectomy combined with orthotopic bladder substitution.
PURPOSE: We retrospectively evaluate the outcome of interstitial cystitis treated with subtrigonal or supratrigonal cystectomy and orthotopic bladder substitution. MATERIALS AND METHODS: Of 22 women and 1 man a mean of 51 years old with interstitial cystitis refractory to conservative therapy 17 were treated with subtrigonal cystectomy and ureteral reimplantation (group 1), and 6 were treated with supratrigonal cystectomy directly above the ureteral orifices (group 2). Both groups underwent orthotopic bladder substitution with an ileocecal pouch (Mainz pouch I). RESULTS: Postoperatively functional capacity significantly increased from a mean plus or minus standard error of mean 46 +/- 5 to 346 +/- 57 ml. in group 1 and 34 +/- 61 to 319 +/- 29 ml. in group 2 (p < 0.001). Daytime and nighttime urinary frequency significantly decreased from 24 +/- 2 to 8 +/- 1 and 7 +/- 1 to 2 +/- 1 ml., respectively, in group 1 and 28 +/- 2 to 6 +/- 1 and 6 +/- 1 to 1 +/- 1 ml., respectively, in group 2 (p < 0.001). At a mean followup of 93.9 months 14 patients in group 1 (82%) are completely symptom-free, and 1 has tolerable residual urinary urgency and suprapubic pain. At a mean followup of 31.5 months all group 2 patients are symptom-free and void spontaneously, whereas 41% of the group 1 patients require self-catheterization after subtrigonal cystectomy. CONCLUSIONS: For interstitial cystitis refractory to conservative treatment subtotal cystectomy with orthotopic bladder substitution with the ileocecal pouch (Mainz pouch I) is a valid therapeutic option. In this series supratrigonal and subtrigonal cystectomy resulted in similar relief of symptoms but the former appears to provide better functional bladder rehabilitation.
Adult↗
Whole-body potassium and bone mineral density up to 30 years after urinary diversion.
OBJECTIVES: To evaluate the long-term effects of different types of urinary diversion on skeletal bone density and whole-body potassium content in patients with an early correction of base excess (< -2.5). PATIENTS AND METHODS: The early correction of base excess (< -2.5) is one of the principles of the treatment of patients undergoing urinary diversion at our institution. In 27 patients with urinary diversion, bone mineral density (assessed by dual-photon absorptiometry), whole-body potassium, electrolyte and creatinine levels were determined, and capillary blood gas analysed. The mean time since surgery was 16.8 years in 16 patients with a rectal reservoir, 20.5 years in six patients with a colonic conduit, 7.8 years in four patients with an ileocaecal pouch and 5 years in one adolescent with an ileal bladder augmentation. RESULTS: Bone mineral density was normal in 25 of the 27 patients; no exact measurement was possible in the remaining two. The whole-body potassium content was reduced in eight of the 27 patients (three with a conduit, two with a rectal reservoir, two with an ileocaecal pouch and one with ileal augmentation). In four of these eight patients the base excess was < -2.5 (-2.7 to -5). Of the other four, two had no regular testing of their base balance and two were obese. The electrolytes were within the normal ranges. None of the patients had hyperchloraemic metabolic acidosis. CONCLUSION: With early correction of the base excess (< -2.5), there was no decrease in bone mineral density and no hyperchloraemic acidosis. There was no significant difference between the different types of urinary diversion. There seems to be a relationship between low base excess and decreased whole-body potassium.
Adolescent↗
History of oral mucosa.
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History, 19th Century↗
Loss of heterozygosity studies and deletion mapping identify two putative chromosome 14q tumor suppressor loci in renal oncocytomas.
Renal oncocytoma is considered to be a benign tumor that shares some phenotypic features with chromophobe renal cell carcinoma (RCC). Recently, we described high frequencies of allelic loss at 1p, 2p, 6p, 10p, 13q, 14q, 17p, and 21q, which correlate significantly with the chromophobe subtype of RCC. To investigate the genetic relationship between these two entities, we examined 12 oncocytomas for loss of heterozygosity (LOH) at these regions. In addition, we included markers for 3p, 5q, 7q, 11p, and 22q. The only chromosomal region showing similarly high frequencies of allelic loss for both subtypes was 14q. Therefore, a genetic relationship between renal oncocytoma and chromophobe RCC seems questionable. Eight of 12 oncocytomas (67%) showed LOH at 14q, a frequency that was significantly higher (P < 0.001, chi(2) test) than the frequencies of LOH in all other regions. To define regions potentially harboring novel tumor suppressor genes, we performed multifluorescence microsatellite analysis with 13 markers spanning 14q. Interstitial deletions at different regions of 14q were detected, with the highest frequencies at D14S258 (14q23-24.3) and D14S292 (14q32.1-32.2). 14q LOH might be associated with advanced-stage RCCs or other tumors, but it does not seem to indicate progression in oncocytomas. Its role in pathogenesis of renal oncocytomas remains to be clarified. Here, we provide evidence for two distinct tumor suppressor gene loci at 14q in renal oncocytoma, which will be useful for further fine-mapping studies of these critical regions.
Adenoma, Oxyphilic↗
Urinary diversion in children and young adults using the Mainz Pouch I technique.
OBJECTIVES: To determine the late complications and consequences for renal function, vitamin and acid-base metabolism after application of the Mainz Pouch I (MZP-I) technique in children and young adults. PATIENTS AND METHODS: To November 1994, the MZP-I procedure was carried out in 463 patients at our institution, 91 of whom were children and adolescents (< or = 20 years old) using bladder augmentation in 21 and a continent cutaneous stoma in 70. A minimum follow-up of 1 year was possible in 87 patients or 163 renal units (RUs) with a mean of 5.5 years (range 1-10.5). RESULTS: At the last examination, 23 of 55 (42%) preoperatively dilated RUs had improved. 131 of the 163 RUs (80%) were stable and nine RUs (5.5%) showed a slight clinical asymptomatic increase in the upper tract dilatation. Through an extraperitoneal flank incision, 11% of the RUs which developed stenosis at the ureterocolic anastomosis were successfully reimplanted (16% in patients with neurogenic disorders, 17% with pre-operative irradiation and 5% in the remaining patients). Two of 32 patients with an intussuscepted and invaginated ileal nipple required re-operation due to incontinence, but none of the patients with an appendiceal stoma were incontinent. Open revision of a stomal stenosis was performed in three and endoscopic treatment in nine patients. In 54 patients, the levels of vitamins A, B1, B2, B6, E, folic and bile acid were within normal ranges. There was no significant decrease in vitamin B12 levels after operation. In none of the patients with normal pre-operative creatinine values had the levels increased and none developed severe acidosis or bowel neoplasm. CONCLUSION: The MZP-I is recommended as a technique for bladder augmentation or continent urinary diversion in children and young adults, with an acceptable complication rate which offers long-term protection of the upper urinary tract.
Acid-Base Equilibrium↗
Pseudotumoral cystitis cystica of the urethra in a boy.
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Child↗
Treatment of patients with bladder exstrophy or incontinent epispadias. A long-term follow-up.
OBJECTIVE: To determine the late outcome concerning urinary continence, late complications, sexuality, and fertility in patients with the exstrophy-epispadias complex. METHODS: Until July 1994, 115 patients underwent surgical treatment at our institution. The mean follow-up period in 102 patients is 16.7 years. Urinary diversion was performed in 88 patients, a modified Young-Dees procedure in 8, a sling plasty in 3, and genital reconstruction alone in 3 patients. RESULTS: The present continence rates are 96% for rectal reservoirs, 97% for Mainz pouch 1, and 67% for the modified Young-Dees procedure. The upper tracts have remained stable, and no bowel neoplasms have developed. 16 of 17 women > or = 18 years of age are satisfied with the cosmetic result after genital reconstruction. All adults engage in sexual intercourse; 5 women have delivered 7 children by cesarean section. 30 of 32 male adults are satisfied with the cosmetic result of the reconstructed external genitalia. Penile deviation was present in 11, distressing in 2 patients. After genital reconstruction 9 developed epididymitis, necessitating 2 orchiectomies and 3 vasectomies. No patient with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas the ejaculation was normal in 3 who did not undergo genital reconstruction. Furthermore, 2 of the 5 have fathered 4 children. CONCLUSIONS: Rectal reservoirs represent our urinary diversion of choice. After failed reconstruction/insufficient anal sphincter, a Mainz pouch I is constructed. The cosmetic results achieved by genital reconstruction are satisfactory. In women, antefixation of the uterus should be performed before or together with an introitus plasty to prevent uterine prolapse. In men, however, surgery is performed at the expense of fertility.
Adult↗
Extramammary Paget's disease of the penis.
This case report describes a penile location of Paget's disease with delayed diagnosis due to initial outside treatment for several suspected benign dermatological disorders until surgery was eventually performed with confirmation of a Paget lesion of the penis. Diagnosis and therapy of this rare urologic disease are discussed and the literature is reviewed.
Aged↗
Vitamin metabolism in patients with a Mainz pouch I: long-term followup.
PURPOSE: We assessed whether creation of the Mainz pouch I, which requires 24 to 36 cm. of terminal ileum and 12 cm. of ascending colon including the ileocecal valve, leads to metabolic disturbances. MATERIALS AND METHODS: In 137 patients the levels of vitamins A, B1, B2, B6, B12, D and E, folic and bile acids, ammonia, and intracorpuscular vitamin B12 and folic acid were evaluated and a red blood count was performed. Patients were divided into children and adults, and into 3 groups according to number of years after surgery (2 or less, more than 2 to 4 and more than 4). RESULTS: In all patients the levels of vitamins A, B1, B2, B6, D and E, folic and bile acids, and ammonia as well as red blood count were within normal ranges. In the 51 children there was no significant decrease in vitamin B12 postoperatively. In the 86 adults mean serum vitamin B12 plus or minus standard deviation decreased significantly from 402 +/- 182 ng./l. during the first 2 years postoperatively to 292 +/- 204 ng./l. after year 4 (normal 240 to 1,100). There was no significant decrease in intracorpuscular vitamin B12 during this period. CONCLUSIONS: In addition to regular examinations, the determination of vitamin B12 levels 4 years after Mainz pouch diversion is mandatory. It remains unclear whether substitution is necessary. However, substitution is easy to achieve and even less expensive than the regular determination of vitamin B12.
Adult↗
Chloride absorption in patients with a continent ileocaecal reservoir (Mainz pouch I).
OBJECTIVE: To determine the rate of radioactive chloride absorption at various times after surgery in patients with a continent ileocaecal reservoir (Mainz pouch I). PATIENTS AND METHODS: The study was conducted on 20 patients (15 males and five females, mean age 47 years, range 15-72) who had undergone a Mainz pouch I urinary diversion. The patients were divided into three groups according to the duration of the post-operative follow-up, i.e. group 1 for < 6 months (n = 7), group 2 for 6-12 months (n = 8) and group 3 for > 12 months (n = 5). Radioactive 38Cl (20 MBq) was instilled into the pouch and the radioactivity determined in the blood, pouch and body interstitium every 20 min for 3 h. Samples for arterial blood-gas and acid-base profiles were obtained before and after instillation. RESULTS: In patients in group 1, 93% of the instilled chloride was absorbed into the circulation, while 51% was absorbed by patients in group 3. The acid-base profile showed no significant changes before and after chloride instillation. CONCLUSION: The absorption of chloride declines markedly with time after surgery in patients with a Mainz pouch I ileocaecal reservoir.
Absorption↗
Quality of life and health in patients with urinary diversion: a comparison of incontinent versus continent urinary diversion.
OBJECTIVE: To compare the quality of life and health in patients with incontinent and continent urinary diversions, in correlation with the initial diagnosis, diversion-related symptoms, psychological status and employment status. MATERIALS AND METHODS: 81 patients (64% male and 36% female) with a mean age of 55 years (18-65 years) were included in this retrospective study. A total of 27 had an incontinent urinary diversion (group A) and 54 a continent diversion (group B). The initial diagnosis was malignant tumor in 75% (n = 61) and nontumor disease in 25% (n = 20). A structured interview and psychometric instrument assessment of the quality of life as well as somatic and psychological symptoms were carried out and analyzed. RESULTS: Patients with nontumor disease, a continent reservoir and employment tended to have the highest level of quality of life. The higher the number and severity of psychological symptoms, such as depression and anxiety, the lower the level of global satisfaction with life, health and urinary diversion, and vice versa. No difference was seen between groups A and B concerning diversion-related symptoms, global satisfaction with life and health and sociodemographic data. CONCLUSIONS: The decision for a continent versus an incontinent urinary diversion must consider not only the medical factors of each individual patient, but also the initial diagnosis, psychological condition and employment status.
Adaptation, Psychological↗
Pregnancy after Mainz pouch urinary diversion.
PURPOSE: We report on our experience with pregnancies and deliveries in patients with a continent ileocecal reservoir with catheterizable stoma (Mainz pouch). MATERIALS AND METHODS: Six women aged 18 to 33 years (mean age 26.8 years) became pregnant after undergoing urinary diversion. A continent ileocecal reservoir was made comprising 5 umbilical stomas and 1 stoma in the left lower abdominal quadrant. Underlying diseases were bladder exstrophy in 4, meningomyelocele in 1 and urogenital sinus in another patient. Cesarean section was jointly performed by the obstetricians and urologists in all patients. RESULTS: Seven pregnancies occurred in a total of 6 women. Three pregnancies were completely uneventful. Pregnancy complications were ureteral dilatation in 4 cases with pyelonephritis and premature labor in 1 case. A nipple prolapse in the patient with the stoma in the left lower abdominal quadrant was easily corrected during cesarean section. All patients underwent cesarean section without complications. Seven healthy children were born. CONCLUSIONS: In women with Mainz pouch urinary diversion there appears to be no contraindication to pregnancy, while other forms of continent diversion await further evaluation. Urologists and obstetricians should be aware of the potential complications of this high risk pregnancy and delivery, and their interdisciplinary cooperation is essential for successful outcome.
Adolescent↗
Allelic losses at chromosomes 1p, 2p, 6p, 10p, 13q, 17p, and 21q significantly correlate with the chromophobe subtype of renal cell carcinoma.
We analyzed 50 sporadic renal cell carcinomas (RCCs) for loss of heterozygosity (LOH) at the chromosomal regions 1p, 2p, 6p, 7q, 10p, 11p, 13q, 14q, 17p, 21q, and 22q. Histologically, the tumors were distinguished into clear cell, chromophilic, and chromophobe carcinomas. Whereas LOH at 14q was identified in 42-64% of all three tumor types, only the chromophobe tumors showed high frequencies of LOH (73-91%) at 1p, 2p, 6p, 10p, 13q, 17p, and 21q. These findings provide substantial evidence that the chromophobe subtype of RCC represents a distinct genetic entity. Thus, specific LOH patterns may define the histogenesis and oncogenesis of chromophobe RCC and may be useful in tumor diagnosis and clinical prognosis.
Adenocarcinoma↗
Five-year experience with combined operative and radiotherapeutic treatment of recurrent gynecologic tumors infiltrating the pelvic wall.
BACKGROUND: Whereas 25 to 50% of selected patients with gynecologic tumors who relapse centrally in an irradiated pelvis can be salvaged by exenteration, postirradiation recurrence infiltrating the pelvic side wall generally has been fatal. We have designed the combined operative and radiotherapeutic treatment (CORT) procedure for the treatment of postirradiation recurrence infiltrating the pelvic wall and developed several new techniques for its realization. The aim of the surgery is as follows: (1) total resection of the tumor with only a microscopic margin (R1) at the pelvic wall, preserving the bony pelvis and the neurovascular support of the leg; (2) modulation of the therapeutic index for a second high-dose irradiation of the pelvic wall by transferring autologous tissue from the abdomen or the thigh, and (3) reconstruction of pelvic organ functions lost due to tumor resection. The tumor bed is irradiated postoperatively with brachytherapy through transcutaneous guide tubes implanted at the pelvic wall. METHODS: Between April 1989 and December 1994, we treated 48 patients with postirradiation recurrent or persistent gynecologic malignancies infiltrating the pelvic wall with CORT and followed them prospectively with the following endpoints: tumor control, survival, complications, and quality of life. RESULTS: At a median follow-up of 33 months (range, 3-71 months), the 5-year survival probability calculated with the Kaplan-Meier method was 44%. The overall local control rate was 68%, and 85% in the last 25 patients in the series. The censored severe complication rate at 5 years was 33%. No patient died as a consequence of the treatment. Quality of life was self-assessed with a validated questionnaire by 15 patients without evidence of disease, and was rated with a total of 74% of the maximum score points. Age of the patient, state of resection at the pelvic wall (R1 vs. R2), and recurrent tumor size independently influenced tumor progression after CORT in this series. CONCLUSIONS: CORT appears to be a feasible, innovative treatment with long term survival potential and acceptable quality of life for selected patients with postirradiation gynecologic tumor recurrence infiltrating the pelvic wall. R1 resection of the tumor at the pelvic wall is mandatory; however, the reconstruction options within the pelvis are limited.
Adult↗