Cloacal exstrophy: a unified management plan

BACKGROUND/PURPOSE: The belief that patients with cloacal exstrophy have a short and therefore useless colon is all too common. Frequently, the colon is used for urinary or vaginal reconstruction, and the possibility of a pull-through is lost. In the authors' experience, the use of a unified management plan allowed most patients to undergo pull-through and avoid a permanent stoma. METHODS: Twenty-five patients were treated for cloacal exstrophy in the authors' institution from 1985 through 1999. In all patients, bladder closure, omphalocele repair, and creation of a colostomy were performed at birth. All available colon, no matter how small, was incorporated into the fecal stream. After at least 1 year, patients were assessed for the ability to form solid stool through their stoma. Normal colonic length, capacity to form solid stool, or success with a bowel management regimen through the stoma were considered indications for pull-through. Genitourinary reconstruction was contingent on the colorectal plan. RESULTS: Colonic length ranged from normal in 12 patients, 40 to 70 cm in 3 patients, 10 to 30 cm in 4 patients, and less than 10 cm in 2 patients. All 25 patients underwent pull-through. Three are totally continent, 4 are continent with occasional soiling, 11 remain clean with a bowel management regimen, and 4 are too young to assess. One patient was clean, but now refuses bowel management. Two early patients, both with less than 10 cm of colon, now have ileostomies. CONCLUSIONS: During neonatal repair, a colostomy should be formed incorporating all pieces of colon, no matter how small. With time, most patients will be able to form solid stool, and a pull-through should be undertaken if that ability exists. Decisions regarding genitourinary reconstruction should be made only after the gastrointestinal plan is established to achieve the optimal use of available bowel.     

Cloacal exstrophy: a 22-year experience

Our 22-year experience in the management of 12 patients with cloacal exstrophy is discussed. All patients underwent functional bladder closure. Of 7 evaluable patients 3 (43 per cent) have continent intervals of 3 to 4 hours and constitute the first report of satisfactory urinary continence following functional bladder closure in patients with cloacal exstrophy. Of 8 patients with an XY karyotype 6 were raised as girls with satisfactory genitoplasty, while 2 raised as boys had functionally inadequate external genitalia. The management of the rudimentary hindgut varied and the alternative approaches are discussed. It appears that this patient population can be rehabilitated to lead productive lives.     

Bladder exstrophy: staged reconstruction

The modern staged approach to bladder exstrophy reconstruction has undergone significant changes since it was first advocated by Jeffs and Cendron in the 1970s. Although varied surgical approaches have been tried in the bladder exstrophy condition, the staged approach to bladder exstrophy repair has withstood the test of time. Progress continues to be made in evaluating the outcome of older types of staged reconstruction, with continuing modification and improvements in the modern approach to staged reconstruction. This treatise will update the reader on recent advances in the treatment of bladder and cloacal exstrophy.     

Cloacal exstrophy: a 25-year experience with 50 cases

PURPOSE: The aim of this study was to evaluate the outcomes of reconstruction of all cloacal exstrophy cases seen by the authors from 1974 to 1999. METHODS: A retrospective chart review and personal follow-up was conducted on all 50 patients, who ranged in age from newborn to 35 years. Thirty-eight were secondary cases, 12 were primary (no previous surgery). Twenty-eight (56%) were genetic boys; 6 were raised as boys and 22 as girls. Forty patients underwent extensive reconstructive surgery; 6 await reconstruction, and 4 were seen only in consultation. For urinary continence, 21 had bladder neck narrowing, 7 received a bowel nipple, and 12 had a Mitrofanoff catheterizable conduit on the abdominal wall, using tapered intestine in 8, ureter in 3 and the appendix in 1. Four patients had a urostomy. Bladder augmentation was performed in 35 patients (18 with stomach, 11 small bowel, and 6 with both). Twenty-five patients had pull-through of their colon to the perineum. Thirty-two have had a vagina constructed. Forty-seven of the 50 patients had spinal cord tethering, and most underwent neurosurgical release. RESULTS: Overall survival rate was 98%; 1 patient died preoperatively at another institution. Acceptable bowel continence was achieved with enema washouts in 19 of 25 pullthroughs; 4 failed and were reversed. One case is too recent to judge. Of 40 reconstructed cases, 31 were dry, 3 had slight leakage, and 1 is too early to evaluate. Five had enough leakage to require further surgery. CONCLUSIONS: The once hopeless anomaly of cloacal exstrophy is treatable with extensive reconstructive surgery. Continence of urine (mainly by catheterization) and stool (mainly by enema washouts) is achievable in most. The hindgut, including the cecum and the usually rudimentary distal colon, should be saved and used as colon, not for urinary or vaginal reconstruction. In genetic boys, the authors continue to believe that gender assignment should depend on the likelihood for reconstructing an adequate phallus.     

The staged approach to bladder exstrophy closure and the role of osteotomies

Since the 1970's, the staged reconstruction of bladder exstrophy has yielded consistent surgical success. The Johns Hopkins Hospital approach begins with early pelvic ring approximation with abdominal wall, bladder, and posterior urethral closure. Within the first 72 hours of life, the malleable pelvis can sometimes be approximated without osteotomies. Beyond this age, the author's prefer a combined vertical iliac and horizontal innominate osteotomy. Second, we typically perform the epispadias closure at 1 year of age. A modified Cantwell-Ransley technique is performed, usually yielding an increase in bladder capacity and very satisfactory results. In the last phase, the modified Young-Dees-Leadbetter continence procedure along with transtrigonal/cephalotrigonal ureteroneocystostomies are performed when the urethra is catheterizable, the bladder capacity is 60cc or greater, and the child will participate in a postoperative voiding program (typically 4–5 years of age). This applied approach usually results in a continent, voiding patient with pleasing external genitalia and preserved renal function.     

Univentricular atrioventricular connection with subaortic stenosis: a staged surgical approach.

A staged surgical approach was developed for the management of hearts with univentricular atrioventricular connection (double-inlet left ventricle or tricuspid atresia) and discordant ventriculoarterial connection with anatomical substrate for the development of subaortic stenosis. This consisted of initial palliation with pulmonary artery banding, followed by early elective relief of subaortic obstruction using a proximal pulmonary artery to ascending aorta anastomosis in infancy. Pulmonary blood flow was maintained at this time by creating a bidirectional superior cavopulmonary anastomosis. Over an 18-month period, 5 children, including 4 seen in the first week of life with aortic arch obstruction, were palliated with this approach. All patients survived operation and are asymptomatic with transcutaneous oxygen saturations of 80% to 85%. Completion of cavopulmonary repair is planned at 2 years of age. Although some authors have considered pulmonary artery banding contraindicated in these infants, the current staged approach offers an attractive alternative to the construction of a pulmonary artery to aorta anastomosis in the neonatal period.     

Cloacal exstrophy: prenatal diagnosis before rupture of the cloacal membrane.

Embryologically, cloacal exstrophy is thought to result from persistence and subsequent rupture of the infraumbilical cloacal membrane during the fifth embryonic week. We report a case of cloacal exstrophy in which a prenatal diagnosis was made prior to rupture of the cloacal membrane. A routine ultrasound at 17 weeks' gestation demonstrated monoamniotic twins. One twin was normal, but the other was found to have a sacral myelomeningocele, "rocker-bottom" feet, splaying of the pubic rami, and a large cystic mass protruding from the infraumbilical anterior abdominal wall. A repeat ultrasound was performed at 22 weeks, with the same findings. At 26 weeks, further examination showed disappearance of the abdominal cyst, a small omphalocele, no demonstrable bladder, and the suggestion of prolapsed bowel inferior to the umbilical cord insertion. After delivery at 34 weeks, the abnormal twin was found to have the typical findings of cloacal exstrophy, myelomeningocele, bilateral lower limb anomalies, and extremely foreshortened small bowel. Rupture of the presumed cloacal membrane after 22 weeks in this case is inconsistent with our current understanding of the embryology of this anomaly, and should stimulate a reexamination of the current concepts. If the characteristic features are recognized, cloacal exstrophy can be diagnosed by prenatal ultrasound, permitting prenatal counseling and appropriate perinatal management.     

Cloacal exstrophy: morbidity associated with abnormalities of the gastrointestinal tract and spine

Background/purpose

The aim of this study was to review the management of the gastrointestinal tract in cloacal exstrophy and to assess the effect of spinal dysraphism on nutritional outcome.

Methods

Twenty-two patients with cloacal exstrophy were reviewed retrospectively. Gastrointestinal and spinal anomalies were documented. The need for nutritional supplementation was recorded. Weights at 1 and 5 years of age were used to assess growth in childhood.

Results

Ten patients were treated initially with ileostomy, 7 with colostomy, and 3 without stoma. Two patients died before surgical reconstruction. Total parenteral nutrition (TPN) was utilized in 8 infants for a median of 10 days (range, 5 to 200). Three patients had complications with colostomies requiring conversion to ileostomy. Patients with an ileostomy required more nutritional supplementation compared with patients with a colostomy (50% v 28%). High-output stoma losses were more common in patients with an ileostomy (40% v 14%). Fourteen patients (67%) with spinal dysraphism had a higher incidence of failure to thrive in the first year of life (69% v 26%) and multiple episodes of enteritis (40% v 25%). Two neonates with duodenal atresia and small bowel deletion died within the first month of life. One patient with short bowel syndrome died of TPN-associated liver disease at 6 months of age. There were no other deaths.

Conclusions

The gastrointestinal tract contributes significantly to the morbidity and mortality in cloacal exstrophy. Nutritional supplementation is more frequently required in patients with an ileostomy. Stoma complications were higher in those with a colostomy. Morbidity is high in patients with spinal dysraphism.     

Muscarinic cholinergic receptors in bladder exstrophy: insights into surgical management

The surgical management of classical bladder exstrophy (functional bladder closure or urinary diversion) should be influenced by the inherent detrusor function of the exstrophied bladder. Cystometrograms performed previously on individuals with successful exstrophy closures demonstrate normal bladder function. The biochemical and neurophysiological properties of the exstrophied bladder have otherwise not been investigated. In this study radioligand receptor binding techniques were used to compare the density and equilibrium dissociation constant of muscarinic cholinergic receptors in control and exstrophy bladders. The density of muscarinic cholinergic receptors in the control and exstrophy groups was 1.97 plus or minus 0.29 and 1.44 plus or minus 0.21 fmol. per microgram deoxyribonucleic acid (mean plus or minus standard error of mean), respectively. The dissociation constant of the control and exstrophy groups was 0.15 plus or minus 0.02 and 0.14 plus or minus 0.02 nM. (mean plus or minus standard error of mean), respectively. These data show that the muscarinic receptor density and binding affinity in control and exstrophy bladders are similar. Therefore, the neurophysiological composition of the exstrophied bladder is not grossly altered during the anomalous development.     

Tetralogy of Fallot: surgical management individualized to the patient

BACKGROUND: Over the past four decades, the surgical trend has been toward early, complete repair of tetralogy of Fallot (TOF). Many centers currently promote all neonates for total correction irrespective of anatomy and symptoms, with some surgeons advocating hypothermic circulatory arrest for repair in small infants. We believe this approach increases morbidity. METHODS: Based on approximately 40 years' experience in 2,175 patients, we developed a management protocol focused on patient size, systemic arterial saturations, and anatomy. Symptomatic patients (hypercyanotic spells, ductal dependent pulmonary circulation) weighing less than 4 kg undergo palliative modified Blalock-Taussig shunt (BTS) followed by complete repair at 6 to 12 months. Asymptomatic patients, weighing less than 4 kg who have threatened pulmonary artery isolation, undergo BTS and repair at 6 to 12 months. All other patients undergo complete repair after 6 months. RESULTS: From July 1, 1995, to December 1, 1999, 144 patients underwent operation for TOF (129 patients) or TOF with atrioventricular septal defect (TOF/AVSD, 15 patients). Ninety-four patients underwent one stage complete repair (88 TOF, 6 TOF/AVSD). Thirty-nine patients underwent repair after initial BTS (32 TOF, 7 TOF/AVSD). Ten patients are awaiting repair after BTS. The mean age and weight at complete repair were 18 months and 9 kg. There were no operative deaths. There have been 3 late deaths with complete follow-up (mortality 3 of 144 [2.1%]). Four of 133 patients (3%) have required reoperation after total correction. CONCLUSIONS: This management strategy optimizes outcomes by individualizing the operation to the patient. Advantages include avoidance of circulatory arrest, low morbidity and mortality, and low incidence of reoperation after complete repair.     

Congenital talipes equinovarus: II. A staged method of surgical management

A staged method of surgical management for congenital talipes equinovarus is described. The hindfoot was corrected and rebalanced early in 125 feet, and in 66 feet a second-stage medial forefoot correction was performed in the second, third or fourth year. The hindfoot relapsed in 19% and the forefoot in 9%; these feet were treated by further soft-tissue surgery. No bony operation was necessary. Assessment before and after operation allows comparison with other series.     

Improved results with surgical treatment of renovascular hypertension: an individualized approach

During a one-year period, 20 patients were treated for renal artery occlusive lesions causing renovascular hypertension. Diagnosis depended on angiography, isotopic renography, and selective renal vein renin determinations in certain patients. Treatment included saphenous aortorenal bypass, 9 patients; endarterectomy, 3 patients; percutaneous angioplasty, 5 patients; and nephrectomy in 3 patients. There was no operative mortality. Eighty-five percent of the patients were improved or cured of hypertension, and the average number of antihypertensive medications required decreased from 3.1 to 1.6. The mean serum creatinine of all patients decreased from 3.6 mg/dl to 2.2 mg/dl. In one subset of patients expanding suprarenal aneurysms caused renal artery stenosis or occlusion. Aortic replacement with renal artery endarterectomy or bypass is the only treatment option for these patients. High risk patients with normal renal function and a glomerular filtration rate less than 15% in the affected kidney can be treated with nephrectomy. Our results demonstrate that an individualized approach to therapy and attention to operative detail is mandatory for successful treatment of renovascular hypertension.     

Classic bladder exstrophy: orthopaedic surgical considerations

Classic bladder exstrophy is an embryologic malformation that results in complex deficiency of the anterior midline, with urogenital and skeletal manifestations. Urogenital reconstruction is a challenging procedure that can be facilitated by closure of the bony pelvic ring by an orthopaedic team. Surgical options include a multiyear staged approach and the single-stage complete repair for exstrophy. The goals of urologic surgery include closure of the bladder and abdominal wall with eventual bladder continence, preservation of renal function, and cosmetic and functional reconstruction of the genitalia. Pelvic osteotomy is done at the time of bladder closure in the patient in whom the anterior pelvis cannot be approximated without tension. Traction or spica casting is used postoperatively. Good outcomes are probable with appropriate management at specialized treatment centers.     

An individualized approach to the management of gastroschisis

A 93% survival rate was achieved in 80 neonates treated for gastroschisis between 1979 and 1986. Uncomplicated gastroschisis occurred in 70 infants (88%); 51% underwent staged silo reduction and 49% had primary fascial closure. Gastroschisis associated with intestinal atresia or volvulus was present in 10 neonates (12%), half of whom had a residual jejunoileum between 10 and 55 cm. Major postoperative complications included gastrointestinal problems (infarction, obstruction, and prolonged dysfunction), wound infection, and catheter-associated difficulties (sepsis, infiltration, and malposition). Three of the six deaths were related to associated conditions (extreme prematurity, trisomy 13, and multiple anomalies) and three were caused by intraoperative hemorrhage, necrotizing enterocolitis, and extensive short-bowel syndrome. No statistical difference in morbidity, mortality, and length of hospitalization was demonstrated between infants treated by silo reduction and primary closure. Safe management of gastroschisis should include an individualized assessment of visceroabdominal disproportion and degree of intraabdominal tension. Vigilant expectation of potentially life-threatening complications is required to decrease postoperative morbidity, irrespective of the technique of abdominal wall closure.     

Factors predisposing to renal scarring: following staged reconstruction of classical bladder exstrophy

Preservation of renal function is one of the major goals of staged reconstruction of bladder exstrophy. In 68 exstrophy patients who have completed staged reconstruction, 10 (14.7%) have developed renal scarring. A multiple factorial analysis of these patients showed the following factors to be statistically related to the development of renal scarring: (1) one or more febrile urinary tract infections prior to bladder neck reconstruction (P less than .001); (2) failure to utilize antibiotic prophylaxis following initial bladder closure (P less than .005); (3) elevated urinary residuals greater than 50 mL (P less than .02); (4) a diagnosis of elevated urinary residuals greater than 6 months following the onset of continence (P less than .001); and (5) one or more febrile urinary tract infections following bladder neck reconstruction (P less than .005).     

乳腺癌诊治进展——多学科与个体化

乳腺癌诊治包括预防、筛检、早期诊断、局部与全身治疗等,均涉及多个科学领域。多学科诊治模式包括多学科门诊和多学科会议等,已为学术界广泛认可。循证医学以随机对照临床试验和荟萃分析为主要工具,在传统医学临床病理学指标的基础上,依据雌激素受体（ER）、孕激素受体（PR）、人表皮生长因子受体2（HER-2）等生物学指标将乳腺癌进一步分成不同类型的亚群。个体化策略是乳腺癌治疗所追求的目标,其任务是利用基因组学的方法找出与疾病预测和预后相关的临床生物学指标,提高疗效并且降低治疗的并发症发生率。     

Three staged approach to the surgical management of renal cell carcinoma extending into the right atrium. Case report

Left renal cell carcinoma extending into the right atrium was treated by angioinfarction, removal of right atrial tumour using cardiopulmonary bypass and ten days later abdominal radical nephrectomy and inferior vena cava thrombectomy. Twenty four months later the patient remains well with no evidence of tumour recurrence.     

Insertional Achilles tendinosis: surgical treatment through a central tendon splitting approach

Twenty-two heels in 21 patients treated surgically for a primary diagnosis of insertional Achilles tendinosis were reviewed on the basis of preoperative and postoperative examinations, office records, and a comprehensive questionnaire administered to each subject. Each patient underwent surgical treatment using a midline-posterior skin incision combined with a central tendon splitting approach for debridement, retrocalcaneal bursectomy, and removal of the calcaneal bursal projection as necessary. The findings at surgery revealed involvement of the middle third of the insertion in 21 of 22 cases with only one patient manifesting isolated lateral involvement. Thirteen of 22 had an associated prominent calcaneal bursal projection and four of 22 a superficially inflamed bursa. Three patients required reinsertion of the tendo Achilles via drill holes and one underwent augmentation with a plantaris tendon. Operative findings and complications were reported. Eight male and 13 female patients underwent 22 procedures (one case bilaterally) with an average follow-up of 33 months. Preoperative symptoms include presence of symptoms over a range of three months to two years and pain associated with activities of daily living (17 of 22), limitation of regular activities (six of 22), and pain present at rest in six of 22. Postoperatively, 20 of 22 patients were able to return to work or routine activities by three months; only 13 of 22 were completely pain free. Only 13 of 22 also claimed that they were able to return to unlimited activities. Overall, there was an 82% (18 of 22) satisfaction rate with surgery and 77% (17 of 22) stated they would have the surgery again.