Pathological Features of the Unilateral Favorable Histology Nephroblastoma with Relapse.

PURPOSE: To evaluate the pathological features of the primary lesion in patients with relapse of unilateral favorable histology nephroblastoma. MATERIAL AND METHODS: Fifty-eight patients with unilateral favorable histology nephroblastoma who underwent initial nephrectomy before chemotherapy were categorized into one of two groups: the nonrelapsed group (n = 52) and the relapsed group (n = 6). The histological subtypes of both groups and pathological features of the relapsed group were re-evaluated retrospectively. RESULTS: The histological subtypes of all relapsed cases were classified as blastemal predominant. In three of six cases with relapse, sheets of spindle-shaped blastemal cells that were histologically reminiscent of synovial sarcoma were predominant (massive sarcomatoid pattern). CONCLUSIONS: The histological blastemal predominant subtype of nephroblastoma is a strong indicator of relapse. In particular, the blastemal predominant subtype with massive sarcomatoid pattern may have a higher risk of relapse.

Valved shunt as a treatment for obstructive uropathy: does pressure make a difference?

PURPOSE: A valved ventriculo-peritoneal shunt (V-P shunt) as a vesico-amniotic shunt (V-A shunt) preserves the filling/emptying cycle and normal bladder development in fetal lambs with bladder outlet obstruction. The optimal pressure for such shunts is unknown. MATERIALS AND METHODS: We created obstructive uropathy in 60-day gestation fetal lambs. A V-A shunt was placed 3 weeks later, using a low-pressure (Group L: 15-54 mmH2O) or a high-pressure (Group H: 95-150 mmH2O) V-P shunt. We included non-shunted (obstructive uropathy, Group O) and control lambs (Group C). All were delivered at 130 days. Bladder volumes, bladder thickness, renal and bladder histology were compared. RESULTS: Seventeen lambs had an obstructive uropathy created. Five Group L (four survived), four Group H (three survived) and five Group O survived. Body weight and crown-to-rump lengths of the three groups were not significantly different. Group H lambs had a dilated urachus, urinary ascites and severe ureteral dilatation similar to Group O lambs. There were four Group C lambs. Bladder volume was 10, 15 and 1,150 ml in Group H, 115 ± 67.9 ml in Group L, 128 ± 99.8 ml in Group O and 24.5 ± 3.84 ml in Group C. Unlike Group O lambs, Group L did not have urinary ascites, urinomas or renal dysplasia. CONCLUSION: Low-pressure shunts preserved both bladder volume and renal development. High-pressure shunts did neither.

Combined free autologous auricular cartilage and fascia lata graft repair for a recurrent tracheoesophageal fistula.

Repair of recurrent tracheoesophageal fistula (TEF) after repair of congenital esophageal atresia continues to be a difficult problem. The most common re-operation for a recurrent TEF involves repair via a right thoracotomy and use of a flap as interposed tissue between the closure sites. Although several materials have been reported for recurrent fistula repair, natural pedicled flaps have been used in most previous reports. The harvesting of a pedicled flap can sometimes be difficult when the patient has had multiple surgeries. In this report, the successful use of a combined free autologous auricular cartilage and free fascia lata graft repair for a complex patient with multiple recurrent TEFs is described. We believe that our technique should be considered as an approach for patients with recurrent TEF.

Long-term outcomes after excision of choledochal cysts in a single institution: operative procedures and late complications.

PURPOSE: The purpose of this study was to evaluate long-term outcomes for a minimum of 3 years after cyst excision in children with choledochal cysts, focusing on the relationship between operative procedures and outcomes. METHODS: Between 1977 and 2008, 138 children underwent cyst excision. Follow-up results were obtained from 120 patients. Their mean age was 20.9 years (range 4-49). The mean interval between surgery and conducting the analysis was 16.6 years (range 3-34). These patients were divided into two groups based on their operative procedures: group A (1977-2000) comprising 76 patients who underwent cyst excision with hepaticojejunostomy below the hilum, and group B (2001-2008) comprising 44 patients who underwent excision of the extrahepatic bile duct from the confluence of the hepatic duct to near the level of the pancreatobiliary junction with wide hilar hepaticojejunostomy. When hepatic strictures were seen near the hilum, duct plasty was made. We evaluated the long-term outcomes in the two groups. RESULTS: Late complications were seen in a total of 18 patients (15.0%). In group A, 16 patients (21.1%) had late complications, which included cholangitis and/or hepatic stones in 9, stones in residual intrapancreatic cysts in 4, intestinal obstruction in 2, and pancreatitis in 1. Of these 16 patients, 12 patients (15.8%), including 7 with hepatic stones (6 IV-A and 1 Ic cysts), 4 with remnant intrapancreatic cysts, and 3 with intestinal obstruction underwent surgical intervention. In group B, none of the patients developed cholangitis, pancreatitis, or stone formation. However, 2 patients (4.5%) developed intestinal obstruction that required surgery. CONCLUSIONS: Although a longer follow-up period is necessary, late complications were more frequent in group A than in group B patients and with type IV-A cysts. We believe that excision of the extrahepatic bile duct with wide hilar hepaticojejunostomy is essential for the prevention of postoperative complications.

Relapse of unilateral favorable histology Wilms' tumor: significant clinicopathological factors.

PURPOSE: To evaluate the clinicopathological features that indicate relapse and suggest a new risk based therapeutic strategy for unilateral Favorable Histology Wilms Tumor (FH-WT). MATERIALS & METHODS: Thirty-three patients with unilateral WT were treated in two institutions between 1986 and 2010. Twenty-eight patients with FH-WT received primary nephrectomy according to the National Wilms' Tumor Study (NWTS) or the Japanese Wilms' Tumor Study (JWiTS) protocol. Retrospective analyses of the non-relapsed group (n=23) and the relapsed group (n=5) compared age, gender, tumor laterality, tumor weight, initial tumor stage, known histological subtype, chemotherapy (2 or 3 drugs), and any irradiation delivered. Stages and histological subtypes of the tumors were re-evaluated according to the Japanese staging system. RESULTS: Five of the twenty-eight tumors relapsed, and one patient died. The initial staging (P=0.029) and the histological subtype (P=0.003) were the only factors indicating relapse. Nine of the twenty-three tumors were histologically classified as blastemal predominant subtype (BPT-WT). Five relapsed. CONCLUSION: According to the basic Japanese therapeutic strategy, all patients underwent a primary nephrectomy before chemotherapy. This study suggests that the histological subtype pre-treatment "BPT-WT" should be included as a strong indicator of poor prognosis. Such patients should be treated as a high-risk group.

Can we predict the prognosis of resectable hepatoblastoma from serum alpha-fetoprotein response during preoperative chemotherapy?

PURPOSE: The objective of this study was to clarify whether the alpha-fetoprotein (AFP) reduction rate during preoperative chemotherapy represents a prognostic factor for hepatoblastoma. METHOD: We divided 14 hepatoblastoma patients who underwent preoperative chemotherapy and curative resection into Group A (no recurrence; n = 10) and Group B (recurrence; n = 4). We then compared AFP levels before and after preoperative chemotherapy between groups. RESULT: Mean AFP level after completing the first cycle of chemotherapy was reduced to 7.28 % (range 1.2-36.8 %) in Group A and 17.05 % (range 12.0-20.5 %) in Group B (p < 0.05). Mean AFP after total preoperative chemotherapy was reduced to 1.42 % (range 0.07-8.5 %) in Group A and 7.55 % (range 3.4-12.4 %) in Group B (p < 0.02). Eight patients in whom AFP levels decreased >1 log after the first cycle of preoperative chemotherapy survived without recurrence. CONCLUSION: A large, early decrease in AFP level during preoperative chemotherapy may offer a strong indicator of survival. Patients in whom AFP levels do not decrease easily during preoperative chemotherapy may have increased risk of recurrence and should be followed very closely.

The long-term prognosis of two-flap anoplasty for mucosal prolapse following anorectoplasty for anal atresia.

AIM: Mucosal prolapse is a common complication following anorectoplasty for anorectal malformation. The symptoms such as soiling, staining, and pain significantly reduce the patients' quality of life. Millard et al. (Plast Reconst Surg 69(3):399-411, 1982) reported the two-flap anoplasty that creates an anal canal using two perineal pedicle skin flaps to form a "deep anus". We have used this procedure for mucosal prolapse since 1990. This study evaluated the long-term benefits of this method. METHODS: From 1990 to 2009, 18 patients suffering mucosal prolapse following anorectoplasty for high imperforate anus were treated using a two-flap anoplasty (TFARP) or just mucosal resection (MR). For each procedure, the long-term clinical follow-up (maximum of 20 years) was assessed by review of medical records against the frequency of recurrence, and the recurrence of preoperative symptoms postoperatively. RESULTS: Of the 18 patients, 8 presented with simple mucosal prolapse, 4 with bleeding, 3 with staining, 2 with incontinence, and 1 with pain. TFARP was performed for 14 patients and MR for 6 patients. In the MR group, during the maximum of 15 years follow-up, two patients (33 %) suffered a recurrence or failed to improve their symptoms such as bleeding and/or soiling. In the TFARP group, during the maximum of 20 years follow-up, there were no recurrences and their preoperative symptoms resolved completely. Furthermore, two patients in the TFARP group gained normal sensation prior to defecation. CONCLUSION: The advantages of this procedure were no recurrences and complete resolution of preoperative symptoms. Moreover, it provides the possibility of gaining sensate defecation, possibly because the skin flap around the anus might help develop sensation.

Outcome of laparoscopic modified Duhamel procedure with Z-shaped anastomosis for Hirschsprung's disease.

BACKGROUND: Several laparoscopic Duhamel-type procedures for Hirschsprung's disease (HD) have been reported, but laparoscopic, Z-shaped, colorectal, side-to-side anastomosis has not been described. Z-shaped anastomosis has been used as the treatment of choice for HD at our clinic for a long time. A laparoscopic approach was adopted to perform this Z-shaped anastomosis in 2001. We describe herein our experiences with laparoscopic Z-shaped anastomosis and evaluate the clinical outcomes. METHODS: Between 2001 and 2010, 26 children with rectosigmoid HD underwent a laparoscopic modified Duhamel procedure comprising Z-shaped anastomosis. Mean age at operation was 17.2 months. Mean weight was 8.7 kg. No children had a colostomy. Seven children had trisomy 21 and one had Klinefelter syndrome. Laparoscopic modified Duhamel procedure was performed using three 5-mm trocars. Intra- and postoperative complications and functional outcomes were evaluated. Patients with trisomy 21 were excluded from the functional evaluation. RESULTS: The operation was completed laparoscopically for 25 of the 26 patients, with only one patient requiring conversion to an open procedure because of injury to the ureter. Mean operating time was 296 min. In one case, a minor leak was observed. No infections or related complications were observed. Mean follow-up period was 50.4 months. Sudden death secondary to rotaviral enterocolitis occurred 8 months postoperatively in one case. Twenty-one of the 25 patients (84%) showed episodes of constipation during the early follow-up period, and one child required late myectomy due to sphincter achalasia. As the child grew older, the need for medication was diminished. Of the 14 patients over 4 years old, excluding those patients with trisomy 21, all achieved normal defecation without incontinence. CONCLUSIONS: Our series revealed that all patients over 4 years old who underwent laparoscopic Z-shaped colorectal anastomosis achieved normal defecation without fecal incontinence. Laparoscopic Z-shaped colorectal anastomosis for HD appears feasible and safe to perform with good results.

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 usefulness of laryngotracheal separation in the treatment of severe motor and intellectual disabilities.

Intractable aspiration is a life-threatening medical problem in patients with severe motor and intellectual disabilities (SMID). Laryngotracheal separation (LTS) is a surgical procedure for the treatment of intractable aspiration which separates the upper respiratory tract from the digestive tract. We performed LTS for 14 patients with SMID to prevent intractable aspiration, performing two types of operation. The standard diversion procedure connected the upper trachea to the esophagus. The modified diversion includes closure of the proximal trachea and a high tracheostomy, avoiding a tracheoesophageal anastomosis. LTS was performed on 14 patients. Operations performed before the LTS included tracheostomy in four patients, fundoplication in six and gastrostomy in two. A standard diversion was performed in 11 patients and a modified diversion in 3. There were no operative complications. Eleven patients were safely transferred to home-care after their LTS. Twelve patients are still alive and two died some months after operation. One patient died from their primary disease and the other died a tracheo-innominate artery fistula (TIAF). We recently experienced a patient who was at high risk of developing a TIAF. LTS is an effective operation, preventing intractable aspiration in patients with severe motor and intellectual disabilities. The results are similar for the standard or modified diversion procedure with the procedure chosen being related to the initial tracheostomy site. The most serious complication is a lethal TIAF.

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 microm; group B, 741 +/- 128 microm; and group C, 374 +/- 120 microm. 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.

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.

Fetal hydrops in experimental obstructive uropathy resolves after vesicostomy formation: is this cause and effect?

The association of obstructive uropathy and hydrops is rare but often fatal. Hydrops has been observed in our fetal lamb model with obstructive uropathy. We created a vesicoamniotic shunt 21 days after creating the obstruction to explore the effect of relieving the obstruction on the hydrops. Fetal lambs underwent urethral and urachal ligation at 60 days gestation. We created a vesicostomy (female) or urethrostomy (male) in 12 lambs to release the pressure 21 days after creating the obstruction. The fetuses were delivered at term (145 days), and the urinary tract was removed for histological examination. Six fetuses had severe hydrops at the time of the vesicostomy (group A), and six had no hydrops (group B). Only two lambs from group A survived (33%), and four lambs survived from group B (66%). Three lambs miscarried, and one was stillborn from group A. Two lambs from group B miscarried. In our fetal lamb model, hydrops appears to be associated with massive urinary ascites. We hypothesize that a connection exists between urinary ascites and hydrops. Vesicostomy, by bypassing the obstruction, may allow the lambs to recover from their hydrops. However, it is possible that by 21 days after creation of the obstruction, the damage created by the hydrops is irreversible.