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.

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.

Primary anastomosis for meconium peritonitis: first choice of treatment.

PURPOSE: Newborn surgery for meconium peritonitis (MP) is sometimes very difficult owing to severe adhesions and bleeding. The aim of this study was to reveal the benefit of primary anastomosis (PA) for MP by comparing PA with multistep operations (MO). PATIENTS AND METHODS: We retrospectively reviewed 38 patients with MP who underwent surgery in our institution from 1983 to 2009. From 1983 to 2000, we essentially used MO. After 2001, we used PA with the exception of 1 patient. We performed MO on 20 patients (group A) and PA on 18 patients (group B). RESULTS: Mortality was 4 in 20 in group A and 1 in 18 in group B. Three patients in group A and 2 in group B required reoperation because of complications. After 2001, 14 of 16 patients underwent PA. Of the 2 patients for whom PA could not be performed, one was postresuscitation from cardiopulmonary arrest and the other was an extremely low-birth-weight infant. The only mortality among the patients who underwent PA occurred in a very low-birth-weight infant who died from intraoperative hepatic hemorrhage. CONCLUSION: PA can be performed for almost all patients with MP except for extremely low-birth-weight infants.

Esophagogastric separation and abdominal esophagostomy via jejunal interposition: a new operation for extreme forms of pathologic aerophagia.

Pathologic aerophagia is sometimes seen in patients with neurologic disorders. It rarely causes massive bowel distention, ileus, and volvulus. Here, we report the use of esophagogastric separation and abdominal esophagostomy via jejunal interposition to prevent bowel distention caused by severe aerophagia in 2 patients with neurologic disorders in whom the usual nonoperative methods of management failed. In both cases, swallowed air was evacuated via the jejunostomy, eliminating bowel distention. This operation may be useful in patients with neurologic disorders associated with severe aerophagia.

Free fascia lata repair for a second recurrent congenital diaphragmatic hernia.

Repair of recurrent congenital diaphragmatic hernia (CDH) continues to be a difficult problem. Although several materials have been used to repair recurrent CDH, the ideal material has yet to be established. We report the successful use of an autologous free fascia lata graft to repair the diaphragm following a second recurrence of CHD. The fascia lata is one of the strongest fascia in the body and is easy to obtain without extremity functional loss even in children. This procedure is regarded as effective for the repair of recurrent CDH.

[Management of gastrointestinal malformations in children with asplenia syndrome].

Asplenia syndrome (AS) is a complex disorder that includes a variety of cardiovascular and gastrointestinal malformations (GMs), and its prognosis remains poor. We treated 40 AS patients between 2000 and 2010, 10 of whom underwent surgery to correct GMs. These comprised 7 hiatus hernias (HH), 1 intestinal malrotation (IM), 1 stomach volvulus, and 1 ompalocele. In this review, we consider the optimal surgical timing and methods of management, especially for HH and IM. Surgery was performed in 6 of 7 HH patients. Four of those underwent laparotomy, 1 thoracotomy, and 1 laparoscopy. The Toupet method or fixation of the His angle or hiatus closure was selected in each case. Because the stomach intruding into the mediastinum may cause pulmonary venous obstruction and respiratory impairment, surgical repair is preferably performed prior to Glenn surgery. Among the 9 IMs, 8 were nonrotational and required no treatment, and only 1 required surgical treatment. GMs in AS are difficult to manage because of the unique anatomy, meaning there are no fixed surgical techniques. Each patient must be carefully examined preoperatively, and the optimum surgical method selected. Close collaboration with cardiovascular surgeons is vital when determining the timing and method of surgery.

Ovarian fibroma with marked ascites and elevated serum levels of CA-125 in a young girl.

We report a case of ovarian fibroma with marked ascites and elevated serum CA-125 levels in a young girl. Ovarian fibromas are rare in children. They usually present as a solid mass and may be associated with ascites and elevated serum CA-125 levels. Because of their solid nature and these associations, they can be mistaken for a malignant tumor, resulting in unnecessary oophorectomy. Ovarian fibromas are benign neoplasms, and the prognosis is extremely good. Surgical management should be an ovarian-sparing tumor excision. Although uncommon in pediatric patients, ovarian fibromas should be included in the differential diagnosis of ovarian mass in children.

Totally laparoscopic management of choledochal cyst: Roux-en-Y Jejunojejunostomy and wide hepaticojejunostomy with hilar ductoplasty.

BACKGROUND: Cyst excision with hepaticojejunostomy is the treatment of choice for choledochal cyst. However, late complications after definitive surgery develop occasionally, including intrahepatic stones and cholangitis, because of bile stasis resulting from anastomotic stricture, intrahepatic bile duct stricture, and remnants of intrahepatic ductal dilatation. In type IV-A choledochal cysts in particular, biliary stricture is frequently observed around the hepatic hilum, and ductoplasty for stricture is necessary. In this article, we present our experiences with totally laparoscopic surgery comprising excision of the extrahepatic bile duct, Roux-en-Y jejunojejunostomy, and wide hepaticojejunostomy combined with hilar ductoplasty for choledochal cyst. METHODS: We performed totally laparoscopic surgery on 8 children with choledochal cyst between June 2009 and October 2010. One of them had undergone bile drainage through gallbladder laparoscopically for biliary perforation. Four patients (1 Ic and 3 IV-A cysts) had hepatic duct stricture around the hepatic hilum. Laparoscopic surgery comprising excision of the extrahepatic bile duct and wide Roux-en-Y hepaticojejunostomy with ductoplasty was performed by using four trocars. RESULTS: The operation was completed laparoscopically for all patients. The mean operation time was 390 minutes (range, 310-460). The mean postoperative stay was 8.4 days (range, 7-14). After surgery, the dilatation of the intrahepatic bile duct was remarkably reduced in size, and all patients are doing well. CONCLUSION: Laparoscopic surgery comprising excision of the extrahepatic bile duct, Roux-en-Y limb formation, and wide hepaticojejunostomy with hilar ductoplasty appears to be feasible for children with choledochal cyst. When there is a stricture near the confluence of the hepatic ducts, laparoscopic ductoplasty appears to be feasible for the surgeon with an advanced laparoscopic skill set.

Laryngotracheal separation using skin flap formation: a novel surgical procedure.

Laryngotracheal separation is often performed to prevent intractable aspiration for children with severe mental and physical disabilities. However, tracheo-innominate artery fistula after surgery can occur as a severe complication and cause fatal hemorrhage. We have therefore developed a novel procedure to prevent tracheo-innominate artery fistula. Surgery was performed by making an H-shaped incision and creating skin flaps. The trachea was not raised anteriorly and the skin flaps were sutured to the trachea. We performed this procedure on nine patients, and no major complications occurred in any case. This procedure can be expected to reduce the occurrence of tracheo-innominate artery fistula.

Laparoscopic splenopexy for wandering spleen: extraperitoneal pocket splenopexy.

Wandering spleen is a very rare disease. It is a very dangerous condition due to the risk of splenic ischemia from persistent pedicle torsion. Here, we describe a case of wandering spleen diagnosed by ultrasound and computed tomography scans in an 11-year-old boy who suffered from frequent urination and enuresis. A laparoscopic splenopexy was successfully performed with fixation of the spleen in an extraperitoneal pocket. Since the operation, the organ has remained in place with good perfusion. The details of the procedure are described.