Current trends and prospects of surgical techniques for hepatoblastoma.

Hepatoblastoma, a common extracranial malignant solid tumor in childhood, is often detected at an advanced stage and is difficult to treat surgically. Despite the availability of multiple comprehensive treatments that can be combined with surgery, hepatoblastoma treatment outcomes remain poor. Surgery is the main treatment strategy for hepatoblastoma, but it faces many challenges, including tumor attachment to surrounding tissues, tumor wrapping or invading of vital organs and tissues, the presence of giant or multiple tumors, distant metastasis, the formation of a tumor thrombus, and significant surgical trauma. In this review, we discuss recent research advances and propose potential strategies for overcoming these challenges. Such strategies may improve the rate of hepatoblastoma resection and local control in children, as well as reduce complications and trauma.

Baseline Sensitivity and Potential Resistance Mechanisms for Fusarium pseudograminearum to Fludioxonil.

Fusarium crown rot (FCR), which is caused by Fusarium pseudograminearum, is one of the most important diseases affecting wheat production in the Huanghuai wheat-growing region of China. Although the phenylpyrrole fungicide fludioxonil is known to have a broad-spectrum activity against a wide range of plant pathogens, including F. pseudograminearum, it has not yet been registered for the control of FCR in China, and further research is needed to assess the biological characteristics and molecular mechanisms associated with fludioxonil resistance, and especially the potential for highly resistant isolates to emerge. The current study demonstrated that the baseline fludioxonil sensitivity of 61 F. pseudograminearum isolates collected from the Henan province of China during the summers of 2019 to 2021 conformed to a unimodal distribution with a mean effective concentration for 50% inhibition (EC50) value of 0.021 ± 0.003 µg/ml, which indicated that none of the isolates exhibited natural resistance to fludioxonil. Nevertheless, four fludioxonil-resistant mutants were attained after repeated exposure to fludioxonil under laboratory conditions. All resistant mutants exhibited significantly lower growth rates on potato dextrose agar (PDA) and lower levels of sporulation and pathogenicity in wheat seedlings. In addition, the resistant mutants also exhibited less growth on PDA amended with either 0.5 M mannitol, 0.5 M glucose, 0.5 M MgCl2, or 0.5 M NaCl, which indicated that they had greater sensitivity to osmotic stress. Molecular analysis of the proposed fludioxonil target protein FpOs1 indicated that the predicted sequences of the resistant mutants contained none of the characteristic amino acid changes previously associated with fludioxonil resistance in other species. Further investigation via quantitative real-time PCR analysis revealed that expression of the FpOs1 gene was significantly altered in the resistant mutants in both the absence and presence of fludioxonil. Meanwhile, plate assays found evidence of cross-resistance between fludioxonil and cyprodinil, as well as with the triazole fungicides tebuconazole and difenoconazole, but not with other commonly used fungicides including prochloraz, fluazinam, and carbendazim. Taken together, these results provide new insights into the mechanism and biological characteristics associated with fludioxonil resistance in F. pseudograminearum and indicate that fludioxonil could provide effective and sustained control of FCR during wheat production.

Single-incision laparoscopic versus conventional laparoscopic endorectal pull-through for Hirschsprung's disease: a comparison of short-term surgical results.

BACKGROUND: Single-incision laparoscopy has recently become a popular procedure in pediatric surgery. The current series is the largest study to evaluate the operative complications and results of single-incision laparoscopic endorectal pull-through (SILEP) for the treatment of HD through a retrospective comparison with conventional laparoscopic endorectal pull-through (CLEP). METHODS: Twenty eight patients who underwent single-incision laparoscopic endorectal pull-through for HD between July 2010 and August 2011 were compared to thirty patients who underwent CLEP. Patient age, gender, transitional zone, operative time, blood loss, intraoperative and postoperative complications, as well as short-term results, were assessed. RESULTS: A total of 28 patients were included for SILEP, and historical reported 30 patients were completed by CLEP. The SILEP and CLEP groups were similar in regard to age, gender, transition zone, operative time, blood loss, hospital stay, and intraoperative complications. Postoperative results were not different, including equal daily defecation frequency and early postoperative complications. No patients with recurrent constipation were seen. Two patients with the transitional zone in the descending colon in the SILEP group had a 3mm trocar added in the left abdomen, and there were no conversions in the conventional laparoscopic group. CONCLUSION: We have shown that in selected HD patients, the SILEP technique was safe and technically feasible in experienced hands. It results in similar operative results compared with conventional laparoscopic endorectal pull-through. However, the operation is more difficult if the transition zone was higher than the rectosigmoid.

10 years of experience with laparoscopic-assisted endorectal Soave pull-through procedure for Hirschsprung's disease in China.

OBJECTIVE: To report early and late results of laparoscopic-assisted endorectal Soave pull-through (LAEPT) with a short and V-shaped partial resection muscular cuff for Hirschsprung's disease (HD) over a 10-year period. METHODS: The clinical courses of 218 patients who underwent modified LAEPT for HD were reviewed. LAEPT was described by the Georgeson technique. The main modifications included less dissection of the bottom of the pelvis, rectal mucosa dissection with a long cuff, coloanal anastomosis with a short cuff, and a V-shaped partial resection in the posterior wall of the muscular cuff. RESULTS: From September 1999 to July 2009, 218 patients were operated on by the same surgeon. Ages ranged from 15 days to 12 years old. The aganglionic segment was located in the rectum or sigmoid colon in 176 patients, in the left colon in 38 children, and in the transverse colon in 4 patients. The median operating time was 176 minutes. Conversion to open surgery was required in 2 patients. Bleeding of the left iliac vein occurred in 1 patient, and a 180° twist of the neorectum occurred in 2 cases. Median first bowel movement time was 23 hours, and median postoperative hospital stay was 10.4 days. Median daily defecation frequency was 4.6 within 2 weeks and 2.3 at 3 months postoperatively. The immediate postoperative complications included intestine herniation from the trocar site in 2 patients, perianal excoriation in 32 patients, and anastomotic leakage in 3 patients. Follow-up ranging from 6 to 120 months was obtained for 182 patients. Late postoperative complications included postoperative adhesive bowel obstruction (1.1%), enterocolitis (7.7%), anasomostic stenosis (2.2%), constipation (1.6%), and soiling (3.6%). Eighty-seven percent had excellent and good bowel function. CONCLUSION: LAEPT with a short muscular cuff with a V-shaped partial resection in the posterior wall is a safe and effective procedure for HD.

Transanal endorectal pull-through for Hirschsprung's disease using long cuff dissection and short V-shaped partially resected cuff anastomosis: early and late outcomes.

BACKGROUND: Transanal endorectal pull-through was described by De la Torre-Mondragon's technique. In the original transanal pull-through procedure, a long rectal muscular cuff was dissected and left for anocolic anastomosis, which would sometimes lead to postoperative obstructive symptoms and enterocolitis. While a modified short mucosal dissection may increase the risk of injury to pelvic and perirectal nerves and other structures when dissected on the outside of the rectum deep in the pelvis. We report early and late results of the modified transanal procedure for Hirschsprung's disease (HD) over 8 years. METHODS: The clinical course of all children with aganglionic rectum or sigmoid colon receiving the modified transanal pull-through between May 2003 and April 2011 were reviewed. The main modifications were rectal mucosa dissection with a long cuff, coloanal anastomosis with a short cuff and a V-shaped partial resection in the posterior wall of the muscular cuff. Children with preliminary stoma or total colonic disease were excluded. RESULTS: Short- and long-term follow-up was obtained in 137 patients with HD operated upon by the same surgeon. The aganglionic segment was located in the rectum or sigmoid colon in all patients. The mean age at surgery was 165 ± 74 days. The mean operating time was 108 ± 38 min. Mean intra-operative blood loss was estimated to be 15 ± 10 ml. No patient required a blood transfusion. Mean postoperative hospital stay was 7 ± 2 days. Early postoperative complications included perianal excoriation in 38 patients (27.7 %), enterocolitis in two patients (1.4 %), and anastomotic leak in two patients (1.4 %). Late postoperative complications included perianal excoriation in 16 patients (11.7 %), anal stricture in two patients (1.4 %), constipation in four patients (2.8 %), enterocolitis in 10 patients (7.3 %), and soiling problems in six patients (4.4 %). Mean follow-up was 56 months (6 months-9 years). In patients older than 4 years, 85.4 % of them had excellent/good bowel function, 9.4 % had fair bowel function, and 5.2 % of patients had bad bowel function. CONCLUSION: Transanal endorectal pull-through with a long cuff dissection and a short V-shaped resected cuff anastomosis is a safe and effective procedure for HD. It reduced incidence of anastomotic stricture and constipation without an increased soiling incidence.

Laparoscopically assisted anorectal pull-through for high imperforate anus in infants: intermediate results.

PURPOSE: The aim of this study was to evaluate the clinical outcomes and postoperative anal function in infants with congenital high imperforate anus treated with laparoscopically assisted anorectal pull-through (LAARP). METHODS: From January 2004 to July 2007, 33 patients (28 boys and 5 girls, age ranging from 3 to 10 months) with high imperforate anus underwent LAARP. Clinical data of the LAARP group were retrospectively compared with those treated by posterior sagittal anorectoplasty (PSARP; n = 28) during the same time period. Anorectal function of these patients was evaluated using the following 3 methods: the Kelly score, anorectal vector volume manometry, and magnetic resonance imaging between the ages of 3.1 and 4.4 years. RESULTS: The mean operative time in LAARP and PSARP groups was 112.5 ± 12.4 and 120.4 ± 18.5 minutes (P > .05), respectively. The mean length of hospital stay in the LAARP group was shorter than that of PSARP group (11.3 ± 2.1 vs 14.6 ± 2.3 days, P < .01). No significant difference was observed between LAARP and PSARP groups regarding the Kelly score (3.52 ± 1.42 vs 3.49 ± 0.82). Although magnetic resonance imaging revealed lower malposition rates of rectum in the LAARP group than those of the PSARP group at both I-line (3.0% vs 14.3%) and M-line (3.0% vs 10.7%) levels, this was not statistically different (P > .05). Compared with the PSARP group, lower asymmetric index, larger vector volume, and higher anal canal pressure at rest and during voluntary squeeze were observed in LAARP group (P < .05). However, there were no significant differences in the length of high-pressure zone (15.2 ± 5.8 vs 15.1 ± 6.2 mm) and the presence of rectoanal relaxation reflex (84.8% vs 85.7%). CONCLUSIONS: Satisfactory fecal continence can be achieved in patients with high-type imperforate anus after LAARP. Laparoscopically assisted anorectal pull-through has advantages over PSARP, including shorter hospital stay and better position of rectum. However, long-term follow-up is necessary to compare the benefits of LAARP against PSARP.

Laparoscopic choledochal cyst excision, hepaticojejunostomy, and extracorporeal Roux-en-Y anastomosis: a technical skill and intermediate-term report in 62 cases.

BACKGROUND: This study aimed to present the authors' technique and the intermediate-term outcome for laparoscopic choledochal cyst excision with Roux-en-Y hepatoenterostomy. METHODS: This retrospective study investigated 62 children (39 girls and 23 boys) who had undergone laparoscopic resection of choledochal cyst. The average age of the children was 2.3 years. The retrospective data and the following investigations about type of choledochal cyst, surgical technique, conversion rate, morbidity, and mortality were analyzed. RESULTS: Of the 62 patients, 43 (69.4%) showed type 1a choledochal cysts, 16 (25.8%) showed type 1c, 2 (3.2%) showed type 4a, and 1 (1.6%) showed type 4b. Total cyst excision could be performed for 51 of the patients (82.3%). The large cysts were opened on the front wall, then divided circumferentially in 29 cases. The small cysts did not need to be opened before excision in 22 cases. For 11 patients (17.7%), Lilly's (Surg Gynecol Obstet 146:254-256, 1978) technique was adopted, and for 5 patients with a huge cyst, the duodenum together with the head of the pancreas had to be mobilized for visualization of the cyst's lower limit. The hepatic duct was excised, and plastic operation of bile duct was performed for two patients. The mean operative time was 226±41.2 min. Eight patients needed blood transfusion, and conversion was required for one patient. The mean hospital stay was 8±1.5 days, and the mean follow-up period was 38 months. The overall morbidity rate was 8.2% (5/61) including bile leakage (n=1), adhesive small bowel obstruction (n=1), intestinal necrosis (n=1), and cholangitis (n=1). Inflammatory edema anastomotic narrowing occurred in one patient. None of the patients needed surgery due to anastomotic stricture. CONCLUSIONS: Laparoscopic choledochal cyst excision, hepaticojejunostomy, and extracorporeal Roux-en-Y anastomosis can be safely and quickly performed for children, with satisfactory intermediate-term results. Extracorporeal Roux-en-Y anastomosis could shorten the operative time.

Endoscopic transaxillary approach for congenital muscular torticollis.

BACKGROUND: Surgical correction of the congenital muscular torticollis (CMT) is recommended for patients with unsuccessful conservative treatment. Open operative techniques all leave noticeable scars. We proposed a modified endoscopic 1-trocar transaxillary and neck microincision approach for the treatment of CMT. METHODS: Endoscopic release of the sternocleidomastoid (SCM) muscle was performed in 45 infants and children aged 6 months to 15 years. One 5-mm incision was made in the anterior axillary fold, and a subcutaneous tunnel over the clavicular and sternal heads of the SCM muscle was made. A subcutaneous space was established by CO2 inflation at a pressure of 8 mm Hg and then endoscopically using a 5-mm endoscope. Two additional 1.5- to 2-mm supraclavicular mini-incisions were made beside the SCM muscle for the introduction of miniforceps and electrocautery, respectively. The sternal and clavicular attachments were dissected and divided by electrocautery. Clinical evaluation was performed using the Lee scoring system. RESULTS: The operation was successfully completed endoscopically in all 45 children. The mean operative time was 40 minutes. No injuries of major blood vessels or nerves were encountered. A small bleed was noted in 1 child owing to reoperation. Follow-up for 6 months to 3 years in 42 patients showed complete muscular release and satisfactory cosmetic appearance with no recurrence. The results were classified as excellent in 88.1% (37/42), good in 9.5% (4/42), fair in 2.4% (1/42), and poor in 0 using the Lee scoring system. The neck scars were not visible 1 month after the procedure. CONCLUSIONS: The subcutaneous endoscopic transaxillary and micro-neck incision approach for the treatment of CMT is a safe, practical procedure that provides good functional and cosmetic outcomes without vascular or neural injury.

Laparoscopic extensive colectomy with transanal Soave pull-through for intestinal neuronal dysplasia in 17 children.

BACKGROUND: Open colectomy has been preferred for intestinal neuronal dysplasia type B (IND) due to its low morbidity rate and good functional results. The aim of this study was to investigate the feasibility and results of laparoscopic colectomy with transanal Soave pull-through for the treatment of IND in children. METHODS: Seventeen infants and children suffering from IND were treated by laparoscopic extensive colectomy with transanal Soave pull-through. The diagnosis of IND was made via anorectal manometry, X-ray contrast enema, suction biopsies, and laparoscopic full-thickness biopsies with hematoxylin-eosin staining. The technique used four or five abdominal ports. The sigmoid, transverse, and right colon up to the last ileal cove were mobilized laparoscopically in the extended form of IND. A modified Soave's anastomosis was performed. The patients' data, surgical procedures, operative data, postoperative complications and clinical outcomes were analyzed. RESULTS: Five patients underwent laparoscopic left colectomy with modified transanal Soave procedures, and the other 12 were treated by laparoscopic subtotal colectomy and required a Deloyers' maneuver for the Soave pull-through. The proximal margin of barium stagnation in patients with left colectomy was restricted to the distal end of the descending colon, sigmoid colon, and that in patients with subtotal colectomy was restricted to the proximal end of the descending colon, transverse colon, hepatic flexure, and ascending colon. Postoperative complications included anastomotic leakage, severe perianal erosions, postoperative enterocolitis, and soiling. During a mean follow-up of 4 years, bowel frequency was 4-10 times per day in 3 months postoperatively in patients with subtotal colectomy. The clinical results were good, with no stool incontinence or constipation. CONCLUSIONS: Laparoscopic procedure for left colectomy and subtotal colectomy with transanal Soave pull-through in infants and children with IND is safe, feasible, and effective. The location of barium stagnation in proximal margin may be used as a method to predict initially the proximal margin of the resected bowel segment.

The evaluation of laparoscopy-assisted cholangiography in the diagnosis of prolonged jaundice in infants.

BACKGROUND: Biliary atresia (BA) is the progressive inflammatory obstruction and fibro-obliteration of all or part of the extrahepatic biliary tree and the intrahepatic bile ducts and has its onset exclusively within the first several months of life. This study was undertaken to present the value of diagnostic laparoscopy in infants with prolonged jaundice and technique for laparoscopic cholangiography. METHODS: A 5-mm umbilical trocar was introduced to create a port for a 30-degree laparoscope. If the gallbladder was of good size, the fundus was exteriorized through the right subcostal trocar site and a catheter was inserted into the gallbladder for cholangiography, following partial dissection from the liver bed, if required. If the gallbladder was atretic, the fundus was not exteriorized and a laparotomy was performed and cholangiography was abandoned, because the lumen of an atretic gallbladder was usually not fully patent. RESULTS: At laparoscopy, 12 patients had good-sized gallbladders and minimal-to-mild liver fibrosis. They underwent cholangiography via the exteriorized fundus, and infantile hepatitis syndrome (HIS) or cholestatic syndrome (CS) in 8 cases, BA in 2 cases, and biliary hypoplasia (CBDH) in 2 cases were identified. Five patients' gallbladders dissected from the liver bed underwent cholangiography, and BA in 3 cases and CBDH in 2 cases were identified. The remaining 21 had atretic gallbladders and varying degrees of liver fibrosis, so cholangiography via the exteriorized fundus was abandoned and converted to open Kasai portoenterostomy. CONCLUSIONS: Laparoscopy-assisted cholangiography is a simple, accurate, and safe method in the diagnosis of prolonged jaundice in infants and allows the anatomic structure of the biliary tree to be obtained accurately with minimal surgical intervention.

Clinical value of pelvic 3-dimensional magnetic resonance image reconstruction in anorectal malformations.

OBJECTIVE: The study aimed to build a 3-dimensional (3D) reconstruction of pelvic magnetic resonance images and evaluate the clinical value in anorectal malformations (ARMs). METHODS: Magnetic resonance imaging (MRI) examinations were performed on a 1.5-T magnet. Sagittal, coronal, and transverse turbo spin-echo T1-weighted and fast spin-echo T2-weighted images of the pelvic region were obtained in 22 children. A 3D reconstruction was made on a computer and assisted by the 3D-Doctor software (Trial Version, Able Software Corp). The level and type of ARM and the developmental state of the striated muscle complex (SMC) were analyzed with 3D reconstruction image. RESULTS: The 3D images of the pelvic were confirmed in 22 cases. Three-dimensional reconstructed images perfectly displayed the anatomical relationships of the SMC and the rectal atresia in these spaces. The 3D configuration of the SMC was different in each of the high- and low-type cases. The high-type malformation of SMCs differed particularly from the descriptions. CONCLUSIONS: Pelvic magnetic resonance 3D reconstructed images were able to show the dimensional anatomical relations of pelvis, bladder, urethra, rectum, and SMC. Both a 3D image and positional information with MRI offers the surgeon a simulated operative profile of the SMC superior to MRI slices alone, which will help in providing morphological data for image diagnosis and operation of the ARM.

Expression and clinical significance of stem cell marker CD133 in human neuroblastoma.

BACKGROUND: Recent evidences indicate that CD133, a kind of transmembrane protein, can be used as a marker to isolate stem cells from tumors originating from neural crest. This study was undertaken to explore the expression and clinical significance of stem cell marker CD133 in neuroblastoma (NB). METHODS: Immunohistochemical staining was used to detect the expression of CD133 in 32 patients with NB and 8 patients with ganglioneuroblastoma (GNB). The relationships were analyzed among CD133 expression, international neuroblastoma staging system (INSS) stages, pathological classification, and postoperative survival time of NB patients. RESULTS: The expression rates of CD133 in NB and GNB were 46.9% (15/32) and 37.5% (3/8) respectively, mainly in cytoplasm of neuroblastoma cells. The expression rates of stage 1-2, stage 3-4 and stage 4S were 30.7%, 57.9% and 37.5%, respectively. The differences in various stages were significant (P<0.05). The positive rate of CD133 in patients with unfavorable histology (52.4%) was significantly higher than that in patients with favorable histology (36.8%) (P=0.007). The survival time of CD133 negative patients was significantly longer than that of CD133 positive patients (P=0.026). CONCLUSIONS: CD133 which might be correlated with the development and progression of NB can serve as one of the important indicators for prognosis of NB.

Diagnosis and treatment of biliary atresia: a retrospective study.

BACKGROUND: Biliary atresia (BA) is the end results of an inflammatory process, which affect the intrahepatic and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tract with the development of biliary cirrhosis. Early diagnosis of BA is difficult, and there is no specific therapy for BA at present. The purpose of this study was to investigate the diagnosis, treatment and postoperative outcome of BA and to explore new diagnostic and therapeutic strategies. METHODS: Thirty-one children with biliary atresia from our hospital and 201 children with such disease from other hospitals in China were reviewed retrospectively in terms of diagnostic modalities, operative age, operative methods and long-term survival rates after operation. RESULTS: The operative age of the 31 patients varied from 13 to 270 days. It was <60 days in 6 children (19%), 61-90 days in 14 (45%), 91-120 days in 8 (26%), and >120 days in 3 (10%). No children underwent transplantation for BA. Their 1-6 year survival rate on average was 32.3% (10/31). The early operative rate in China was 12.9% (30/232). One child received liver transplantation with a long-term survival rate of 31.9%. CONCLUSIONS: Early recognition of babies with BA is critical for optimal intervention for preventing progressive fibrosis. Clinical presentation, imaging and laparoscopy are helpful in enhancing the early diagnostic rate of BA patients. The long-term survival rates can be obtained after the improvement of operative methods, liver transplantation for children with advanced BA, and development of other medical strategies.

[The genetic analysis of a family with syndactyly and adactylism].

A family with syndactyly and adactylism was reported in this paper. There are four sufferers, suffering from syndactyly and adactylism, with the lack of metacarpus and metatarsus in two generations. According to genetic analysis, this disease is caused by autosomal dominant inheritance.