Efficacy of Bleomycin-Lauromacrogol Foam in Pediatric Macrocystic Lymphatic Malformations With and Without Intracapsular Hemorrhage.

BACKGROUND: Sclerotherapy is purportedly less effective in patients with hemorrhagic than with non-hemorrhagic lymphatic malformations (LMs). We aimed to compare the efficacy of bleomycin-lauromacrogol foam (BLF) sclerotherapy in the treatment of macrocystic LMs with and without intralesional hemorrhage. METHODS: Fifty-five children with macrocystic LMs admitted to the Pediatric Surgery Department were retrospectively included. The patients were allocated into a hemorrhage group (23 cases) or a non-hemorrhage group (32 cases) based on the occurrence of an intracapsular hemorrhage. The diagnosis was confirmed by physical examination, color ultrasound, magnetic resonance imaging, and puncture findings. BLF was injected into the capsule after draining the cystic fluid under color ultrasound guidance. Patients whose lesions were unchanged or showed minor change after 1 month were treated again using the same method. Changes in lesion size and the number of treatments were recorded. Effectiveness was classified as excellent (volume reduction ≥90%), good (50%≤volume reduction<90%), or poor (volume reduction <50%). RESULTS: In the hemorrhage group, 17, 6, and 0 patients' outcomes were classified as excellent, good, and poor, respectively. The overall efficacy rate was 100%. In the non-hemorrhage group, 23, 7, and 2 patients' outcomes were classified as excellent, good, and poor, respectively. The overall efficacy rate was 93.8%. There was no significant difference in efficacy rate between groups (P = 0.767). CONCLUSIONS: BLF is an effective and safe treatment for macrocystic LMs with bleeding. The results were similar in patients with and without bleeding. LEVEL OF EVIDENCE: Treatment, Level III.

Laparoscopic approach for managing intussusception in children: Analysis of 65 cases.

BACKGROUND: Intussusception can be managed by pneumatic reduction, ultrasound-guided hydrostatic reduction, open or laparoscopic surgery, but laparoscopy in such cases remains controversial. AIM: To explore the clinical characteristics, effectiveness, and complications of surgical reduction for intussusception using laparoscopy in children. METHODS: This study was a retrospective case series of pediatric patients with intussusception who underwent surgical reduction by laparoscopy from May 2011 to April 2016 at Taizhou Hospital of Zhejiang Province. Clinical characteristics (operation time, intraoperative blood loss, conversion rate of laparotomy, reasons for conversion, postoperative hospital stay, and adverse events) were described. RESULTS: The 65 patients included 45 boys and 20 girls. The average age was 2.3 years (27.5 ± 24.5 mo). Of the 65 patients, 61 underwent surgical reduction by laparoscopy after a failed enema reduction of intussusception, and four underwent the procedure directly. All patients were treated successfully and 57 (87.7%) patients underwent successful laparoscopic surgery, two of which had a spontaneous reduction. Among the remaining cases, one was converted to open surgery via right upper quadrant incision, and seven required enlarged umbilical incisions. Intestinal resection was performed in 5 patients because of abnormal bowel lesions. There were no complications (intestinal perforations, wound infections, or intestinal adhesions) during the follow-up of 3 years to 8 years. Two patients experienced a recurrence of intussusception; one was resolved with pneumatic reduction, and the other underwent a second laparoscopic surgery. CONCLUSION: Laparoscopic approach for pediatric intussusception is feasible and safe. Bowel resection if required can be performed by extending umbilical incision without the conventional laparotomy.

Single-Site Umbilical Laparoscopic Pyloromyotomy Using a Pyloric Electrocoagulation Chisel Combined with a Left-Handed Main Operation for Congenital Hypertrophic Pyloric Stenosis.

Background: Laparoscopic pyloromyotomy has become a gold standard for the treatment of congenital hypertrophic pyloric stenosis (HPS). There have been recent reports on the use of transumbilical single-site laparoscopic surgery for congenital HPS; however, using transumbilical single-site laparoscopic surgery in pediatric cases is still controversial due to the difficulty with manipulation. In this study, some preliminary experience with the application of a novel transumbilical single-site laparoscopic approach in congenital HPS is described. Methods: A retrospective study was conducted involving 25 patients with congenital HPS treated in our hospital from August 2016 to August 2019. A pyloric electrocoagulation chisel combined with a left-handed main operation was completed in all of the patients and the operative times, postoperative length of stay, and operative complications were recorded. Results: The laparoscopic operation was completed in 25 patients with an average operative time of 21.9 ± 5.5 minutes, average postoperative length of stay of 2.5 ± 0.9 days, and no perforations of the pyloric mucosa, recurrent obstruction, surgical incision infections, and incision hernias. All of the patients had at least 3 months of follow-up, good growth and development, and the parents were satisfied with the postoperative scars. Conclusion: A pyloric electrocoagulation chisel combined with a left-handed main operation in the treatment of congenital HPS by a single-site umbilical laparoscopic pyloromyotomy is safe and effective, and can achieve a satisfactory cosmetic effect.

The Application of Pyloric chisel in the Treatment of Hypertrophic Pyloric Stenosis by Single-Site Umbilical Laparoscopic Pyloromyotomy.

OBJECTIVE: The feasibility and perspective of pyloric chisel were discussed through the comparison of pyloric chisel and knife in the treatment of hypertrophic pyloric stenosis (HPS) in single-site umbilical laparoscopic pyloromyotomy (SSULP). METHODS: Fifty-eight cases of HPS treated in our hospital from February 2011 to March 2016 were retrospectively analyzed, in which 30 patients underwent pyloric chisel (Pyloric chisel Group) and 28 patients underwent knife (Knife Group). Operative time, estimated blood loss, and complications between the two groups were analyzed. RESULTS: The operative time was shorter in Pyloric chisel Group than Knife Group (P < .05). The estimated blood loss was lower in Pyloric chisel Group than Knife Group (P < .05). The complication was less in Pyloric chisel Group than Knife Group (P < .05). There were 2 cases of mucosal perforations requiring conversions to open in Knife Group. Five cases of serous tearing occurred in the Knife Group. There was 1 case of serous tearing in the Pyloric chisel Group. All patients were followed up for 3 months, and there was no distinct scar in the umbilical. CONCLUSIONS: Patients were satisfied with no distinct scars in abdominal wall by pyloric chisel or knife to treat HPS in SSULP, but pyloric chisel is more effective and safer.

RET 3'UTR polymorphisms and its protective role in Hirschsprung disease in southeastern Chinese.

BACKGROUND: Hirschsprung disease (HSCR) is a complex congenital disorder characterized by intestinal obstructions owing to the absence of the intestinal ganglion cells of the nerve plexuses in variable lengths of the digestive tract. Several RET polymorphisms and haplotypes have been described as underrepresented in HSCR patients with respect to controls. We thus sought to investigate whether polymorphisms in RET 3'UTR are associated with isolated HSCR in the Chinese population. METHODS: Polymerase chain reaction amplification and direct sequencing were used to screen polymorphisms in RET 3'UTR in patients with sporadic HSCR and ethnically matched controls in Han Chinese populations. Association tests of RET 3'UTR variants and haplotypes with HSCR were performed. RESULTS: We examined a total of 107 Chinese sporadic HSCR patients and 89 ethnically matched controls by sequencing the 3'UTR of the RET gene. Five single nucleotide polymorphisms (SNPs) and 2 monomorphic SNPs were identified. The genotype distributions and the allele frequencies of the 5 SNPs were significantly different between HSCR cases and controls and occurred more frequently in the control population. Haplotype analysis has shown a higher frequency of haplotypes comprising variant alleles in controls as compared with cases. CONCLUSIONS: The significant deviations of the genotype distributions and the allele frequencies of these SNPs in the HSCR population compared with the control population demonstrate that these SNPs have a strong negative association with HSCR and could act as protective alleles.

Association analysis of the RET proto-oncogene with Hirschsprung disease in the Han Chinese population of southeastern China.

Hirschsprung disease (HSCR) is a complex congenital disorder characterized by intestinal obstructions caused by the absence of the intestinal ganglion cells of the nerve plexuses in variable lengths of the digestive tract. This study investigated a possible role of the RET proto-oncogene in sporadic HSCR patients in the Han Chinese population. Our results indicated that rs1800858, rs1800860, rs1800863, and rs2075912, located in exons 2, 7, 15, and intron 19 of RET, are strongly associated with the disease (P < 0.01), with rs1800860 and rs1800863 playing a protective role in the pathogenesis of HSCR in the Chinese population. We also showed that the haplotype consisting of four SNPs is significantly associated with HSCR. We did not find a significant difference in the CA-repeat in intron 5 of RET between cases and controls. Our study provided further evidence that the RET gene is involved in the susceptibility to HSCR in the Han Chinese population.

Association analysis of the PHOX2B gene with Hirschsprung disease in the Han Chinese population of Southeastern China.

BACKGROUND: Hirschsprung disease (HSCR, OMIM 142623) is a complex congenital disorder characterized by intestinal obstructions caused by the absence of the intestinal ganglion cells of the nerve plexuses in variable lengths of the digestive tract. The PHOX2B gene is involved in neurogenesis and disruption of Phox2b in mice results in a HSCR-like phenotype. The first association study of the PHOX2B gene with HSCR derived from Chinese population in Hong Kong; here, we address the question of whether PHOX2B acts as a predisposing factor in HSCR pathogenesis in Chinese population in mainland. METHODS: To investigate the contribution of PHOX2B to the HSCR phenotype, polymerase chain reaction amplification and direct sequencing were used to screen PHOX2B coding regions and intron/exon boundaries for mutations and polymorphisms in 102 patients with HSCR and 96 ethnically matched controls, in Han Chinese populations of Southeastern China. RESULTS: In this study, we genotyped 4 single nucleotide polymorphisms (SNPs) (including 1 novel SNP) located within the PHOX2B gene. Statistically significant differences were found for c.701 A > G and IVS2 + 100 A > G, and the log-additive model was accepted as the best inheritance model (odds ratio [OR], 1.79; 95% confidence interval [CI], 1.11-2.87) for IVS2 + 100 A > G. We also showed that the haplotype-A G A N composed of 4 SNPs exhibited significant association with the disease (P = .03); this haplotype was more frequently observed in cases than in controls (OR, 2.31; 95% CI, 1.11-4.82). CONCLUSIONS: Our study provided further evidence that the PHOX2B gene is involved in the susceptibility to HSCR in the Han Chinese population. Our findings are in accordance with the involvement of PHOX2B in the signaling pathways governing the development of enteric neurons.