Use of a new vertical traction device for early traction-assisted staged closure of congenital abdominal wall defects: a prospective series of 16 patients.

PURPOSE: Abdominal wall closure in patients with giant omphalocele (GOC) and complicated gastroschisis (GS) remains to be a surgical challenge. To facilitate an early complete abdominal wall closure, we investigated the combination of a staged closure technique with continuous traction to the abdominal wall using a newly designed vertical traction device for newborns. METHODS: Four tertiary pediatric surgery departments participated in the study between 04/2022 and 11/2023. In case primary organ reduction and abdominal wall closure were not amenable, patients underwent a traction-assisted abdominal wall closure applying fasciotens®Pediatric. Outcome parameters were time to closure, surgical complications, infections, and hernia formation. RESULTS: Ten patients with GOC and 6 patients with GS were included. Complete fascial closure was achieved after a median time of 7 days (range 4-22) in GOC and 5 days (range 4-11) in GS. There were two cases of tear-outs of traction sutures and one skin suture line dehiscence after fascial closure. No surgical site infection or signs of abdominal compartment syndrome were seen. No ventral or umbilical hernia occurred after a median follow-up of 12 months (range 4-22). CONCLUSION: Traction-assisted staged closure using fasciotens®Pediatric enabled an early tension-less fascial closure in GOC and GS in the newborn period.

[Spectrum of Minimally Invasive Surgical Techniques for the Managment of Inguinal Hernias in Infants and Children]. / Spektrum der minimalinvasiven Operationstechniken zur Versorgung von Leistenhernien bei Säuglingen und Kindern.

One of the most common surgical procedures in infants and children is the repair of an indirect inguinal hernia. This can be carried out by open technique or using minimally invasive surgery (MIS). Since 1998, numerous different MIS techniques have been described. Scientifically proven advantages include a shorter operation time for bilateral hernias, along with a lower risk of metachronal, contralateral hernia. Nevertheless, the proportion of inguinal hernias treated using MIS in children in this country is relatively low, at around 8% of all operations. The aim of this synopsis is to describe the different MIS techniques for inguinal hernia repair in children, including their respective advantages and disadvantages.This video compilation shows the spectrum of different techniques for laparoscopic inguinal hernia repair in children. It includes the intracorporeal suturing technique, the incision of the peritoneum, extracorporeal percutaneous techniques, and the cauterisation of the open peritoneal vaginal process in girls.Although minimally invasive inguinal hernia repair in children is technically and scientifically established, it is not yet being widely used. This video manuscript provides an overview of the various techniques, thus facilitating clinical application.

CHARGE syndrome and related disorders: a mechanistic link.

CHARGE syndrome is an autosomal dominant malformation disorder caused by pathogenic variants in the chromatin remodeler CHD7. Affected are craniofacial structures, cranial nerves and multiple organ systems. Depending on the combination of malformations present, its distinction from other congenital disorders can be challenging. To gain a better insight into the regulatory disturbances in CHARGE syndrome, we performed RNA-Seq analysis on blood samples of 19 children with CHARGE syndrome and a confirmed disease-causing CHD7 variant in comparison with healthy control children. Our analysis revealed a distinct CHARGE syndrome pattern with downregulation of genes that are linked to disorders described to mimic the CHARGE phenotype, i.e. KMT2D and KDM6A (Kabuki syndrome), EP300 and CREBBP (Rubinstein-Taybi syndrome) and ARID1A and ARID1B (Coffin-Siris syndrome). Furthermore, by performing protein-protein interaction studies using co-immunoprecipitation, direct yeast-two hybrid and in situ proximity ligation assays, we could demonstrate an interplay between CHD7, KMT2D, KDM6A and EP300. In summary, our data demonstrate a mechanistic and regulatory link between the developmental disorders CHARGE-, Kabuki- and Rubinstein Taybi-syndrome providing an explanation for the overlapping phenotypes.

Absence of lymphatic vessels in term placenta.

BACKGROUND: There has been debate about the existence of lymphatic vessels in placenta. Lymphatic endothelial cell (LEC) markers such as LYVE-1 and podoplanin/D2-40 have been found, although PROX1 has not been detected. The most reliable marker for LECs is the double staining for CD31 and PROX1, which has not been performed yet. METHODS: We studied three term placentas and dissected them into three areas: i.) basal plate area, ii.) intermediate area, and iii.) chorionic plate area. We used immunofluorescence single and double staining with antibodies against CD31, PROX1, LYVE-1, VEGFR-3, D2-40/PDPN, CD34, CCBE-1, and vimentin, as well as nested PCR, qPCR, Western blot and transmission electron microscopy (TEM). RESULTS: At TEM level we observed structures that have previously mistakenly been interpreted as lymphatics, however, we did not find any CD31/PROX1 double-positive cells in placenta. Absence of PROX1 was also noted by nested PCR, qPCR and Western blot. Also, LEC marker VEGFR-3 was expressed only in a small number of scattered leukocytes but was absent from vessels. The LEC marker D2-40/PDPN was expressed in most stromal cells, and the LEC marker LYVE-1 was found in a considerable number of stromal cells, but not in endothelial cells, which were positive for CD31, CD34, CCBE-1 and vimentin. Additionally, vimentin was found in stromal cells. CONCLUSIONS: Our studies clearly show absence of lymphatics in term placenta. We also show that the functional area of the mother's endometrium is not penetrated by lymphatics in term pregnancy.

Sonography-guided hydrostatic reduction of ileocolic intussusception in children: analysis of failure and success in consecutive patients presenting timely to the hospital.

UNLABELLED: In children with ileocolic intussusception sonography is increasingly being used for diagnosis, whereas fluoroscopy is frequently used for guiding non-invasive reduction. This study assessed the success rate of radiation-free sonography-guided hydrostatic reduction in children with ileocolic intussusception, using novel well-defined success rate indices. All children were evaluated who presented from 2005 to 2013 to the local university hospital with ileocolic intussusception. The patients were treated with sonography-guided hydrostatic reduction unless primary surgery was clinically indicated. The according success rate was determined by indices of Bekdash et al. They represent the ratio of persistently successful non-surgical reductions versus four different denominators, depending on including/excluding cases with primary surgery and including/excluding cases requiring bowel resection/intervention. Fifty-six consecutive patients were included (age, 3 months to 7.8 years). About 80% of the patients presented until 24 h and 20% until 48 h after the onset of symptoms. Seven patients underwent primary surgery, with bowel resection required in three cases. Hydrostatic reduction was attempted in 49 patients, being permanently successful in 41 cases (selective reduction rate 41/49 = 83.7%; crude reduction rate 41/56 = 73.2%). The remaining eight patients underwent secondary surgery, with just two patients not requiring surgical bowel resection/intervention (corrected selective reduction rate 41/43 = 95.3%). The composite reduction rate was 87.2% (successful/feasible reductions, 41/47). CONCLUSION: Radiation-free sonography-guided hydrostatic reduction has a good success rate in children with ileocolic intussusception. It may be particularly valuable in centers that are already experienced with using sonography for the diagnosis.

Robotic Anxiety-Parents' Perception of Robot-Assisted Pediatric Surgery.

In contrast to many other countries, robot-assisted (RA) pediatric surgery is not yet very common in Germany. Although the first pediatric RA intervention was published in 2001, RA pediatric surgery is still perceived as a "new technology". As a consequence, little is known about parents' perception of this operation method. In this study, we analyzed parents' intention to let their child undergo RA and laparoscopic (LA) surgery. Two subsamples (online and at the University Medical Center Goettingen) received a questionnaire addressing attitude towards RA and LA pediatric surgery with the help of a case example. Results showed that parents had a higher intention to consent to LA surgery. Perceiving more benefits, assuming a positive attitude of the social environment, and feeling less anxiety increased intention. A mediation analysis indicated that the type of surgery affected intentions through assumed attitude of the social environment. Exploratory analyses showed that the perception of risks and anxiety reduced intention for only RA surgery. These findings should be considered in preoperational discussions with parents. Anxiety and perceived risks should especially be addressed in order to encounter hesitancy.

Splenic torsion with involvement of pancreas and descending colon in a 9-year-old boy.

Splenic torsion is an uncommon condition becoming clinically apparent when the spleen twists or rotates around the organ's vascular pedicle. In the case of a wandering spleen the organ is only attached to an elongated vascular pedicle while the peritoneal attachments are absent. However, splenic torsion could also occur in patients with abnormal laxity of the splenic peritoneal attachments. We report a case of a splenic torsion due to absence of splenic ligaments with pancreatic volvulus and partial involvement of descending colon in a 9-year-old boy.

Successful use of recombinant factor VIIa in a preterm infant with life-threatening haematuria.

We report the case of a preterm male infant with a gestational age of 28 + 1 weeks and birth weight of 715 g who presented with life-threatening haematuria on day 28 of life. The haematuria was unresponsive to administration of platelet concentrates and fresh frozen plasma, but then successfully treated with recombinant factor VIIa. The resulting obstructive uropathy was managed by continuous bladder irrigation through suprapubic and urethral catheters. No other adverse affects were noted, and the boy was discharged from the hospital on day 108 of life.