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Absent or reversed end diastolic flow (AREDF) in umbilical artery (UA) Doppler are known phenomena during fetal interventions such as fetal spina bifida (fSB) repair. The clinical importance of these Doppler findings and the impact on children's outcome is not yet clarified.Between 2010 and 2019, 132 patients underwent SB repair at our center. The group with AREDF-UA was compared to the group with normal UA Doppler during the intervention. Primary endpoint was the FIGO-Score of the cardiotocography (CTG) 1, 2, and 6 hours postoperatively and their correlation to the ARED-UA flow. Secondary endpoints were the neonatal parameters and two-year neurodevelopmental outcome assessed using the Bayley Scale of Infant and Toddler Development Third Edition (Bayley-III).None of the fetuses showed AREDF before SB repair. In 107 patients (82%) normal UA-Doppler and in 23 (18%) AREDF-UA was observed during or immediately after SB surgery. AREDF-UA was more often observed after version of the fetus (p = 0.045). 17 (13 %) cases showed AEDF-UA and 6 (5%) cases REDF-UA. On the first day after SB surgery the AREDF-UA disappeared in all 23 cases. No significant difference in the CTGs at 1, 2, and 6 hours postoperatively and on the first postoperative day between the two groups was found (p 0.05). GA at delivery, UA-pH, 5-minute Apgar and birth weight were comparable between both groups and there was no difference regarding the two-year neurodevelopmental outcome (p0.05). Also the neurodevelopmental outcome did not differ between children with an absent or a reversed EDF-UA.The postoperative CTGs after a short-term phase of AREDF-UA during fSB repair normalize similarly as in normal UA-Doppler cases. AREDF-UA during fSB repair did not negatively influence postnatal or 2-year neurodevelopmental outcome compared with a normal UA-flow. This article is protected by copyright. All rights reserved.

Introduction: In select cases, in utero surgery for myelomeningocele (MMC) leads to better outcomes than postnatal repair. However, maternal HIV infection constitutes a formal exclusion criterion due to the potential of vertical HIV transmission. Encouraged by a previous case of a successful fetal spina bifida repair in a Hepatitis Bs antigen-positive woman, a plan was devised allowing for fetal surgery. Case Report: In utero MMC repair was performed although the mother was HIV-infected. To minimize the risk of in utero HIV transmission, the mother was treated by highly active antiretroviral therapy throughout gestation as well as intravenous zi­dovudine administration during maternal-fetal surgery. The mother tolerated all procedures very well without any sequelae. The currently 20 month-old toddler is HIV negative and has significantly benefitted from fetal surgery. Discussion/Conclusion: This case shows that maternal HIV is not a priori a diagnosis that excludes fetal surgery. Rather, it might be a surrogate for moving towards personalized medicine and away from applying too rigorous exclusion criteria in the selection of candidates for maternal-fetal surgery.

Increased pulse wave reflection in the fetal arterial system, illustrated by a second systolic peak (M-sign) in middle cerebral artery (MCA) Doppler waveforms, allows interpretation of fetal systemic vasoconstriction. Little is known about fetal vascular regulation during fetal spina bifida (fSB) repair. Therefore, the aim of this study was to analyze MCA-Doppler waveform changes before, during, and after fSB repair.31 pregnant women who underwent fSB repair were included. Fetal MCA-Doppler waveforms were prospectively analyzed before, during and after fSB repair, and categorized as follows: normal systolic downslope, systolic shoulder, second systolic peak (M-sign), and concave systolic downslope. These MCA waveforms were related to maternal and fetal characteristics, to anesthetic medication, and to umbilical artery (UA) waveforms.Before fSB repair, all fetuses repeatedly presented M-signs. After initiation of desflurane for general anesthesia, systolic shoulder and the M-sign vanished in 24/31 (78%) fetuses and 19/31 (61%) showed transient UA ARED flow. A significant association between these two Doppler findings was found (p=0.007). After fSB repair, signs of increased pulse wave reflection reappeared but resolved over time (23 days ± 20, SD) in all fetuses.Both fSB and intrauterine repair influence fetal vascular regulation. This phenomenon can be illustrated by MCA-Doppler waveforms. While anesthetic agents transiently eliminated M-signs and often provoked a UA ARED flow, fSB repair finally led to normalization of MCA-Doppler waveforms indicating return to normal fetal vascular regulation.Eine erhöhte Pulswellenreflektion im fetalen Kreislauf, im Sinne erhöhter Vasoaktivität, spiegelt sich in einem zweiten systolischen Ausschlag (M-Zeichen) der A. cerebri media (ACM) wider. Bisher ist kaum etwas über die fetale Kreislaufregulation während der fötalen Spina Bifida (fSB) Operation bekannt. Daher war das Ziel dieser Studie, die Änderungen im ACM-Dopplerwellenmuster vor, während und nach fSB-Operationen zu analysieren.31 konsekutiv für fSB operierte Feten wurden eingeschlossen. ACM-Doppler wurden prospektiv vor, während und nach der fSB-Operation analysiert und wie folgt kategorisiert: normal, systolische Schulter, M-Zeichen, konkaves Muster. Die Wellenmuster wurden mit maternalen und fetalen Charakteristika sowie mit Narkosemitteln und dem A. umbilicalis-(UA)-Muster korreliert.Alle Feten zeigten M-Zeichen vor der fSB-Operation. Nach Beginn der Vollnarkose mit Desfluran verschwanden die Zeichen der Vasokonstriktion in 24/31 (78%) – und 19/31 (61%) der Feten zeigten einen Block oder Rückfluss in der UA. Der Zusammenhang zwischen beiden Dopplerbefunden war signifikant (p=0,007). Zeichen erhöhter Pulswellenreflektion sind nach der fSB-Operation initial wieder erschienen, über die Zeit (23 Tage ± 20, SD) jedoch bei allen Feten verschwunden.Die fSB-Läsion und eine fetale Operation beeinflussen den fetalen Kreislauf. Dieses Phänomen lässt sich gut mittels ACM-Doppler illustrieren. Während die Narkosemittel das M-Zeichen passager verschwinden lassen und oft ein pathologisches UA-Muster provozieren, resultiert die fSB-Operation letztlich in einer Normalisierung des ACM-Dopplermusters bzw. des fetalen Kreislaufes.

Introduction: Vaginal dysbiosis affects pregnancy outcomes, however, the relevance of abnormal findings on pre/post-surgical vaginal culture in women undergoing fetal spina bifida (fSB) repair is unknown. Objectives: To describe the incidence of normal and abnormal pre- and post-surgical vaginal microorganisms in fSB patients and to investigate potential associations between the type of vaginal flora and the occurrence of preterm prelabour rupture of membranes (PPROM) and preterm birth (PTB). Methods: 99 women undergoing fSB repair were eligible (2010–2019). Pre-surgical vaginal culture was routinely taken before surgery. Post-surgical cultures were taken on indication. Vaginal flora was categorized into four categories: healthy vaginal flora (HVF), bacterial vaginosis (BV), desquamative inflammatory vaginitis (DIV), and yeast infection. Results: The incidence of HVF, BV, DIV, or yeast infections was not statistically different between the pre- and postoperative patients. Furthermore, an abnormal pre/post-surgical vaginal flora was not associated with PPROM (OR 1.57 (0.74–3.32), p = 0.213)/OR 1.26 (0.62–2.55), p = 0.515), or with PTB (OR 1.19 (0.82–1.73), p = 0.315)/(OR 0.86 (0.60–1.24), p = 0.425). Conclusions: Abnormal vaginal microbiome was not associated with PPROM and PTB when appropriate treatment was performed.

Purpose: Laparoscopic ureteroureterostomy (LUU) has been proposed as an alternative to common sheath ureteral reimplantation (CSUR) in children with symptomatic duplex kidneys. However, data is limited for LUU in the pediatric population. The aim of this study was to analyze our experience with LUU and to compare the results with those after CSUR to assess whether a less invasive surgical approach could be a valid alternative.Patients and methods: The data of all children with duplex kidneys who underwent either LUU or CSUR at our center from 2006 to 2018 were reviewed retrospectively. After parental counseling, the option of LUU was provided as an alternative to CSUR for unilateral procedures and in the absence of vesicoureteral reflux to the receiving ureter. Baseline characteristics, indication for surgery, hospitalization and operative times, and intraoperative, post-operative, and late complications were analyzed. Preoperative and 1-year post-operative sonographies were reviewed by a pediatric radiologist. Increasing renal pelvic diameter (Δ >5 mm) was regarded as a sign of ureteral obstruction.Results: Forty children were included in this study, with 16 children receiving LUU and 24 children receiving CSUR. The children had a mean age of 2.7 years (7 months−9.8 years) and were followed up in our outpatient clinic for an average of 3.9 years (3 months−10.6 years) after surgery. The median hospital stay was 2 days shorter after LUU. Initially, a considerably longer time was needed for LUU, but after more experience was gained, similar operative times were observed for both procedures. Complications were encountered in both groups. After LUU, two patients developed anastomotic leakage: one was managed conservatively, and one required temporary nephrostomy. In the CSUR group, one patient developed vesicoureteral obstruction during follow-up and required reoperation with LUU. The occurrence of post-operative urinary tract infections was similar in both groups. No complications related to the ureteral stump after LUU arose.Conclusion: LUU is a safe and efficacious treatment option for children with duplex kidney anomalies and can be used as an alternative to CSUR. All children receiving LUU showed a non-obstructive, patent anastomosis and no signs for stenotic compromise of the receiving ureter.

An intricate problem during open human fetal surgery for spina bifida regards back skin closure, particularly in those cases where the skin defect is much too large for primary closure. We hypothesize that tissue engineering of fetal skin might provide an adequate autologous skin substitute for in utero application in such situations. Eight sheep fetuses of four time-mated ewes underwent fetoscopic skin biopsy at 65 days of gestation. Fibroblasts and keratinocytes isolated from the biopsy were used to create fetal dermo-epidermal skin substitutes. These were transplanted on the fetuses by open fetal surgery at 90 days of gestation on skin defects (excisional wounds) created during the same procedure. Pregnancy was allowed to continue until euthanasia at 120 days of gestation. The graft area was analyzed macroscopically and microscopically. The transplanted fetal dermo-epidermal skin substitutes was well discernable in situ in three of the four fetuses available for analysis. Histology confirmed healed grafts with a close to natural histological skin architecture four weeks after in utero transplantation. This experimental study generates evidence that laboratory grown autologous fetal skin analogues can successfully be transplanted in utero. These results have clinical implications as an analogous procedure might be applied in human fetuses undergoing prenatal repair to facilitate primary skin closure. Finally, this study may also fertilize the field of fetal tissue engineering in general, particularly when more interventional, minimally invasive, and open fetal surgical procedures become available.

Objective: The majority of patients counseled for prenatal open spina bifida repair (SBR) do not undergo fetal surgery. The aim of this study was to analyze the reasons for this phenomenon. Materials and Methods: The data of the first 160 patients seeking counseling or referred to the Zurich Center for Fetal Diagnosis and Therapy for prenatal SBR between December 2010 and March 2017 were retrospectively analyzed. Results: A total of 104 (65%) patients did not undergo prenatal SBR. Of this subgroup, 52% met the exclusion criteria, 35% decided to terminate pregnancy, 4% chose to continue pregnancy without fetal intervention, and 3% sought care in other European centers. In 6%, data about the ensuing course of pregnancy were not recorded. The main exclusion criteria were delayed presentation (30%), absence of hindbrain herniation (28%), and concomitant spinal anomalies (17%). Conclusion: The high percentage of patients not qualifying for prenatal SBR underscores the necessity of a standard evaluation of every single patient at a qualified referral center. To allow a higher proportion of women carrying a fetus with open spina bifida to be timely and correctly informed about a potential fetal intervention, much more effort is mandatory to spawn correct, objective, and understandable information among all groups of people potentially exposed to this topic.

Introduction Meningococcal septicemia is not merely an acute disease with a high lethality, but patients surviving the fulminant phase can suffer from serious long-term sequelae. Materials and Methods The records of 165 patients admitted to the hospital from 1968 to 2008 with the diagnosis of meningococcal septicemia were retrospectively reviewed for early signs at presentation, intensive care management, acute symptoms, the necessity of plastic and orthopaedic surgical management, and long-term orthopaedic sequelae. Possible predictors of these conditions were determined. Results Overall lethality was 17.5%, and mean time of hospitalization time was 28 days. Integument involvement occurred in 45%, often resulting in the necessity of plastic surgical procedures. Young age is a significant indicator or skin involvement. Amputations were necessary in 9% of all cases, affecting the lower extremities more often than the upper extremities. Six percent of all children suffered from long-term orthopaedic sequelae, such as growth retardation and angular deformities, appearing up to 11 years (mean 4.9 years) after onset of the acute disease. The incidence of amputations and long-term orthopaedic sequelae correlated significantly with severity of the disease. Conclusion Meningococcal septicemia can entail devastating long-term consequences in children surviving the acute phase of the disease. Sequelae may become apparent only years later and cause further damage. To prevent this, a systematic follow-up till adulthood is necessary.

Necrotizing fasciitis (NF) and purpura fulminans (PF) are conditions with extensive septic skin necroses that are associated with significant morbidity and mortality. NF is caused by fulminant bacterial spread on the superficial muscle fascia, Group A streptococcus being the main microorganism responsible for it. The major challenge NF poses is timely recognition. Although crucial for patient survival, early diagnosis is difficult because paucity of specific early findings does not allow setting NF apart from other, less severe, differential diagnoses. Surgical therapy consists of early and aggressive debridement of all affected tissue, even if large disfiguring wounds are left back. The responsible microorganism for PF in children is predominantly Neisseira meningitidis. Endotoxin triggered misbalance of anticoagulant and procoagulant activities of endothelial cells leads to disseminated intravascular coagulation (DIC) followed by microvascular thrombosis and bleeding, resulting in hemorrhagic skin infarction and limb ischemia. Although survival in PF is not dependent on surgery, and surgery plays not a key role in the early phase of the disease, early surgical consult to assess if limb perfusion can be improved to achieve limb salvage is still absolutely necessary. Debridement should be postponed until clear demarcation has established. Large defects after NF and PF can be successfully reconstructed with vacuum-assisted fixation of Integra (Integra LifeSciences Corporation, Plainsboro, New Jersey, United States) artificial skin before split-thickness skin grafting. This provides good functional and cosmetic results as well as good stump coverage in case of amputation in PF.