Single-center outcome analysis of 46 fetuses with megacystis after intrauterine vesico-amniotic shunting with the Somatex®intrauterine shunt.

OBJECTIVES: To assess the spectrum of underlying pathologies, the intrauterine course and postnatal outcome of 46 fetuses with megacystis that underwent intrauterine vesico-amniotic shunting (VAS) with the Somatex® shunt in a single center. METHODS: Retrospective analysis of 46 fetuses with megacystis that underwent VAS either up to 14 + 0 weeks (early VAS), between 14 + 1 and 17 + 0 weeks (intermediate VAS) or after 17 + 0 weeks of gestation (late VAS) in a single tertiary referral center. Intrauterine course, underlying pathology and postnatal outcome were assessed and correlated with the underlying pathology and gestational age at first VAS. RESULTS: 46 fetuses underwent VAS, 41 (89%) were male and 5 (11%) were female. 28 (61%) fetuses had isolated and 18 (39%) had complex megacystis with either aneuploidy (n = 1), anorectal malformations (n = 6), cloacal malformations (n = 3), congenital anomalies overlapping with VACTER association (n = 6) or Megacystis-Microcolon Intestinal-Hypoperistalsis Syndrome (MMIHS) (n = 2). The sonographic 'keyhole sign' significantly predicted isolated megacystis (p < 0.001). 7 pregnancies were terminated, 4 babies died in the neonatal period, 1 baby died at the age of 2.5 months and 34 (74%) infants survived until last follow-up. After exclusion of the terminated pregnancies, intention-to-treat survival rate was 87%. Mean follow-up period was 24 months (range 1-72). The underlying pathology was highly variable and included posterior urethral valve (46%), hypoplastic or atretic urethra (35%), MMIHS or prune belly syndrome (10%) and primary vesico-ureteral reflux (2%). In 7% no pathology could be detected postnatally. No sonographic marker was identified to predict the underlying pathology prenatally. 14 fetuses underwent early, 24 intermediate and 8 late VAS. In the early VAS subgroup, amnion infusion prior to VAS was significantly less often necessary (7%), shunt complications were significantly less common (29%) and immediate kidney replacement therapy postnatally became less often necessary (0%). In contrast, preterm delivery ≤ 32 + 0 weeks was more common (30%) and survival rate was lower (70%) after early VAS compared to intermediate or late VAS. Overall, 90% of liveborn babies had sufficient kidney function without need for kidney replacement therapy until last follow-up, and 95% had sufficient pulmonary function without need for mechanical respiratory support. 18% of babies with complex megacystis suffered from additional health restrictions due to their major concomitant malformations. CONCLUSIONS: Our data suggest that VAS is feasible from the first trimester onward. Early intervention has the potential to preserve neonatal kidney function in the majority of cases and enables neonatal survival in up to 87% of cases. Despite successful fetal intervention, parents should be aware of the potential of mid- or long-term kidney failure and of additional health impairments due to concomitant extra-renal anomalies that cannot be excluded at time of intervention.

Postnatal surgical treatment and complications following intrauterine vesicoamniotic shunting with the SOMATEX® intrauterine shunt. A single center experience.

INTRODUCTION: Intrauterine vesicoamniotic shunting (VAS) using a Somatex® shunt was shown to significantly affect survival of male fetuses with megacystis in suspected lower urinary tract obstruction (LUTO) [Figure 1]. Data on postnatal surgical management and complications are largely lacking. OBJECTIVE: To describe the postnatal management of patients with prenatal VAS for megacystitis in suspected severe LUTO. STUDY DESIGN: All male newborns with previous intrauterine VAS using a Somatex® shunt treated in our institution were retrospectively analyzed. We evaluated the spectrum of urethral pathologies and postnatal surgical management, especially focusing on shunt removal. RESULTS: Between 2016 and 2022, 17 patients (all male) were treated postnatally in our institution after VAS for suspected severe LUTO. Five fetuses with dislocated shunts underwent re-implantation in utero. Overall, premature birth before the 38th week of gestation was observed in eight patients (8/17). Seven shunts could be removed without further anesthesia as a bedside procedure. Ten patients required surgical shunt removal under general anesthesia due to migration (59%). Laparoscopic shunt extraction was performed in 8/10 cases. Most frequently, dislocated shunts were located incorporated in the detrusor in eight cases and the removal required a bladder suture in 2/8 patients. In one case, the shunt was removed from the abdominal wall and in one case from the intestine wall [Figure 2]. Posterior urethral valves were found in 8/17 patients, 6/17 patients showed a urethral atresia and one patient had urethral duplication. In two patients, we identified a high grade bilateral vesicoureteral reflux without LUTO. CONCLUSION: In our observation, more than half of the newborns with megacystis in suspected LUTO require a shunt removal surgery after early VAS using a Somatex® shunt. Urethral atresia may be found more frequently in these patients. These data should be taken into consideration for prenatal counselling of parents and planning of postnatal management.

Extrarenal Manifestations in Shigatoxin-associated Haemolytic Uremic Syndrome.

BACKGROUND: Shigatoxin-associated haemolytic uremic syndrome (STEC-HUS) is the most frequent cause of acute kidney injury in children worldwide. Extrarenal manifestations are the main determinants for both, short- and long-term prognosis of patients with STEC-HUS. PATIENTS: 46 patients treated over the last 10 years for STEC-HUS in a single center. METHODS: This retrospective study analysed the incidence and outcome of extrarenal manifestations in our cohort of children with STEC-HUS. Risk factors for extrarenal involvement and adverse outcome were assessed by detailed chart review. RESULTS: Eleven extrarenal manifestations occurred in 9/46 patients comprising 8 neurological, 2 gastro-intestinal, and 1 cardiovascular complication. One patient died from cerebral bleeding. Liver transplantation was required in a girl 18 months after HUS due to secondary sclerosing cholangitis. PATIENTS with extrarenal manifestations were significantly younger and presented with higher leucocyte counts and higher alanine aminotransferase levels at admission. Renal replacement therapy was necessary for a longer period than in patients without extrarenal complications. CONCLUSION: Extrarenal manifestations occurred in about 20% of our patients with STEC-HUS. The identification of risk-factors will help to provide a better management of these patients which might also include novel treatment strategies like complement inhibition.

Post-appendectomy intra-abdominal abscesses--can they successfully be managed with the sole use of antibiotic therapy?

PURPOSE: Controversy persists concerning the management of post-appendectomy intra-abdominal abscesses. We hypothesised that most of these abscesses can be successfully managed by antibiotic treatment alone, avoiding the complications of surgical treatment. METHODS: Hospital records of children treated in our unit for intra-abdominal post-appendectomy abscesses over a 6-year period were reviewed retrospectively. RESULTS: This study investigates a series of 26 children from 2 to 15 years of age presenting with one or more post-appendectomy intra-abdominal abscesses. After an average delay of 7 days after initial surgery, 23 children had developed an isolated abscess, while 3 children had multiple abscesses. Twenty-two patients (84.8 %) were treated conservatively by intravenous triple antibiotic therapy alone. Complete clinical, radiological and biological resolution of the abscesses was obtained in all of these children after a mean hospitalisation of 8 days. Four children (15.2 %) were treated surgically: three children with a stable patient status and one child with septic shock requiring urgent surgery. CONCLUSION: The results suggest that intravenous triple antibiotic therapy alone is an efficacious first-line treatment in children developing intra-abdominal abscesses following appendectomy. Surgical intervention is rarely necessary except in patients with an alarming patient status or with signs of septic shock.

[Quality of guidelines for the treatment of alcohol related disorders -- a systematic review and content analysis]. / Qualität aktueller Leitlinien zur Diagnostik und Behandlung alkoholbezogener Störungen -- Eine systematische Ubersicht und inhaltliche Analyse.

BACKGROUND: Alcohol related disorders (dependent, harmful or at risk drinking) display a high prevalence in the community. We systematically reviewed and assessed the quality of available guidelines in the context of developing an evidence-based pathway of care for the treatment of those disorders in primary care. METHODS: We searched the internet for guidelines published in English, German, Italian, French or Spanish. The search was performed using the following sources: Internet Search engines, guideline providing agencies via internet, MEDLINE, PsychInfo and Cochrane Library. In addition, national professional societies were contacted to provide existing guidelines. We used a standardized instrument to assess methodological quality in three dimensions using dichotomous items: methodology of guideline development (21 items), content of guideline (17 items) and application (6 items). Each guideline was evaluated independently by two reviewers (MB, SH). Disagreement was documented using a disagreement protocol and resolved by discussion. Results were expressed as percent of total (domain) score. RESULTS: We included 9 published guidelines and 2 drafts. The methodological quality was heterogeneous. The mean quality score was below 50 % of the maximum score. Major flaws were lack of clear grounding in evidence-based medicine, too much information provided, lack of consumer involvement or insufficient involvement of all care providers as well as lack of implementation strategies. The scores of the guidelines published by professional societies for primary care were higher than the others. DISCUSSION: As demonstrated by the identified guidelines, more interdisciplinary panels and evidence-based processes need to be incorporated in the development process of guidelines for the treatment of alcohol related disorders. Our findings suggest that it might be advisable and effective to adapt already available valid and evidence-based guidelines or to collaborate with international working groups for the development of guidelines. There is a strong need for the scientific evaluation of implementation processes.