Amniotic fluid content in children with kidney and urinary tract anomalies determines pre- and postnatal development.

BACKGROUND: Renal oligohydramnios (ROH) describes an abnormally low volume of amniotic fluid (AF) during pregnancy. ROH is mostly caused by congenital fetal kidney anomalies. The ROH diagnosis frequently implies an increased risk of peri- and postnatal fetal mortality and morbidity. The present study aimed to evaluate the impact of ROH on pre-and postnatal development in children with congenital kidney anomalies. METHODS: This retrospective study included 168 fetuses with anomalies in the kidney and urinary tract. Based on the amount of AF measured by ultrasound, patients were divided into three groups: normal amniotic fluid (NAF), amniotic fluid in the lower normal range (LAF), and ROH. These groups were compared with respect to prenatal sonographic parameters, perinatal outcomes, and postnatal outcomes. RESULTS: Among the 168 patients with congenital kidney anomalies, 26 (15%) had ROH, 132 (79%) had NAF, and 10 (6%) had LAF. Of the 26 families affected by ROH, 14 (54%) decided to terminate pregnancy. Of 10 live-born children in the ROH group, 6 (60%) survived the observation time; of these, 5/6 presented with chronic kidney disease, stages I-III, at their last examination. The main differences in postnatal development between the ROH group and the NAF and LAF groups were: restricted height and weight gain, respiratory issues, complicated feeding, and the presence of extrarenal malformations. CONCLUSIONS: ROH is not a mandatory indicator of severe postnatal kidney function impairment. However, children with ROH have complicated peri-and postnatal periods, due to the presence of concomitant malformations, which must be considered in prenatal care. A higher resolution version of the Graphical abstract is available as Supplementary information.

Operative Volume of Newborn Surgery in German University Hospitals: High Volume Versus Low Volume Centers.

INTRODUCTION: Adequate patient volume is essential for the maintenance of quality, meaningful research, and training of the next generation of pediatric surgeons. The role of university hospitals is to fulfill these tasks at the highest possible level. Due to decentralization of pediatric surgical care during the last decades, there is a trend toward reduction of operative caseloads. The aim of this study was to assess the operative volume of the most relevant congenital malformations at German academic pediatric surgical institutions over the past years. METHODS: Nineteen chairpersons representing university-chairs in pediatric surgery in Germany submitted data on 10 index procedures regarding congenital malformations or neonatal abdominal emergencies over a 3-year period (2015 through 2017). All institutions were categorized according to the total number of respective cases into "high," "medium," and "low" volume centers by terciles. Some operative numbers were verified using data from health insurance companies, when available. Finally, the ratio of cumulative case load versus prevalence of the particular malformation was calculated for the study period. RESULTS: From 2015 through 2017, a total 2,162 newborns underwent surgery for congenital malformations and neonatal abdominal emergencies at German academic medical centers, representing 51% of all expected newborn cases nationwide. The median of cases per center within the study period was 101 (range 18-258). Four institutions (21%) were classified as "high volume" centers, four (21%) as "medium volume" centers, and 11 (58%) as "low volume" centers. The proportion of patients operated on in high-volume centers varied per disease category: esophageal atresia/tracheoesophageal fistula: 40%, duodenal atresia: 40%, small and large bowel atresia: 39%, anorectal malformations: 40%, congenital diaphragmatic hernia: 56%, gastroschisis: 39%, omphalocele: 41%, Hirschsprung disease: 45%, posterior urethral valves: 39%, and necrotizing enterocolitis (NEC)/focal intestinal perforation (FIP)/gastric perforation (GP): 45%. CONCLUSION: This study provides a national benchmark for neonatal surgery performed in German university hospitals. The rarity of these cases highlights the difficulties for individual pediatric surgeons to gain adequate clinical and surgical experience and research capabilities. Therefore, a discussion on the centralization of care for these rare entities is necessary.

Diagnosis and Therapy of Female Genital Malformations (Part 1). Guideline of the DGGG, OEGGG and SGGG (S2k Level, AWMF Registry Number 015/052, May 2019).

Objectives Female genital malformations may take the form of individual entities, they may involve neighboring organs or they may occur in the context of complex syndromes. Given the anatomical structures of the vulva, vagina, uterus and adnexa, the clinical picture of malformations may vary greatly. Depending on the extent of the malformation, organs of the urinary system or associated malformations may also be involved. Methods This S2k-guideline was developed by representative members from different medical specialties and professions as part of the guidelines program of the DGGG, SGGG and OEGGG. The recommendations and statements were developed using a structured consensus process with neutral moderation and voted on. Recommendations The guideline is the first comprehensive presentation of the symptoms, diagnosis and treatment options for female genital malformations. Additional chapters on classifications and transition were included.

Diagnosis and Therapy of Female Genital Malformations (Part 2). Guideline of the DGGG, OEGGG and SGGG (S2k Level, AWMF Registry Number 015/052, May 2019).

Objectives Female genital malformations may be present in the form of individual entities, they may involve neighboring organs or they may occur in the context of complex syndromes. Given the anatomical structures of the vulva, vagina, uterus and uterine appendages, the clinical picture of malformations varies greatly. Methods This S2k-guideline was developed by representative members from different medical specialties and professions as part of the guidelines program of the DGGG, SGGG and OEGGG. The recommendations and statements were developed and voted on using a structured consensus process with neutral moderation. Recommendations This guideline is the first comprehensive summary of female genital malformations from infancy to adulthood which covers clinical examinations, diagnostic workups and treatment options. Additional chapters have been included on complex urogenital malformations, vascular malformations, psychosomatic care, and tumor risk.

Primary Segmental Small Bowel Volvulus in an Adolescent Female.

Small bowel volvulus is a rare but important cause of abdominal pain and small bowel obstruction in children and adults. In the neonate, small bowel volvulus is a well-known complication of malrotation. Segmental small bowel volvulus is a lesser-known condition, which occurs in children and adults alike and can rapidly progress to bowel ischemia. Primary segmental small bowel volvulus occurs in the absence of rotational anomalies or other intraabdominal lesions and is rare in Europe and North America. Clinical presentation can be misleading, causing a delay in diagnosis and treatment, in which case the resection of necrotic bowel may become necessary. We report on a 14-year-old girl who presented with severe colicky abdominal pain but showed no other signs of peritoneal irritation or bowel obstruction. An emergency magnetic resonance imaging was highly suspicious for small bowel volvulus. Emergency laparotomy revealed a 115 cm segment of strangulated distal ileum with no underlying pathology. We performed a detorsion of the affected bowel segment. Despite the initial markedly ischemic appearance of the affected bowel segment, the patient achieved full recovery without resection of bowel becoming necessary.

Case Report: Report of 2 Different Cases of Ovarian Teratoma Evaluated by Dynamic Contrast-Enhanced Ultrasound.

Ovarian masses are not easily differentiated on transabdominal ultrasound in children. A useful supplement in various pediatric applications is dynamic contrast-enhanced ultrasound (dynCEUS). It can be performed quickly and easily. However, the literature for dynCEUS on pediatric ovarian masses is limited. We compared two cases with ovarian teratoma in which dynCEUS was a helpful additional tool.

Successful treatment of a huge congenital cervical teratoma.

Congenital cervical teratomas are extremely rare tumors with high perinatal mortality and morbidity rates particularly due to compression and distortion of the infant's airway. Hence, these mostly benign malformations require immediate excision, whereas surgery of these tumors is challenging for a multidisciplinary team. We report on a recent case of congenital cervical mature teratoma with total excision and cure. The aim of this case study is to report the authors' experience in managing a case of congenital cervical teratoma to provide a structured approach and help in decision making, once prenatal diagnosis is made.

Xanthogranulomatous pyelonephritis in children: diagnostic and therapeutic aspects.

Xanthogranulomatous pyelonephritis (XGP) is a rare, chronic inflammatory disease of the kidneys. It is characterized by destruction of renal parenchyma and accumulation of lipid-laden foamy macrophages. Diffuse and focal forms are known. The condition is mainly observed in middle-aged women, and it is very rare in childhood. Of 32 nephrectomies carried out in children for various diseases in our hospital over the course of 2 years, there were two cases of diffuse XGP. In both cases, the preoperative diagnosis based on ultrasound findings was highly suggestive of XGP. We present the two cases and define the typical ultrasonographic signs for distinguishing XGP from other renal masses. The diagnostic and therapeutic management is discussed and an overview of the literature is given.

Posterior urethral valves: prenatal diagnostic signs and outcome.

INTRODUCTION: Urethral valves can be of enormous clinical importance. Both the prognosis for an affected fetus and the indication for a prenatal therapeutic intervention depend to a high degree on the accuracy of the prenatal diagnosis. PATIENTS AND METHODS: The sonographic findings and the results of the postnatal diagnostic workup of 24 boys treated for urethral valves in our institute are analyzed in the present paper. RESULTS: Out of a group of 900 children, diagnosed prenatally as having urinary tract anomalies, the postnatal diagnostic workup revealed 24 boys suffering from posterior urethral valves. The combination of megacystis, oligohydramnios, and bilateral renal abnormalities was present in 3 boys, and it was only in these cases that urethral valves were correctly identified as the underlying pathology. The largest group (n = 16) of these fetuses presented with bilateral hydronephrosis with and without megaureter. Megacystis in any combination with other findings was detected only in 4 patients. Oligohydramnios as a sign of impaired renal function was observed in four pregnancies. Four children suffered postnatally from beginning renal insufficiency. Eight infants (33%) required some form of respiratory support. This group included the 4 newborns with oligohydramnios during the pregnancy. CONCLUSIONS: Posterior urethral valves represent a rare but severe congenital malformation. They can disrupt the development of the entire urinary tract and may be life-limiting to the affected boy. Only rarely, however, are they manifested as the complete pathological picture on prenatal ultrasound. In most cases, bilateral impairment of the fetal renal development suggests severe subvesical obstruction. Thus in cases with bilateral renal impairment on prenatal ultrasonography, posterior urethral valves are to be excluded postnatally. Oligohydramnios is a predictor of a poor outcome of the renal function.

Antenatal diagnostic aspects of unilateral multicystic kidney dysplasia--sensitivity, specificity, predictive values, differential diagnoses, associated malformations and consequences.

OBJECTIVES: Unilateral multicystic kidney dysplasia (MCKD) is the second most common urinary tract abnormality diagnosed antenatally. Whilst an isolated unilateral MCKD has a good prognosis, a poor outcome must be expected when MCKD is associated with other complex abnormalities. MATERIAL: Out of 11,176 cases, 693 fetuses were suspected of having urinary tract abnormalities. Urological findings were confirmed in 548 of them. Unilateral cystic kidney was diagnosed prenatally in 85 cases. RESULTS: The study results in a total of 107 cases with proven MCKD. Eighty-five pregnancies with a prenatal diagnosis of MCKD were analysed. The antenatal diagnosis of MCKD was confirmed in 56 cases. Fifty-one children were found to have unilateral MCKD where this had not been explicitly suspected from antenatal scanning. CONCLUSION: Unilateral MCKD is a malformation with an excellent prognosis for child survival and global renal function if encountered in isolation. However, our analysis of live infants and autopsy cases demonstrates a high proportion of severe associated malformations of the urinary tract and other organ systems.