congenital malformations
Recently Published Documents


TOTAL DOCUMENTS

3474
(FIVE YEARS 579)

H-INDEX

89
(FIVE YEARS 6)

Author(s):  
Martin Lacher ◽  
Winfried Barthlen ◽  
Felicitas Eckoldt ◽  
Guido Fitze ◽  
Jörg Fuchs ◽  
...  

Abstract 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.


2022 ◽  
Vol 0 (0) ◽  
Author(s):  
Marie Uecker ◽  
Benno Ure ◽  
Julia Hannah Quitmann ◽  
Jens Dingemann

Abstract Survival rates of patients with visceral congenital malformations have increased considerably. However, long-term morbidity in these patients is high. In the last decades, these circumstances have led to a shift in goals of caretakers and researchers with a new focus on patients’ perspectives and long-term morbidity. Health-related quality of life (HrQoL) is the most commonly used patient-reported outcome measure to assess the impact of chronic symptoms on patients’ everyday lives. Most pediatric surgical conditions can cause a significantly decreased HrQoL in affected patients compared to the healthy population. In order to guarantee life-long care and to minimize the impact on HrQoL a regular interdisciplinary follow-up is obligatory. The period of transition from child-centered to adult-oriented medicine represents a critical phase in the long-term care of these complex patients. This scoping review aims to summarize relevant pediatric surgical conditions focusing on long-term-morbidity and HrQoL assessment in order to demonstrate the necessity for a well-structured and standardized transition for pediatric surgical patients.


2022 ◽  
Vol 11 (2) ◽  
pp. 01-06
Author(s):  
F. Bouchenaki

The term spina includes several types of congenital malformations including closed spinal dysraphisms, among these 10% are dermal sinuses.Our series includes 29 patients, the majority of which were asymptomatic, but these can present with different clinical pictures including neurological and / or infectious signs indicating a neglected dermal sinus and which therefore requires urgent treatment in the environment. Neurosurgical.MRI confirmed the diagnosis showing the dermal sinus tract communicating from the surface of the skin to the intra-dural space.Once the diagnosis has been made, surgical treatment becomes imperative and urgent because, despite the benignity of the lesion, the majority of which are located in the lumbar region, can lead to formidable neurological sequelae compromising the functional prognosis or even the vital prognosis in some cases.All of our patients were operated on and we obtained 90% good results, however we deplore one case of aggravation and one death.


2022 ◽  
Vol 100 (S267) ◽  
Author(s):  
Sunayna Best ◽  
Jenny Lord ◽  
Matthew Roche ◽  
Christopher Watson ◽  
James Poulter ◽  
...  

2022 ◽  
Vol 226 (1) ◽  
pp. S721
Author(s):  
Katherine Pressman ◽  
Anthony Kendle ◽  
Tara Randis ◽  
Keyur donda ◽  
Jason L. Salemi ◽  
...  

2022 ◽  
Vol 23 (1) ◽  
pp. 95
Author(s):  
KennethSalazar Madrigal ◽  
NoeliaChaves Alfaro ◽  
Diego Morales-Neira

Author(s):  
Naoyuki AIHARA ◽  
Anna MOMOKI ◽  
Nanase HATTORI ◽  
Hironobu MURAKAMI ◽  
Motoharu OISHI ◽  
...  

2022 ◽  
Vol 52 (7) ◽  
Author(s):  
Júlia Gabriela Wronski ◽  
Fernando Froner Argenta ◽  
Regina Tose Kemper ◽  
Jacqueline Raiter ◽  
Natasha Rossoni de Oliveira ◽  
...  

ABSTRACT: Multiple congenital malformations can occur concomitantly in several species since the anomaly in one organ may lead directly to the malformation of another. Additionally, the etiology is not always clarified. Choristoma refers to an ectopic tissue that is histologically normal in an abnormal location. A case of pulmonary and nodal choristoma associated with cranioschisis, hydrocephalus, and syringomyelia in a new-born calf is reported here. Clinically, the calf had a mass in the frontal region of the head associated with local bone deformation. At necropsy, there was cranioschisis in the junction of the frontal bones and a 14 × 10 × 7 cm mass, grossly consistent with well-differentiated pulmonary tissue covered by skin, covering the opening between the frontal bones, and extending into the cranial cavity, leading to local cerebral compression. With the pulmonary choristoma, there was a well-differentiated lymphoid tissue. Additionally, in the central nervous system, there was severe hydrocephalus involving lateral ventricles and multiple areas of syringomyelia in the spinal cord.


2021 ◽  
Author(s):  
Yousef Khader ◽  
Nihaya Al-Sheyab ◽  
Mohammad Alyahya ◽  
Ziad El-Khatib ◽  
Khulood Shattnawi ◽  
...  

BACKGROUND Stillbirth and neonatal mortality declined significantly in high- and some middle- income countries because of the significant improvements in obstetric and neonatal care. Yet, stillbirth and neonatal mortality rates remain high in low-income countries. The main reason for low progress in reducing such stillbirths and neonatal deaths in Jordan is the scarcity of data on causes and contributing factors leading to these deaths. OBJECTIVE This study aimed to determine the rates, causes and risk factors of stillbirth and neonatal mortality in Jordan. METHODS An electronic stillbirth and neonatal deaths surveillance system was established in five large hospitals in Jordan. Data on all births, stillbirths and neonatal deaths and their causes during the period May 2019 – December 2020 were exported from the system and analyzed. RESULTS A total of 29,592 women gave birth to 31,106 babies during a period of 20 months in the selected hospitals. The stillbirth rate was 10.5 per 1,000 total births, the neonatal death rate was 14.2 per 1,000 live births, and the perinatal death rate was 21.4 per 1,000 total births. Of all neonatal deaths, 29.4% died within the first day of life and 77.8% died during the first week of life. For neonatal deaths occurred pre-discharge, the leading causes of death were respiratory and cardiovascular disorders (35.0%), low birth weight and prematurity (32.7%), and congenital malformations, deformations and chromosomal abnormalities (19.5%). Almost one third of stillbirths had unspecified cause of death (33.3% of antepartum stillbirths and 28.9% of intrapartum stillbirths). Acute antepartum event was responsible of 27.4% of antepartum stillbirths and acute intrapartum event was responsible for 13.2% of intrapartum stillbirths. Congenital malformations, deformations and chromosomal abnormalities contributed to 18.1% of antepartum stillbirths and 34.2% of intrapartum stillbirths. CONCLUSIONS There is a relative stability of stillbirth and neonatal mortality rates in Jordan. Several identified maternal and/or fetal conditions that contributed to stillbirths and/or neonatal deaths in Jordan are preventable. Focused care needs to be directed high-risk pregnant women and to neonates with low birthweight and respiratory problems.


2021 ◽  
Vol 2021 (71) ◽  
pp. 193-200
Author(s):  
Е.М. Оmelchenko ◽  
◽  
О.О. Polka ◽  
О.V. Lynchak ◽  
L.А. Каramzina ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document