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Congenital surgical malformation

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https://www.readbyqxmd.com/read/29786408/impact-of-additional-tricuspid-valve-annuloplasty-in-tof-patients-undergoing-pulmonary-valve-replacement
#1
Sabrina Lueck, Eike Bormann, Kathrin Rellensmann, Sven Martens, Andreas Rukosujew
BACKGROUND: Many patients with tetralogy of Fallot (TOF) who underwent surgical correction of their congenital cardiac malformation during infancy develop right ventricular dysfunction and exercise intolerance in the long term. The right ventricle (RV) dilates due to the development of severe pulmonary regurgitation (and secondary tricuspid insufficiency). To reduce RV dilation and improve exercise tolerance pulmonary valve replacement (PVR) is the common therapeutic strategy. Whether concomitant tricuspid valve repair (TVR) is beneficial in these pure volume-overload conditions is still unknown...
May 22, 2018: Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/29782301/lymphangioma-circumscriptum-post-radiotherapy-for-penile-cancer-treated-with-co%C3%A2-laser
#2
Dietmar Schulz, Andreas Lein, Ancuta Proca Nicula, Katrin Schierle, Caius Solovan
Lymphangioma circumscriptum (LC) is a rare, benign condition, predominantly characterized by the malformation of lymphatic skin vessels. Its onset may be congenital or due to secondary causes such as radiotherapy, infections, or surgical procedures. We present the case of a 55-year-old patient with a pathologic history of squamous cell carcinoma of the penis followed by radical penectomy. Due to metastasis to the locoregional lymph nodes, the entire affected area was subsequently treated with radiation therapy, receiving a total dose of 55...
April 2018: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/29773423/management-of-giant-embryonic-vein-in-klippel-tr%C3%A3-naunay-syndrome
#3
Animesh Rathore, Peter Gloviczki, Haraldur Bjarnason
Klippel-Trénaunay syndrome is a rare mixed malformation characterized by congenital varicose veins, low-flow venous and lymphatic malformations, hypertrophy of soft tissue and bone, and capillary malformations. A 35-year-old man with a diagnosis of Klippel-Trénaunay syndrome presented to the clinic with significant pain and swelling in the left leg. Initial conservative management with compression therapy failed. He was then managed surgically with preoperative placement of an inferior vena cava filter (because of a history of deep venous thrombosis and pulmonary embolism), followed by resection of the lateral embryonic vein, ligation of large perforators, and excision of smaller varicosities...
May 14, 2018: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/29767556/hydronephrosis-is-associated-with-elevated-plasmin-in-urine-in-pediatric-patients-and-rats-and-changes-in-ncc-and-%C3%AE-enac-abundance-in-rat-kidney
#4
Rikke Zachar, Ammar Al-Mashhadi, Henrik Dimke, Per Svenningsen, Boye L Jensen, Mattias Carlström
Obstruction of urine flow at the level of the pelvo-ureteric junction (UPJO) and subsequent development of hydronephrosis is one of the most common congenital renal malformations. UPJO is associated with development of salt-sensitive hypertension, which is set by the obstructed kidney, and with a stimulated renin-angiotensin-aldosterone system (RAAS) in rodent models. This study aimed at investigating the hypothesis that i) in pediatric patients with UPJO the RAAS is activated prior to surgical relief of the obstruction; ii) in rats with UPJO the RAAS activation is reflected by increased abundance of renal aldosterone-stimulated Na+ transporters; and iii) the injured UPJO kidney allows aberrant filtration of plasminogen leading to proteolytic activation of the epithelial sodium channel gamma subunit (γ-ENaC)...
May 16, 2018: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29762087/genomics-and-epigenomics-of-congenital-heart-defects-expert-review-and-lessons-learned-in-africa
#5
Nicholas Ekow Thomford, Kevin Dzobo, Nana Akyaa Yao, Emile Chimusa, Jonathan Evans, Emmanuel Okai, Paul Kruszka, Maximilian Muenke, Gordon Awandare, Ambroise Wonkam, Collet Dandara
Congenital heart defects (CHD) are structural malformations found at birth with a prevalence of 1%. The clinical trajectory of CHD is highly variable and thus in need of robust diagnostics and therapeutics. Major surgical interventions are often required for most CHDs. In Africa, despite advances in life sciences infrastructure and improving education of medical scholars, the limited clinical data suggest that CHD detection and correction are still not at par with the rest of the world. But the toll and genetics of CHDs in Africa has seldom been systematically investigated...
May 2018: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/29740591/postnatal-and-adult-aortic-heart-valves-have-distinctive-transcriptional-profiles-associated-with-valve-tissue-growth-and-maintenance-respectively
#6
Emily Nordquist, Stephanie LaHaye, Casey Nagel, Joy Lincoln
Heart valves are organized connective tissues of high mechanical demand. They open and close over 100,000 times a day to preserve unidirectional blood flow by maintaining structure-function relationships throughout life. In affected individuals, structural failure compromises function and often leads to regurgitant blood flow and progressive heart failure. This is most common in degenerative valve disease due to age-related wear and tear, or congenital malformations. At present, the only effective treatment of valve disease is surgical repair or replacement and this is often impermanent and requires anti-coagulation therapy throughout life...
2018: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29739453/spontaneous-renal-hemorrhage-secondary-to-choriocarcinoma-in-a-man-with-congenital-hypospadias-and-cryptorchidism-a-case-report-and-literature-review
#7
Yi Li, Gang Chen, Han Chen, Shuang Wen, Chao-Yu Xiong, Zi-Yi Yang, Yun-Xiao Zhu, Nathan Jeffreys
BACKGROUND: Choriocarcinoma is a rare malignant germ-cell tumour, most commonly found in adult women. It infrequently presents as spontaneous renal haemorrhage (SRH). Genital malformation and SRH secondary to choriocarcinoma has previously been only reported in females. We present what we believe to be the first case of a male patient with genital malformation (hypospadias and cryptorchidism) and SRH at presentation of choriocarcinoma. CASE PRESENTATION: A 25-year-old man presented to the department with intense pain in the right flank region and lower back...
May 8, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29730740/congenital-portosystemic-shunts-diagnosis-and-treatment
#8
Stéphanie Franchi-Abella, Emmanuel Gonzales, Oanez Ackermann, Sophie Branchereau, Danièle Pariente, Florent Guérin
Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts...
May 5, 2018: Abdominal Radiology
https://www.readbyqxmd.com/read/29726261/-artificial-bowel-sphincter-ten-years-later
#9
J Hoch, R Škába, Z Jech
INTRODUCTION: Congenital anorectal malformation is the most common cause of fecal incontinence in children and young adults. Surgical treatment options are limited. One of the treatment methods is the implantation of an artificial bowel sphincter. The goal of this study was to investigate the efficacy of the artificial bowel sphincter in reaching long-term fecal continence in patients with anorectal malformation. METHODS: Young adults with fecal incontinence due to anorectal malformation were included in the study...
2018: Rozhledy V Chirurgii: Měsíčník Československé Chirurgické Společnosti
https://www.readbyqxmd.com/read/29721247/anesthesia-in-mowat-wilson-syndrome-information-on-11-italian-patients
#10
Marianna Spunton, Livia Garavelli, Paola Cerutti Mainardi, Uta Emmig, Enrico Finale, Andrea Guala
Mowat-Wilson syndrome is a genetic disease caused by heterozygous mutations or deletions of the ZEB2 gene and characterized by typical clinical features. The congenital malformations typical of this syndrome call for early diagnostic and surgical procedures requiring general anesthesia, but few information about the anesthesiology management of such patients is available. We enrolled 11 families of patients with Mowat-Wilson syndrome who had undergone surgical or diagnostic procedures requiring general anesthesia, and sent them a retrospective questionnaire including 16 open questions about the procedures...
March 22, 2018: Pediatric Reports
https://www.readbyqxmd.com/read/29719980/congenital-cystic-adenomatoid-malformation-in-adults-presenting-as-a-single-cyst
#11
Adriana Scamporlino, Andrea Ambrosini, Ercole Turrini, Uliano Morandi, Alessandro Stefani
Congenital cystic adenomatoid malformations are lung anomalies usually detected prenatally or in newborns and infants. Type 1 congenital cystic adenomatoid malformations appears as a multicystic lesion, with cysts up to 2 cm in diameter, or as a single large cyst. In the latter case, when detected in adults, the preoperative diagnosis is challenging because congenital cystic adenomatoid malformations can be confused with other more common lesions. We describe two cases of uniloculated type 1 congenital cystic adenomatoid malformation in adults...
January 1, 2018: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/29713311/cryptorchidism-in-boys-with-cerebral-palsy-is-associated-with-the-severity-of-disease-and-with-co-occurrence-of-other-congenital-anomalies
#12
Julia Spencer Barthold, Anton Wintner, Jennifer A Hagerty, Kenneth J Rogers, Md Jobayer Hossain
Background: Cryptorchidism is reported in 40-50% of small case series of cerebral palsy (CP) and attributed to hypothalamic-pituitary-gonadal axis abnormalities, intellectual disability (ID), or cremaster spasticity. We collected demographic and clinical data to define the frequency of cryptorchidism and clinical comorbidities in a large CP population. Methods: Electronic health record data were collected for all male patients ≥7 years of age seen in a large, multidisciplinary CP clinic between 2000 and 2016...
2018: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29712514/prenatal-diagnosis-of-suprarenal-mass-by-magnetic-resonance-imaging-a-case-series
#13
Pedro Castro, Ana Paula Matos, Heron Werner, Tatiana Fazecas, Renata Nogueira, Pedro Daltro, Edward Araujo Júnior
OBJECTIVE: The aim of this study was to evaluate the perinatal outcomes and postnatal follow-up of prenatally diagnosed suprarenal masses (SRMs) evaluated by magnetic resonance imaging (MRI). METHODS: Thirteen fetuses with SRMs detected during routine prenatal ultrasound screening were evaluated by MRI between February 2007 and May 2015. The prenatal characteristics of the masses, complications of the pregnancies, and related malformations were collected as the postnatal clinical, radiological, and pathological data...
May 15, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29712482/does-pregnancy-interval-after-laparoscopic-sleeve-gastrectomy-affect-maternal-and-perinatal-outcomes
#14
Alper Basbug, Aşkı Ellibeş Kaya, Sami Dogan, Mevlut Pehlivan, Gokhan Goynumer
BACKGROUND: Obesity is a global health epidemic and is associated with many maternal and neonatal complications. Laparoscopic sleeve gastrectomy (LSG) is among the surgical treatments for obesity. The appropriate timing of pregnancy following LSG remains controversial and few studies have evaluated this public health issue. OBJECTIVE: To evaluate the effect of pregnancy timing after LSG on maternal and perinatal outcomes. STUDY DESIGN: We performed a retrospective observational study of 23 pregnant women who underwent LSG at a tertiary hospital in Turkey...
April 30, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29707692/successful-surgical-resection-of-giant-arteriovenous-malformation-in-supraclavicular-fossa
#15
Kohei Horikawa, Hiroyuki Nishi, Naosumi Sekiya, Mitsutomo Yamada, Toshiki Takahashi
A 42-year-old woman with a large congenital giant arteriovenous malformation in the left supraclavicular fossa underwent surgical resection. Although endovascular treatment was initially planned, it was impossible to occlude the multiple feeding arteries (transverse cervical, clavicular branch of left internal mammary, thoracoacromial, anterior/posterior circumflex humeral), and the anatomy was difficult. After removal of the left clavicle, the arteriovenous malformation was exposed. Care was taken to not injure the brachial plexus, and each feeding artery was ligated, followed by division of the drainage veins...
June 2018: Journal of Vascular Surgery Cases and Innovative Techniques
https://www.readbyqxmd.com/read/29682473/uniportal-thoracoscopic-resection-of-intralobar-and-extralobar-pulmonary-sequestration
#16
Andrea Dell'Amore, Domenica Giunta, Alessio Campisi, Stefano Congiu, Giampiero Dolci, Niccolò Antonino Barbera, Roberto Agosti, Francesco Buia
Pulmonary sequestration (PS) is a rare congenital malformation of the respiratory tract. Two main variants are described, the intralobar and the extralobar PS. Clinical manifestations vary from accidental findings to life threatening complications. Surgical resection is the definitive and indicated treatment of PS. The operation could be performed through an open thoracotomy or video-assisted thoracic surgery approach. We report the management of two patients with diagnosis of extralobar PS in the first case and intralobar PS in the second case...
2018: Journal of Visualized Surgery
https://www.readbyqxmd.com/read/29681956/congenital-cystic-adenomatoid-malformation-diagnostic-and-therapeutic-procedure-8-year-experience-of-one-medical-centre
#17
Bogumiła Strumiłło, Andrzej Jóźwiak, Anna Pałka, Krzysztof Szaflik, Anna Piaseczna-Piotrowska
Introduction: Congenital cystic adenomatoid malformation (CCAM) is a rare anomaly. The mechanisms and the time at which the abnormality develops are still unclear. The malformation is characterized by the presence of single large or multiple but smaller cysts. Aim: To present the experience of our medical centre, the Polish Mother's Memorial Hospital - Research Institute. Material and methods: We analysed the medical records of 32 neonates hospitalized in 2008-2017 at the Department of Paediatric Surgery and Urology ICZMP due to pre- or postnatally diagnosed congenital cystic adenomatoid malformation...
March 2018: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
https://www.readbyqxmd.com/read/29664316/endovascular-repair-of-interrupted-aortic-arch-approach-with-hope-for-fewer-complications
#18
Ata Firouzi, Bahram Mohebbi, Ali Shafiei
Interrupted aortic arch (IAA) is a rare congenital malformation defined as complete discontinuity between ascending and descending parts of aorta. We present a case of IAA, which was referred to us due to dilatation of proximal and mid parts of his thoracic aorta accompanied by narrowing of aorta proximal to the branching of the left subclavian artery. Further evaluation revealed interruption of aorta at the proximal part of descending thoracic aorta by a transverse septum along with several collateral formations...
December 23, 2017: Archives of Iranian Medicine
https://www.readbyqxmd.com/read/29664213/a-patient-centred-multi-domain-instrument-for-improving-the-clarity-of-outcomes-reporting-and-documentation-in-complex-airway-surgery
#19
S A R Nouraei, K J Heathcote
Laryngotracheal stenosis refers to abnormal narrowing of the central airways from larynx to carina. It is caused by an eclectic group of conditions that include intubation-related airway stenosis, granulomatosis with polyangiitis, tracheal compression or malignancy, and congenital laryngotracheal malformations. A significant proportion of patients with this condition can be satisfactorily treated using a combination of endoscopic and open surgical techniques and when definitive treatment is not possible, most of the remaining patients can be managed with a combination of endoscopic airway maintenance, stents, T-tubes, and/or tracheostomies...
April 17, 2018: Clinical Otolaryngology
https://www.readbyqxmd.com/read/29658947/pulmonary-sequestration-associated-with-congenital-pulmonary-airway-malformation
#20
Zahira A De León-Ureña, Stanislaw Sadowinski-Pine, Lourdes Jamaica-Balderas, Jaime Penchyna-Grub
Introduction: Congenital pulmonary malformations are a rare cause of neonatal morbidity. Some of them have a common origin, which allows the identification of combined lesions. Its diagnosis can be made prenatally by ultrasound, with the limitation that this study is performed in specialized centers and depends on the expertise of the operator. The association of pulmonary sequestration and congenital malformation of the airway has been described in approximately 40-60 cases since its first description in 1949...
2018: Boletín Médico del Hospital Infantil de México
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