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Congenital surgical malformation

Mohamad Abbass, Michael K Tso, Ezekiel Weis, Alim P Mitha
BACKGROUND: Orbital lymphaticovenous malformations (LVM) are congenital vascular lesions that are typically infiltrative in nature. There have been reports of orbital LVMs extending intracranially through orbital fissures, but there have been no reports of intradural extension. CASE DESCRIPTION: We report the first case of orbital LVM extending intradurally through a bony defect in the medial orbital roof. A modified orbitozygomatic approach was used to successfully obliterate this lesion...
March 16, 2018: World Neurosurgery
Antonio Borzelli, Andrea Paladini, Francesco Giurazza, Salvatore Tecame, Flavio Giordano, Enrico Cavaglià, Francesco Amodio, Fabio Corvino, Daniela Beomonte Zobel, Giulia Frauenfelder, Anna Giacoma Tucci, Raffaella Niola
Pulmonary sequestration is a congenital malformation characterized by dysplastic pulmonary tissue which receives blood supply by arterial systemic system, not in communication with tracheobronchial tree. Although it could be asymptomatic, it can also cause recurrent infections and hemoptysis, rarely massive and fatal. The conventional treatment consists in surgical resection of the pulmonary sequestration, but in the last few years endovascular embolization has been proposed as a valid therapeutic alternative...
February 2018: Radiology Case Reports
Dorit Schöller, Marieke Hölting, Diana Stefanescu, Helen Burow, Birgitt Schönfisch, Katharina Rall, Florin-Andrei Taran, Grigoris F Grimbizis, Attilio Di Spiezio Sardo, Sara Y Brucker
PURPOSE: Several classification systems for female genital tract anomalies exist but are of limited use in clinical practice. We, therefore, assessed the applicability and ease of use of the new ESHRE/ESGE classification, using only patient records. METHODS: This retrospective, single-center, proof-of-principle study systematically analyzed the surgical reports and other hospital records of 920 inpatients and outpatients treated for confirmed female genital tract congenital malformations at a major German university hospital during 2003-2013...
March 16, 2018: Archives of Gynecology and Obstetrics
Mireguli Tuersunjiang, Xing Long, Yuchuan Fu, Jin Ke, He Huijun, Jian Li
Bifid nose, an indicator of Tessier No.0, is a rare congenital malformation. Because of its rarity, few cases were reported and the optimal surgical procedure and the best time for surgery have not been widely acknowledged. In this brief report, a 9-year-old girl with mild bifid nose and unilateral mini-microform cleft lip, and its surgical management, is presented. We focused our attention on modifying the shape of the nose through open rhinoplasty without excising the surplus skin on the nasal dorsum and achieved good results...
March 14, 2018: Journal of Craniofacial Surgery
Rizwana Yasmin, Dorte R Stærk, Anna Kalhauge, Henrik J Hansen, Tina E Olsen, Lisa L Maroun
Bilateral pulmonary sequestration (PS) is a very rare congenital malformation. We describe a case of bilateral intralobar pulmonary sequestration (ILS) in a newborn. Both sequestrations received arterial supply from separate branches of the descending aorta and venous drainage was into ipsilateral inferior pulmonary veins. Prenatal ultrasonography showed cystic changes in the lungs. Computed tomography angiography (CTA) with supplemental two-dimensional (2D) and three-dimensional (3D) images was performed to clearly define the pathology and revealed bilateral intralobar pulmonary sequestration with aberrant blood supply...
March 2018: Acta Radiologica Open
Floor Wt Vergouwe, Madeleine Gottrand, Bas Pl Wijnhoven, Hanneke IJsselstijn, Guillaume Piessen, Marco J Bruno, René Mh Wijnen, Manon Cw Spaander
Esophageal atresia (EA) is one of the most common congenital digestive malformations and requires surgical correction early in life. Dedicated centers have reported survival rates up to 95%. The most frequent comorbidities after EA repair are dysphagia (72%) and gastroesophageal reflux (GER) (67%). Chronic GER after EA repair might lead to mucosal damage, esophageal stricturing, Barrett's esophagus and eventually esophageal adenocarcinoma. Several long-term follow-up studies found an increased risk of Barrett's esophagus and esophageal carcinoma in EA patients, both at a relatively young age...
March 7, 2018: World Journal of Gastroenterology: WJG
Joanna L Kaplan, Catherine T Gunther-Harrington, Jessie S Sutton, Joshua A Stern
BACKGROUND: The teratogenic effects of immunomodulatory and certain antimicrobial therapies are described in small rodents and humans. While the described teratogenic effects in small rodents have been extrapolated to make conclusions about its use in the pregnant dam, teratogenic effects of prednisone and doxycycline have not yet been reported in the dog. Here we report and describe midline defects observed in a litter of golden retriever puppies exposed to mid-gestational immunosuppressive and antimicrobial therapy...
March 12, 2018: BMC Veterinary Research
D Diaz-Aguilar, T Niu, S Terterov, R Scharnweber, A Tucker, J Woodard, H Brara, C Merna, H Shah, S Wang, S Rahman
Background: Neurenteric cysts (NECs) are rare developmental malformations of the central nervous system (CNS) which originate as benign congenital lesions. They originate from developmental foregut precursors, and are presumed to be the result of abnormal partitioning of the embryonic notochord plate. Such NECs predominantly arise in the cervical region in patients around 6 years of age or in their twenties or thirties. Notably, NECs of the conus medullaris are exceedingly rare, especially in patients of advanced age...
2018: Surgical Neurology International
Ali Mohtashami, Andrew Kiat, Jane Cross, Robert Simon, Austin Curtin
INTRODUCTION: Abernethy malformations are extremely rare congenital anomalous portosystemic shunts. We report the case of a patient with a rare variant Abernethy malformation between the superior mesenteric vein and left renal vein, associated with a massive jejunal diverticulum. PRESENTATION OF CASE: A 37-year-old Caucasian female presented to our emergency department with severe abdominal pain and proceeded to laparotomy for a presumed small bowel obstruction...
February 27, 2018: International Journal of Surgery Case Reports
Antonio F Corno, Saravanan Durairaj, Robert H Anderson
Aorto-ventricular tunnel is an extremely rare congenital heart defect, consisting of failure of attachment of an aortic leaflet along the semilunar hinge. In all published reports the leaflet involved was either the right coronary leaflet, most frequently, or the left coronary leaflet, in most of the cases opening toward the left ventricle, with only one-eighth of the reported cases communicating with the right ventricle. Treatment of the aorto-ventricular tunnel has been anecdotally reported by interventional closure with a device and more frequently with surgical approach, either as an isolated malformation or as associated lesions...
2018: Frontiers in Pediatrics
Mustafa Mert Başaran, Selmin Özgürsoy Karataylı, Arzu Pampal, Şefik Halit Akmansu
Lymphatic malformations (LM) are benign congenital malformations of the lymphatic system. They are frequently seen in the neck and sometimes can be life-threatening due to compression to the airway. Treatment modalities are widespread including surgical excision, radiotherapy, laser therapy, and application of intralesional sclerosing agents. We report the successful treatment of cervical LM in a 3-year-old boy who presented with a sudden onset of a large cystic mass in the posterior cervical triangle and was treated with repeated injections of intralesional bleomycin...
December 2017: Turkish Archives of Otorhinolaryngology
Eric B Jelin, Elizabeth M O'Hare, Tim Jancelewicz, Isam Nasr, Emily Boss, Daniel S Rhee
PURPOSE: We sought to determine optimal timing for CPAM resection within the first year of life. METHODS: We queried the National Surgical Quality Improvement Program pediatric database from 2012 to 2015 for elective CPAM resections on patients less than 1year of age. Patients were divided by age in months: 1-3 (n=57), 4-6 (n=135), and 6-12 (n=214). Patient operative variables and 30-day postoperative outcomes were compared. RESULTS: A total of 406 patients were included with no differences in demographics or comorbidities...
February 10, 2018: Journal of Pediatric Surgery
Omar Toumi, Mohamed Ali Chaouch, Abdesslem Ghedira, Ibtissem Korbi, Mohamed Nasr, Faouzi Noomene, Khadija Zouari, Randa Salem, Badii Hamida, Mondher Golli
INTRODUCTION: Congenital bile duct cysts (CBDC) is a rare congenital malformation. It results from an anomaly of the biliopancreatic junction (AJBP). This condition is often diagnosed at a young age. Although, it can be asymptomatic and discovered only at an advanced age. The aim of our work was to describe the diagnosis, therapeutic and evolutionary aspects of BVCD through a series of 11 cases diagnosed in adult cases. METHODS: This is a descriptive, retrospective and monocentric study...
June 2017: La Tunisie Médicale
Jim C H Wilde, Ana M Calinescu, Barbara E Wildhaber
Biliary atresia (BA) and congenital choledochal malformations (CCM) are rare. Both pathologies must (BA) or may (CCM) be operated during the neonatal period or early infancy. We briefly describe the classical operative techniques for both pathologies, followed by the most common and severe perioperative complications encountered during and up to 30 days after surgery in children operated for BA and CCM early in life. For patients with BA, intestinal complications represent the most common and hazardous perioperative surgical complications...
March 6, 2018: European Journal of Pediatric Surgery
Karina Miura Costa, Amulya Kumar Saxena
BACKGROUND: Postoperative chylothorax occurs due to trauma to lymphatic vessels and can occur after any thoracic procedure. This study reviewed recent literature to evaluate the management and outcomes of surgical chylothorax in neonates. METHODS: PubMed database was searched for articles in English, Portuguese and Spanish from 2000 to 2016. Data were collected for surgery, chylothorax management, complications, mortality and length of hospital stay (LOS). RESULTS: Twenty studies offered 107 neonates: congenital diaphragmatic hernia (CDH) (n = 76, 71%), cardiac malformations (n = 25, 23...
March 5, 2018: World Journal of Pediatrics: WJP
Tarun Raina Ramman, Nilanjan Dutta, Kuntal Roy Chowdhuri, Sunny Agrawal, Sumir Girotra, Sushil Azad, Sitaraman Radhakrishnan, Parvathi Unninayar Iyer, Krishna Subramony Iyer
Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects...
January 1, 2018: World Journal for Pediatric & Congenital Heart Surgery
Naziha Khen-Dunlop, Konstantinos Farmakis, Laureline Berteloot, Francesca Gobbo, Julien Stirnemann, Jacques De Blic, Francis Brunelle, Christophe Delacourt, Yann Revillon
OBJECTIVES: Bronchopulmonary sequestration (BPS) is the second most common congenital lung malformation, with an estimated incidence ranging from 0.15% to 1.8%. Surgical treatment is elective in patients with symptoms, but the management of asymptomatic patients remains controversial. METHODS: We retrospectively reviewed the medical records of 99 patients treated for BPS in our institution from January 2000 to December 2015. BPS was diagnosed prenatally in 86 (87%) cases...
February 28, 2018: European Journal of Cardio-thoracic Surgery
Nelly Reynaud, Frédérique Courtois, Pierre Mouriquand, Nicolas Morel-Journel, Kathleen Charvier, Marina Gérard, Alain Ruffion, Jean-Etienne Terrier
BACKGROUND: The bladder exstrophy-epispadias complex is a rare congenital malformation associated with severe dysfunction of the genital and urinary tracts and requiring a staged surgical reconstruction. AIM: The primary aims of this study were to report the sexuality, infertility, and urinary incontinence outcomes in a cohort of men born with bladder exstrophy-epispadias complex. The secondary aim was to highlight some predictive factors of infertility in this population...
March 2018: Journal of Sexual Medicine
Igino Simonetti, Piero Trovato, Francesco Verde, Luca Tarotto, Roberto Della Casa, Maria Concetta Lonardo, Gianfranco Vallone, Maria Grazia Caprio
Persistent urogenital sinus (PUGS) is a congenital pathological condition characterized by an abnormal communication between the urethra and vagina, which has an estimated incidence of 0.6/10,000 female births. It could be the only known malformation or part of a syndrome. PUGS is commonly shown by a pelvic mass, related to a distended bladder, hydrometrocolpos which is due to an obstruction leading to the dilation of the vagina and uterus (i.e., imperforate hymen, transverse vaginal septum or atresia, and PUGS) or both...
March 3, 2018: Journal of Ultrasound
Mohammed Ashi, Rehab Assur, Basim Awan, Hattan Aljaaly
INTRODUCTION: Split Hand-Foot malformation (SHFM) is a congenital limb defect that affects the central rays of the hands and/or feet. It is a rare condition that has genetic and environmental etiologies. It ranges in severity depending on the extent of the malformation. We report on two siblings with severe SHFM affecting all limbs. METHODS: We described two cases of siblings with SHFM and discuss the possible causes of the condition. This research did not require ethical approval due to the institute not requiring it for this type of study...
December 27, 2017: International Journal of Surgery Case Reports
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