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Congenital surgical malformation

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https://www.readbyqxmd.com/read/29150347/-perineal-groove-case-report-and-review-of-the-literature
#1
Y de La Monneraye, G Benoist, L Marchesi, A-S Crosnier, V Guinard-Samuel
Perineal groove is a rare anoperineal congenital malformation, usually occurring in girls, unknown to many pediatricians. We report the case of a 17-day-old girl admitted for urinary infection, who presented a wet sulcus with mucous membrane, extending from the vaginal fourchette to the anterior border of the anus. The diagnosis of perineal groove was clinically confirmed, after elimination of misdiagnosis such as infection or trauma. This benign malformation tends to be self-epithelialized in the 1st year of life, and no surgical treatment is recommended, except for cosmetic reasons or for recurrent infections...
November 14, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29147813/noninvasive-induction-of-angiogenesis-in-tissues-by-external-suction-sequential-optimization-for-use-in-reconstructive-surgery
#2
G Giatsidis, L Cheng, A Haddad, K Ji, J Succar, L Lancerotto, J Lujan-Hernandez, P Fiorina, H Matsumine, D P Orgill
In reconstructive surgery, tissues are routinely transferred to repair a defect caused by trauma, cancer, chronic diseases, or congenital malformations; surgical transfer intrinsically impairs metabolic supply to tissues placing a risk of ischemia-related complications such as necrosis, impaired healing, or infection. Pre-surgical induction of angiogenesis in tissues (preconditioning) can limit postsurgical ischemic complications and improve outcomes, but very few preconditioning strategies have successfully been translated to clinical practice due to the invasiveness of most proposed approaches, their suboptimal effects, and their challenging regulatory approval...
November 17, 2017: Angiogenesis
https://www.readbyqxmd.com/read/29137094/a-novel-non-contrast-enhanced-mra-using-silent-scan-for-evaluation-of-brain-arteriovenous-malformation-a-case-report-and-review-of-literature
#3
Jin Il Moon, Hye Jin Baek, Kyeong Hwa Ryu, Hyun Park
RATIONALE: Brain arteriovenous malformations (AVMs) are congenital vascular abnormalities involving abnormal connections between arteries and veins. In clinical practice, imaging studies help evaluate feeding arteries, niduses, draining venous systems, and coexisting complications in patients with brain AVM. They also have an impact on decision-making regarding clinical management. We applied a novel non-contrast-enhanced MR angiography (MRA) technique, termed "silent MRA," for evaluating an incidental brain AVM...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29136712/-long-term-outcome-and-prognostic-factors-in-pregnant-women-with-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#4
Q T Ou, J K Lu, J Zhang, Y Chen, Q Li, J L Zhang
Objective: To investigate the perinatal outcome, risk factors and long-term outcome of pregnancy complicated with pulmonary arterial hypertension(PAH) and congenital heart diseases (CHD). Methods: Clinical data of 110 pregnant women who were diagnosed as PAH-CHD were retrospectively analyzed in the Department of Obstetrics and Gynecology and Surgical Intensive Care Unit at Beijing Anzhen Hospital from 2004 to 2013. The survival and treatment status were followed up. Results: 110 subjects consisted of 11 mild PAH, 33 moderate and 66 severe ones...
November 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29131674/congenital-malformations-resembling-vacterl-association-in-a-golden-retriever
#5
Araceli Gamito-Gómez, Rodrigo Gutierrez-Quintana, Annette Wessmann
A 2 mo old golden retriever presented with malformation of the left thoracic limb and a small circular indentation of the skin in the cranial thoracic spine. Radiographs showed a cleft between the second and fifth metacarpal bones of the left thoracic limb compatible with ectrodactyly and spina bifida affecting T4 and T5 vertebrae. Magnetic resonance imaging of the thoracic spine showed dorsal reposition of the spinal cord and a tract connecting from the dura mater to the skin. No other malformations were detected...
November 13, 2017: Journal of the American Animal Hospital Association
https://www.readbyqxmd.com/read/29127963/minimally-invasive-surgery-in-the-management-of-anorectal-malformations
#6
REVIEW
Sarah B Cairo, David H Rothstein, Carroll M Harmon
Imperforate anus, a variant of anorectal malformation (ARM), is a common congenital anomaly requiring surgical attention in the newborn period. It may present with a variety of anatomic configurations, largely dependent on the presence and location of a fistula. The location (or characteristics) of a fistula, which usually lies between the gastrointestinal tract and the genitourinary tract or perineum, is often used in determining the type and timing of operative management. This article discusses the work-up and management, modes of treatment and their postoperative outcomes, and continued controversy regarding the use of minimally invasive surgical approaches to ARM...
December 2017: Clinics in Perinatology
https://www.readbyqxmd.com/read/29125417/loss-of-mechanosensitive-sclerostin-may-accelerate-cranial-bone-growth-and-regeneration
#7
Kyung Shin Kang, Jeff Lastfogel, Laurie L Ackerman, Andrew Jea, Alexander G Robling, Sunil S Tholpady
OBJECTIVE Cranial defects can result from trauma, infection, congenital malformations, and iatrogenic causes and represent a surgical challenge. The current standard of care is cranioplasty, with either autologous or allogeneic material. In either case, the intrinsic vascularity of the surrounding tissues allows for bone healing. The objective of this study was to determine if mechanotransductive gene manipulation would yield non-weight-bearing bone regeneration in a critical size calvarial defect in mice. METHODS A mouse model of Sost deletion in Sost knockout (KO) mice was created in which the osteocytes do not express sclerostin...
November 10, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29121837/triple-outlet-right-ventricle-with-duplication-of-the-aortic-root-and-intrapericardial-ascending-aorta
#8
Ashish Katewa, Balswaroop Sahu, Vishal Jain, Robert H Anderson
We present a case of a highly unusual congenital cardiac malformation, namely, triple outlet right ventricle with duplication of the aortic root and the intrapericardial component of the ascending aorta. A girl, aged five, presented with complaints of cyanosis and effort intolerance and was diagnosed with double-outlet right ventricle and subpulmonary infundibular stenosis. Intraoperatively, we noted that the aortic root was guarded by two separate aortic valves, oriented anteroposteriorly relative to each other, and separated within the right ventricle by a muscle bar...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29111080/morbidity-and-mortality-among-big-babies-who-develop-necrotizing-enterocolitis-a-prospective-multicenter-cohort-analysis
#9
Cristine S Velazco, Brenna S Fullerton, Charles R Hong, Kate A Morrow, Erika M Edwards, Roger F Soll, Tom Jaksic, Jeffrey D Horbar, Biren P Modi
BACKGROUND: Necrotizing enterocolitis (NEC) is classically a disease of prematurity, with less reported regarding morbidity and mortality of this disease among other infants. METHODS: Data were prospectively collected from 2009 to 2015 at 252 Vermont Oxford Network member centers on neonates with birth weight>2500g admitted to a participating NICU within 28days of birth. RESULTS: Of 1629 neonates with NEC, gestational age was 37 (36, 39) weeks, and 45% had major congenital anomalies, most commonly gastrointestinal defects (20%), congenital heart defects (18%), and chromosomal anomalies (7%)...
October 12, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29110824/long-term-psychosocial-consequences-of-surgical-congenital-malformations
#10
Trond H Diseth, Ragnhild Emblem
Surgical congenital malformations often represent years of treatment, large number of hospital stays, treatment procedures, and long-term functional sequels affecting patients' psychosocial functioning. Both functional defects and psychosocial difficulties that occur commonly in childhood may pass through adolescence on to adulthood. This overview presents reports published over the past 3 decades to elucidate the long-term psychosocial consequences of surgical congenital malformations. Literature searches conducted on PubMed database revealed that less than 1% of all the records of surgical congenital malformations described long-term psychosocial consequences, but with diverse findings...
October 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29108843/outcomes-following-elective-resection-of-congenital-pulmonary-airway-malformations-are-equivalent-after-3-months-of-age-and-a-weight-of-5-kg
#11
Brian C Gulack, Harold J Leraas, Brian Ezekian, Jina Kim, Christopher Reed, Obinna O Adibe, Henry E Rice, Elisabeth T Tracy
PURPOSE: Resection of congenital pulmonary airway malformations (CPAMs) is often performed to reduce the risk of recurrent infection and malignant transformation. However, there is substantial variation in the timing of resection. This study was performed to determine the association of age and weight on outcomes following elective resection of CPAMs. METHODS: The American College of Surgeons National Surgical Quality Improvement Program-Pediatric database from 2012 to 2014 was queried for infants undergoing elective resection of a CPAM...
October 9, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29081395/intradural-extramedullary-bronchogenic-cyst-clinical-and-radiological-characteristics-surgical-outcomes-and-literature-review
#12
Jian-Cong Weng, Jun-Peng Ma, Shu-Yu Hao, Liang Wang, Zhen Wu, Li-Wei Zhang, Da Li, Jun-Ting Zhang
OBJECTIVE: An intradural extramedullary bronchogenic cyst (IEBC) is a congenital malformation and an extremely rare type of endodermal cyst. This study aims to report the clinical and radiological characteristics and surgical outcomes of IEBCs and to review the available literature. METHODS: The medical records of 6 patients (3 females) with pathologically confirmed bronchogenic cysts involving the spinal cord between 2009 and 2016 were retrospectively reviewed, and follow-up evaluations were performed...
October 25, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29079318/transitions-in-care-from-pediatric-to-adult-general-surgery-evaluating-an-unmet-need-for-patients-with-anorectal-malformation-and-hirschsprung-disease
#13
Sarah B Cairo, Priscilla P L Chiu, Roshni Dasgupta, Karen A Diefenbach, Allan M Goldstein, Nicholas A Hamilton, Andrea Lo, Michael D Rollins, David H Rothstein
BACKGROUND: The provision of timely and comprehensive transition of care from pediatric to adult surgical providers for patients who have undergone childhood operations remains a challenge. Understanding the barriers to transition from a patient and family perspective may improve this process. METHODS: A cross-sectional survey was conducted of patients with a history of anorectal malformation (ARM) or Hirschsprung Disease (HD) and their families. The web-based survey was administered through two support groups dedicated to the needs of individuals born with these congenital abnormalities...
October 7, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29071783/age-is-not-a-good-predictor-of-irreversibility-of-pulmonary-hypertension-in-congenital-cardiac-malformations-with-left-to-right-shunt
#14
Amir-Reza Hosseinpour, Marie-Hélène Perez, David Longchamp, Jacques Cotting, Nicole Sekarski, Michel Hurni, René Prêtre, Stefano Di Bernardo
OBJECTIVE: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here. DESIGN: A retrospective observational study...
October 25, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/29062381/a-rare-case-of-pulmonary-artery-sling-with-the-vacterl-association-in-a-20-month-old-infant
#15
Yazdan Ghandi, Akbar Shafiee, Mehrazad Sharifi, Najmeh Sadat Bolandnazar
The VACTERL association, co-occurrence of vertebral, anorectal, cardiac, tracheoesophageal, genitourinary, and limb malformations, is a rare congenital anomaly. Several cardiac anomalies have been reported as a part of the VACTERL association, particularly ventricular and atrial septal defects. Pulmonary artery sling is a rare congenital abnormality in which the left pulmonary artery arises from the right pulmonary artery. This anomaly is not frequently observed in the VACTERL association and has been rarely reported...
July 2017: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/29058584/radiographic-characteristics-in-congenital-scoliosis-associated-with-split-cord-malformation-a-retrospective-study-of-266-surgical-cases
#16
Fan Feng, Haining Tan, Xingye Li, Chong Chen, Zheng Li, Jianguo Zhang, Jianxiong Shen
BACKGROUND: Vertebrae, ribs, and spinal cord are anatomically adjacent structures, and their close relationships are clinically important for planning better corrective surgical approach. The objective is to identify the radiographic characteristics in surgical patients with congenital scoliosis (CS) and coexisting split cord malformation (SCM). METHODS: A total of 266 patients with CS and SCM underwent surgical treatment at our hospital between May 2000 and December 2015 was retrospectively identified...
October 23, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/29057367/an-unusual-case-of-intradural-intramedullary-dorsal-bronchogenic-cyst-in-spine
#17
Tarun Dusad, Vishal Kundnani, Shumayou Dutta, Ankit Patel, Gaurav Mehta, Mahendra Singh
Bronchogenic cysts are congenital malformations derived from anomalous budding of the embryonic foregut. Intraspinal bronchogenic cysts are extremely rare and most of them are extramedullary. There has been only one case of intramedullary spinal bronchogenic cyst reported. We present an 18-year-old male patient with spastic paraparesis and bowel and bladder involvement. MRI revealed a 2 cm diameter intradural and intramedullary lesion at D2-D3 level which was hyperintense on T1 and hypointense on T2 imaging...
September 2017: Journal of Spine Surgery (Hong Kong)
https://www.readbyqxmd.com/read/29057363/congenital-malformed-posterior-arch-of-atlas-with-fusion-defect-a-case-of-developmental-canal-stenosis-causing-cervical-myelopathy
#18
Siddharth Shah, Samir Dalvie, Ravi Ranjan Rai
Congenital anomalies of the posterior arch of the atlas (PAA) are usually asymptomatic and diagnosed incidentally. Very rarely, they present with cervical myelopathy, usually being associated with partial aplasia or agenesis of PAA. We describe a 44-year-old lady with cervical myelopathy secondary to a malformed PAA with developmental atlas-level spinal stenosis and a congenital posterior fusion defect with persistent midline cleft showing significant non-osseous fibro-cartilaginous hypertrophy, causing critical cord compression...
September 2017: Journal of Spine Surgery (Hong Kong)
https://www.readbyqxmd.com/read/29049244/isolated-heptadactylia-a-case-report-of-central-polydactyly-of-the-foot
#19
Nicolas Piette, Pierre-Yves Zambelli, Daniel N'Dele
RATIONALE: Heptadactylia is a rare congenital disorder from the polydactyly family. Polydactyly is generally classified into 3 major groups: preaxial (medial ray), postaxial (lateral ray), and central polydactyly. Most common cases are related to preaxial or postaxial polydactyly. The rarity of central polydactyly can be explained in 3 ways. First, central polydactyly with duplication appearing on metatarsal is pretty uncommon. Second, the duplication appears isolated on the foot. Polydactyly is mostly associated with other physical defects or others duplications...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29046943/congenital-pulmonary-airway-malformations-state-of-the-art-review-for-pediatrician-s-use
#20
REVIEW
Claire Leblanc, Marguerite Baron, Emilie Desselas, Minh Hanh Phan, Alexis Rybak, Guillaume Thouvenin, Clara Lauby, Sabine Irtan
Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are diagnosed prenatally, improving the prenatal and immediate postnatal care and ultimately the knowledge on CPAM pathophysiology. CPAM natural evolution can lead to infections or malignancies, whose exact prevalence is still difficult to assess. The aim of this "state-of-the-art" review is to cover the recently published literature on CPAM management whether the pulmonary lesion was detected during pregnancy or after birth, the current indications of surgery or surveillance and finally its potential evolution to pleuro-pulmonary blastoma...
December 2017: European Journal of Pediatrics
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