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Congenital surgical malformation

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https://www.readbyqxmd.com/read/28106514/endonasal-management-of-pediatric-congenital-transsphenoidal-encephaloceles-nuances-of-a-modified-reconstruction-technique-technical-note-and-report-of-3-cases
#1
Mehdi Zeinalizadeh, Seyed Mousa Sadrehosseini, Zohreh Habibi, Farideh Nejat, Harley Brito da Silva, Harminder Singh
OBJECTIVE Congenital transsphenoidal encephaloceles are rare malformations, and their surgical treatment remains challenging. This paper reports 3 cases of transsphenoidal encephalocele in 8- to 24-month-old infants, who presented mainly with airway obstruction, respiratory distress, and failure to thrive. METHODS The authors discuss the surgical management of these lesions via a minimally invasive endoscopic endonasal approach, as compared with the traditional transcranial and transpalatal approaches. A unique endonasal management algorithm for these lesions is outlined...
January 20, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28101147/a-double-gallbladder-with-a-common-bile-duct-stone-treated-by-laparoscopy-accompanied-by-choledochoscopy-via-the-cystic-duct-a-case-report
#2
Wei Yu, Huisheng Yuan, Shi Cheng, Ying Xing, Wenmao Yan
A double gallbladder is a rare congenital malformation. The present study describes a case of double gallbladder with secondary common bile duct stones. By way of laparoscopic choledochoscopy, the exploration and removal of a common bile duct stone was performed through the cystic duct. The process involved a primary suture of the cystic duct and was performed without using a T-tube, and completed a surgical removal of the gallbladder. The present case was successfully treated by laparoscopic surgery. From a review of previous studies published in the English language, this study, to the best of our knowledge, is the first report of such a case...
December 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28092970/surgery-of-anterior-skull-base-lesions-in-children
#3
Thomas Karl Hoffmann, Marc Oliver Scheithauer, Fabian Sommer, Jörg Lindemann, Ernst-Johannes Haberl, Ulrike Friebe-Hoffmann, Marie-Nicole Theodoraki
INTRODUCTION: Lesions affecting the anterior skull base represent a rare group of craniofacial pathologies. A tumor of the facial midline, meningitis, or rhinoliquorrhea may be indicative of malformations like dermoid cysts, gliomas, encephaloceles, or nasal fistulas. METHODS: We present a case series of 13 children (4 months to 12 years; 8 males, 5 females) with lesions involving the anterior skull base, which were treated surgically in an interdisciplinary setting...
January 1, 2017: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/28087956/intralesional-injection-of-ok-432-in-cystic-hygroma
#4
M Hazim, S S Moses, I P Tan
BACKGROUND: Lymphangiomas are congenital malformations of the lymphatic system with characteristic dilated endothelium-lined spaces. It is vulnerability to infection or chemical irritants cause spontaneous reduction in size and in some cases complete resolution. Intralesional injection of OK-432 or Picibanil (lyophilized incubation mixture of Group A Streptococcus pyogenes of human origin) is slowly gaining recognition as its safety and efficacy standards have shown to avoid complications resulting from surgical interventions...
December 2016: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28087231/psychosocial-outcome-in-adult-men-born-with-hypospadias
#5
L Örtqvist, M Andersson, A Strandqvist, A Nordenström, L Frisén, G Holmdahl, A Nordenskjöld
INTRODUCTION: Hypospadias, which is a surgically treated congenital malformation of the male urethra, may have a negative impact on quality of life. This aspect has previously been subject to limited research. This study examined the long-term psychosocial outcome of a large cohort of adult males born with hypospadias. OBJECTIVE: The purpose of this case-control study was to assess a possible negative influence on the psychosocial outcome in adult males with hypospadias...
September 9, 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28066618/tracheal-and-bronchial-stenoses-and-other-obstructive-conditions
#6
REVIEW
Claudia Schweiger, Aliza P Cohen, Michael J Rutter
Although tracheal stenosis and bronchial stenosis are relatively rare in the pediatric population, they are both associated with significant morbidity and mortality. While most cases of congenital tracheal stenosis in children present as complete tracheal rings (CTRs), other congenital tracheal obstructions are also encountered in clinical practice. In addition, acquired obstructive tracheal conditions stemming from endotracheal trauma or previous surgical interventions may occur. Many affected children also have associated cardiovascular malformations, further complicating their management...
November 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28064423/pathophysiology-diagnosis-and-management-of-glaucoma-associated-with-sturge-weber-syndrome
#7
REVIEW
Usman Javaid, Muhammad Hassaan Ali, Samreen Jamal, Nadeem Hafeez Butt
PURPOSE: Sturge-Weber syndrome (SWS), also known as encephalotrigeminal angiomatosis, is a condition which includes leptomeningeal hemangioma, facial angiomatosis or nevus flammeus, and ocular changes. SWS can lead to severe complications of anterior segment involving conjunctiva and eyelids, whereas posterior segment of the eye may also be affected by diffuse choroidal hemorrhages. This article was written with the objectives to determine the pathophysiology, diagnosis, and treatment of glaucoma associated with this rare and challenging disorder...
January 7, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28064298/case-report-of-a-chest-wall-cystic-hygroma-in-a-teenager
#8
K Aimanan, M P Putera, L G Chew, T Ramesh, M I Muhd Azim, B Badmanaban
Cystic hygroma or cystic lymphangioma is a congenital malformation of lymphatic origin. Their occurrence on the chest wall is very rare, and they progressively grow with age infiltrating into the local tissues, around muscle fibers and nerves, making them difficult and hazardous to remove. There are various treatment modalities of such lesion. Based on the literature surgical excision is the preferred treatment of choice in cystic hygroma because it gives a better cure rate compared to other modalities. We report a case successful excision of anterolateral chest wall cystic hygroma in a teenager in Hospital Serdang...
October 2016: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28050458/congenital-cystic-adenomatoid-malformation-a-tertiary-care-hospital-experience
#9
Asmita Anilkumar Mehta, Naveen Viswanathan, Anil Kumar Vasudevan, Roopa Paulose, Mohan Abraham
INTRODUCTION: Congenital Cystic Adenomatoid Malformation (CCAM) is an uncommon developmental deformity affecting the terminal respiratory structures. It is characterized by broncho pulmonary foregut malformations. The reason behind it is an arrest in lung development between 4(th) and 7(th) week of fetal life. AIM: The present study was conducted to assess the clinical and radiological profile and also to study the role of surgical intervention in patients with CCAM...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28049537/imperforated-cor-triatriatum-dexter-in-a-dog-with-concurrent-caudal-vena-cava-wall-mineralization
#10
Tetyda Paulina Dobak, Gregory Starrak, Kathleen Linn, Elisabeth Christine Roberston Snead
BACKGROUND: Cor triatriatum dexter (CTD) is a rare congenital cardiac malformation with various manifestations and has been sporadically described in dogs. Clinically the dogs present with nonspecific signs of right heart failure or Budd-Chiari-like syndrome. Other associated concurrent cardiovascular anomalies are commonly reported. Diagnosis and full characterization of this complex malformation requires careful investigation and often a multimodal imaging approach. CASE PRESENTATION: A 10-week-old, male intact, Golden Retriever was presented with clinical signs of stunted growth, anorexia, and progressive ascites...
January 3, 2017: Acta Veterinaria Scandinavica
https://www.readbyqxmd.com/read/28045821/treatment-of-congenital-choanal-atresia-via-transnasal-endoscopic-method
#11
Secaattin Gulşen, Elif Baysal, Fatih Celenk, Ismail Aytaç, Cengiz Durucu, Muzaffer Kanlikama, Semih Mumbuç
OBJECTIVES: Congenital choanal atresia (CCA) is a very rare abnormality of the nose, but in the case of bilateral presence, it becomes a life-threatening malformation. Various surgical treatment options, such as transpalatal, transseptal, and open rhinoplasty techniques, as well as the transnasal approach, have been defined for the repair of CCA. In this study, the authors intended to evaluate the outcomes of transnasal endoscopic surgery for CCA, and stent implementation's impact on surgical success...
December 30, 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28043459/repair-of-parachute-and-hammock-valve-in-infants-and-children-early-and-late-outcomes
#12
Eva Maria Delmo Walter, Mariano Javier, Roland Hetzer
Parachute and hammock valves in children remain one of the most challenging congenital malformations to correct. We report our institutional experience with valve-preserving repair techniques and the early and late surgical outcomes in parachute and hammock valves in infants and children. From January 1990-June 2014, 20 infants and children with parachute (n = 12, median age = 2.5 years, range: 2 months-13 years) and hammock (n = 8, median age = 7 months, range: 1 month-14.9 years) valves underwent mitral valve (MV) repair...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28040760/efficacy-of-segmental-resection-in-patients-with-prenatally-diagnosed-congenital-lung-malformations
#13
Sungkwang Lee, Do Hyung Kim, Sang Kwon Lee
OBJECTIVES: Lung segmental resection is a better treatment option than lobectomy for patients with prenatally diagnosed congenital lung malformations (CLMs). However, data are lacking on the effects of this procedure in prenatally diagnosed CLM patients. In this study, we explored whether parenchyma-saving resection was feasible in patients with this condition. METHODS: A retrospective analysis was performed on 27 patients prenatally diagnosed with CLM, who subsequently underwent surgery between March 2011 and September 2015...
December 31, 2016: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28032156/complex-truncal-masses-in-the-setting-of-cloves-syndrome-aesthetic-and-functional-implications
#14
Jason M Weissler, Valeriy Shubinets, Martin J Carney, David W Low
BACKGROUND: Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal abnormalities (CLOVES) is a complex overgrowth syndrome with dramatic aesthetic and functional implications. The truncal masses characteristic of CLOVES syndrome are described as vascular malformations or lipomatous lesions with variable vascular components. Herein, we describe our single-institution experience with surgical excision of CLOVES-related truncal masses and discuss future directions in treatment of these complex anomalies...
December 28, 2016: Aesthetic Plastic Surgery
https://www.readbyqxmd.com/read/28031647/are-congenital-arteriovenous-malformations-of-proximal-upper-extremity-more-on-the-right-case-report-and-literature-review
#15
Satyen Parida, Sumanlata Gupta, B V Sai Chandran
This is a case of a 9-year-old boy with a congenital arteriovenous malformation (AVM) of the right upper extremity arising from the right subclavian artery. He underwent open surgical excision of the vascular tumor. Upon reviewing the literature, a high incidence of right-sided congenital AVM of the proximal upper extremity was identified. It has been suggested that this high incidence may be related to the complexity of the embryologic development of the right subclavian artery.
December 2016: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
https://www.readbyqxmd.com/read/28027255/evidence-based-medicine-cleft-palate
#16
Albert S Woo
LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Describe the incidence of cleft palate and risk factors associated with development of an orofacial cleft. 2. Understand differences among several techniques to repair clefts of both the hard and soft palates. 3. Discuss risk factors for development of postoperative fistulas, velopharyngeal insufficiency, and facial growth problems. 4. Establish a treatment plan for individualized care of a cleft palate patient...
January 2017: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28027181/treatment-of-the-crooked-nose-the-final-steps-to-perfection
#17
Michele Pascali, Armando Boccieri, Francesco Carinci, Valerio Cervelli
Perfection is sometimes approached in treatment of the crooked nose today but not fully achieved due to the continued existence of flaws. While the traditional surgical algorithm envisages the use of 2 series of procedures to straighten the nasal bones and cartilaginous septum, the addition of a third appears very useful with a view to obtaining truly excellent results, above all in the case of marked deviation. The authors present their experience in the use of certain procedures designed to correct asymmetry of the upper lateral and lower lateral cartilages, as well as the soft covering tissues where necessary...
December 23, 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28018799/caudal-duplication-syndrome-the-vital-role-of-a-multidisciplinary-approach-and-staged-correction
#18
Inbal Samuk, Marc Levitt, Elena Dlugy, Dragan Kravarusic, David Ben-Meir, Gustavo Rajz, Osnat Konen, Enrique Freud
Caudal duplication syndrome is a rare entity that describes the association between congenital anomalies involving caudal structures and may have a wide spectrum of clinical manifestations. A full-term male presented with combination of anomalies including anorectal malformation, duplication of the colon and lower urinary tract, split of the lower spine, and lipomyelomeningocele with tethering of the cord. We report this exceptional case of caudal duplication syndrome with special emphasis on surgical strategy and approach combining all disciplines involved...
December 2016: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28018469/type-i-chiari-malformation-presenting-orthostatic-syncope-who-treated-with-decompressive-surgery
#19
Hyun-Seung Shin, Jeong A Kim, Dong-Seok Kim, Joon Soo Lee
Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28011359/detection-of-a-rare-complication-of-endovascular-treatment-for-brain-avm-by-echocardiography
#20
Mozhgan Parsaee, Sedigheh Saedi, Sepideh Emami, Alireza Serati
A brain arteriovenous malformation (AVM) is a congenital malformation of the vascular system, in which an abnormal short circuit (vascular malformation) connects cerebral arteries and veins. When the brain AVMs are diagnosed, the majority of them must be closed with surgical or endovascular treatment; because only a small portion of the brain AVMs remain asymptomatic during the patient's life. Endovascular trans-arterial embolization using Onyx is a suitable method of treating brain AVMs. Previously known complications of onyx usage are bleeding and neurologic deficits...
December 21, 2016: World Neurosurgery
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