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Da Li, Yu-Ming Jiao, Liang Wang, Fu-Xin Lin, Jun Wu, Xian-Zeng Tong, Shuo Wang, Yong Cao
OBJECTIVE Surgical management of brainstem lesions is challenging due to the highly compact, eloquent anatomy of the brainstem. This study aimed to evaluate the safety and efficacy of preoperative diffusion tensor imaging (DTI) and diffusion tensor tractography (DTT) in brainstem cavernous malformations (CMs). METHODS A prospective randomized controlled clinical trial was performed by using stratified blocked randomization. The primary eligibility criterion of the study was being a surgical candidate for brainstem CMs (with informed consent)...
March 16, 2018: Journal of Neurosurgery
J J Brudvig, J T Cain, G G Schmidt-Grimminger, D J Stumpo, K J Roux, P J Blackshear, J M Weimer
Axons of the corpus callosum (CC), the white matter tract that connects the left and right hemispheres of the brain, receive instruction from a number of chemoattractant and chemorepulsant cues during their initial navigation towards and across the midline. While it has long been known that the CC is malformed in the absence of Myristoylated alanine-rich C-kinase substrate (MARCKS), evidence for a direct role of MARCKS in axon navigation has been lacking. Here, we show that MARCKS is necessary for Netrin-1 (NTN1) signaling through the DCC receptor, which is critical for axon guidance decisions...
March 15, 2018: Molecular Neurobiology
Takeshi Konuma, Syunsuke Sakamoto, Syuhei Toba, Ayano Futsuki, Naoki Yamamoto, Shinji Kanemitsu, Hideto Shimpo
OBJECTIVES: Postoperative left pulmonary artery (PA) or bronchus compression occasionally follows narrowing of the retroaortic space after the Norwood procedure. We examined hypoplastic left heart syndrome (HLHS)-specific anatomical malformations and hypothesized that a long main pulmonary arterial trunk might contribute to a larger retroaortic space. In this study, we introduced a modified Norwood procedure [pulmonary artery trunk-saving method (PATS)] and evaluated the results. METHODS: HLHS-specific anatomical malformations were examined using computed tomographic data from 23 consecutive patients who underwent bilateral pulmonary banding...
March 12, 2018: Interactive Cardiovascular and Thoracic Surgery
E Verheij, L Elden, T B Crowley, F A Pameijer, E H Zackai, D M McDonald-McGinn, H G X M Thomeer
BACKGROUND AND PURPOSE: The 22q11.2 deletion syndrome is characterized by a heterogenic phenotype, including hearing loss. The underlying cause of hearing loss, especially sensorineural hearing loss, is not yet clear. Therefore, our objective was to describe anatomic malformations in the middle and inner ear in patients with 22q11.2 deletion syndrome. MATERIALS AND METHODS: A retrospective case series was conducted in 2 tertiary referral centers. All patients with 22q11...
March 15, 2018: AJNR. American Journal of Neuroradiology
T N Booth, C Wick, R Clarke, J W Kutz, M Medina, D Gorsage, Y Xi, B Isaacson
BACKGROUND AND PURPOSE: Cochlear malformations may be be subtle on imaging studies. The purpose of this study was to evaluate the angle and depth of the lateral second interscalar ridge or notch in ears without sensorineural hearing loss (normal ears) and compare them with ears that have a documented incomplete type II partition malformation. MATERIALS AND METHODS: The second interscalar ridge notch angle and depth were measured on MR imaging in normal ears by a single experienced neuroradiologist...
March 15, 2018: AJNR. American Journal of Neuroradiology
Erol Arslan, Selim Büyükkurt, Mete Sucu, Mehmet Özsürmeli, Selahattin Mısırlıoğlu, S Cansun Demir, I Cüneyt Evrüke
OBJECTIVE: Fetal structural malformations affect approximately 2-3% of all pregnancies. Only focusing on trisomy screening in first trimester and deferring the anatomical screening to second trimester may result with late detection of major anomalies that can be diagnosed earlier with a careful examination. MATERIAL AND METHODS: This was a descriptive study retrospective data that was obtained from all terminated single pregnancies due to ultrasonographic findings of major anomalies from 2011 to 2016 in our department...
March 16, 2018: Journal of the Turkish German Gynecological Association
Sharad Rajpal, Colson Tomberlin, Andrew Bauer, Robert C Forsythe, Sigita Burneikiene
BACKGROUND: The variety of diagnostic characteristics associated with neurocysticercosis have been well studied, however, its potential to be implicated in other differential diagnosis has not been well demonstrated. CASE DESCRIPTION: We report the case of a 55-year-old Hispanic man who underwent a Chiari decompression surgery, which was complicated with hydrocephalus. Despite a ventriculoperitoneal (VP) shunt placement, he continued to have headaches and was soon found to have several skull base subarachnoid lesions, which were later diagnosed as the sequelae of an active neurocysticercosis infection...
March 12, 2018: World Neurosurgery
Mio Noma, Muneaki Matsubara, Chiho Tokunaga, Tomomi Nakajima, Bryan James Mathis, Hiroaki Sakamoto, Yuji Hiramatsu
BACKGROUND: Although pulmonary artery banding (PAB) is a common palliative procedure for pediatric heart malformation, there are concerns of pressure overload and concomitant immune reactions in the right ventricle causing postsurgical complications such as pericardial effusion. At this time, no clear guidelines as to potential risk factors or procedural contraindications have been widely disseminated. Therefore, a study was undertaken to examine wide-ranging factors to find potential biomarkers for postsurgical pericardial effusion formation risk...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
Wibke Uller, Sherif El-Sobky, Ahmad I Alomari, Steven J Fishman, Samantha A Spencer, Amir H Taghinia, Gulraiz Chaudry
PURPOSE: The purpose of this study was to evaluate the safety and efficacy of preoperative percutaneous n-butyl cyanoacrylate (nBCA) embolization of venous malformations in children. MATERIAL AND METHODS: Clinical data were retrospectively reviewed in children who underwent embolization using nBCA followed by resection of venous malformations. RESULTS: A total of 17 embolizations were performed in 14 patients (9 females, mean age: 5.5 years; median age: 3 years; range 0...
January 1, 2018: Vascular and Endovascular Surgery
Donal Bisanzio, Felipe Dzul-Manzanilla, Hector Gomez-Dantés, Norma Pavia-Ruz, Thomas J Hladish, Audrey Lenhart, Jorge Palacio-Vargas, Jesus F González Roldan, Fabian Correa-Morales, Gustavo Sánchez-Tejeda, Pablo Kuri Morales, Pablo Manrique-Saide, Ira M Longini, M Elizabeth Halloran, Gonzalo M Vazquez-Prokopec
Response to Zika virus (ZIKV) invasion in Brazil lagged a year from its estimated February 2014 introduction, and was triggered by the occurrence of severe congenital malformations. Dengue (DENV) and chikungunya (CHIKV) invasions tend to show similar response lags. We analyzed geo-coded symptomatic case reports from the city of Merida, Mexico, with the goal of assessing the utility of historical DENV data to infer CHIKV and ZIKV introduction and propagation. About 42% of the 40,028 DENV cases reported during 2008-2015 clustered in 27% of the city, and these clustering areas were where the first CHIKV and ZIKV cases were reported in 2015 and 2016, respectively...
March 15, 2018: PLoS Neglected Tropical Diseases
Frank Niemeyer, Lutz Claes, Anita Ignatius, Nicholaus Meyers, Ulrich Simon
Distraction osteogenesis is an effective method for generating large amounts of bone in situ for treating pathologies such as large bone defects or skeletal malformations, for instance leg-length discrepancies. While an optimized distraction procedure might have the potential to reduce the rate of complications significantly, our knowledge of the underlying mechanobiological processes is still insufficient for systematic optimization of treatment parameters such as distraction rate or fixation stiffness. We present a novel numerical model of lateral distraction osteogenesis, based on a mechanically well-controlled in vivo experiment...
2018: PloS One
Plansky Hoang, Jason Wang, Bruce R Conklin, Kevin E Healy, Zhen Ma
The creation of human induced pluripotent stem cells (hiPSCs) has provided an unprecedented opportunity to study tissue morphogenesis and organ development through 'organogenesis-in-a-dish'. Current approaches to cardiac organoid engineering rely on either direct cardiac differentiation from embryoid bodies (EBs) or generation of aligned cardiac tissues from predifferentiated cardiomyocytes from monolayer hiPSCs. To experimentally model early cardiac organogenesis in vitro, our protocol combines biomaterials-based cell patterning with stem cell organoid engineering...
April 2018: Nature Protocols
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
Rajani Singh, Rajnish Arora, Raj Kumar
Chiari malformations (CMs) are variant structural setup of cerebellum and brain stem at the craniovertebral junction. Normally the cerebellum and parts of the brain stem lie above the foramen magnum. When the part of the cerebellum and/or brainstem protrudes into the upper spinal canal through foramen magnum, it is defined as CM. Chiari malformations may develop when part of the skull is smaller than normal or misshapen, due to which the cerebellum herniates into spinal canal through foramen magnum. This compresses the cerebellum and brainstem affecting functions controlled by these parts and blocks the flow of cerebrospinal fluid that surrounds and cushions the brain and spinal cord...
March 14, 2018: Journal of Craniofacial Surgery
Jun Ma, Bin Chen, Dong Li, Yue Zhang, Zhaoxia Ying
Port-wine stain (PWS) birthmark is a congenital microvascular malformation of the skin. A 1064-nm Nd:YAG laser can achieve a deeper treatment, but the weak absorption by blood limits its clinical application. Multiple laser pulses (MLPs) are a potential solution to enhance the curative effect of a Nd:YAG laser. To reduce the pulse number (pn ) required for the thermal destruction of the blood vessel, the effect of glucose in conjunction with MLP was investigated. In vivo experiments were performed on a dorsal skin chamber model...
March 14, 2018: Lasers in Medical Science
Masashi Shimohira, Tatsuya Kawai, Takuya Hashizume, Masahiro Muto, Masanori Kitase, Yuta Shibamoto
The article "Usefulness of Hydrogel-Coated Coils in Embolization of Pulmonary Arteriovenous Malformations," written by Masashi Shimohira, Tatsuya Kawai, Takuya Hashizume, Masahiro Muto, Masanori Kitase and Yuta Shibamoto, was originally published electronically on the publisher's Internet portal (currently SpringerLink) on January 17, 2018, without open access.
March 14, 2018: Cardiovascular and Interventional Radiology
Ibtessam R Hussein, Rima S Bader, Adeel G Chaudhary, Randa Bassiouni, Maha Alquaiti, Fai Ashgan, Hans-Juergen Schulten, Mohammad H Al Qahtani
Congenital heart defects (CHDs) are the most common birth defects in neonatal life. CHDs could be presented as isolated defects or associated with developmental delay (DD) and/or other congenital malformations. A small proportion of cardiac defects are caused by chromosomal abnormalities or single gene defects; however, in a large proportion of cases no genetic diagnosis could be achieved by clinical examination and conventional genetic analysis. The development of genome wide array-Comparative Genomic Hybridization technique (array-CGH) allowed for the detection of cryptic chromosomal imbalances and pathogenic copy number variants (CNVs) not detected by conventional techniques...
March 14, 2018: Pediatric Cardiology
Richa Thakur, Ali S Haider, Ashley Thomas, Steven Vayalumkal, Umair Khan, Tijani Osumah, Kyle Doughty, Sam Finn, Kennith F Layton
A number of treatment options are available for cerebral arteriovenous malformations (AVMs) including surgical resection, stereotactic radiosurgery, and endovascular embolization. Endovascular embolization may be used pre-operatively to reduce the size of large AVMs and thus reduce surgical complications. Here we present two patients who successfully underwent preoperative embolization of their AVMs and subsequent surgery. Preoperative embolization is a viable option for AVMs to reduce complications and improve patient outcomes...
January 8, 2018: Curēus
Furkan Diren, Serra Sencer, Tayfun Hakan
Objective: Arteriovenous malformation (AVM) is the most common form of intracranial vascular malformations in adults. Intracranial pediatric AVMs are rare. AVM located in the vicinity of the brain stem in children are even more rare. Case report: This study reports a rare case of acute obstructive hydrocephalus following aqueductal stenosis caused by an unruptured grade IV perimesencephalic arteriovenous malformation. An 11-year-old boy admitted to the hospital with progressive headache, nausea and vomiting throughout a month...
2018: Open Neuroimaging Journal
Weiwei Ruan, Li Cao, Zhonghua Chen, Mingxiang Kong, Qing Bi
Hereditary multiple osteochondroma (HMO) is an autosomal dominant genetic disorder characterized by multiple outgrowing bony tumors capped by cartilage, generally affecting the metaphyses. The disease is known as hereditary multiple exostoses, familial exostosis, multiple cartilaginous exostoses or hereditary malformation of cartilage. The prevalence of HMO in Europe and the Unites States is ~1:100,000, although it has not been reported in China. The disease is often accompanied by pain, asymmetry and skeletal malformations, including forearm and leg bending deformities, limb length discrepancies, and knee internal and external rotation abnormalities...
April 2018: Oncology Letters
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