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https://www.readbyqxmd.com/read/28733728/totally-extraperitoneal-approach-for-ventral-hernia
#1
Sante Capitano
BACKGROUND: In open surgery, extraperitoneal sublay mesh implantation is generally preferred to intraperitoneal placement, following the same principles as in "giant prosthetic reinforcement of the visceral sac" described for inguinal hernia repair [1, 2]. Miserez and Penninckx in 2002 described an endoscopic totally preperitoneal ventral hernia repair in a small cohort of 15 cases [3]. Unfortunately, this technique has not spread, probably because of the technical difficulties that require, but not for effectiveness...
July 21, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28730275/comparative-analysis-of-open-and-robotic-transversus-abdominis-release-for-ventral-hernia-repair
#2
James G Bittner, Sameer Alrefai, Michelle Vy, Micah Mabe, Paul A R Del Prado, Natasha L Clingempeel
BACKGROUND: Transversus abdominis release (TAR) is a safe, effective strategy to repair complex ventral incisional hernia (VIH); however, open TAR (o-TAR) often necessitates prolonged hospitalization. Robot-assisted TAR (r-TAR) may benefit short-term outcomes and shorten convalescence. This study compares 90-day outcomes of o-TAR and r-TAR for VIH repair. METHODS: A single-center, retrospective review of patients who underwent o-TAR or r-TAR for VIH from 2015 to 2016 was conducted...
July 20, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28726148/first-human-use-of-hybrid-synthetic-biologic-mesh-in-ventral-hernia-repair-a-multicenter-trial
#3
James G Bittner, Kevin El-Hayek, Andrew T Strong, Melissa Phillips LaPinska, Jin S Yoo, Eric M Pauli, Matthew Kroh
BACKGROUND: Mesh options for reinforcement of ventral/incisional hernia (VIH) repair include synthetic or biologic materials. While each material has known advantages and disadvantages, little is understood about outcomes when these materials are used in combination. This multicenter study reports on the first human use of a novel synthetic/biologic hybrid mesh (Zenapro(®) Hybrid Hernia Repair Device) for VIH repair. METHODS: This prospective, multicenter post-market clinical trial enrolled consecutive adults who underwent elective VIH repair with hybrid mesh placed in the intraperitoneal or retromuscular/preperitoneal position...
July 19, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28714291/mechanism-of-midline-defect-causing-mutation-p151l-in-mid1-revealed
#4
Linda K Nicholson
The P151L mutation in the B-box1 domain of MID1 causes midline defects in X-linked Opitz G Syndrome. MID1 is known to be a key regulator of phosphatase PP2A through formation of a complex with its catalytic (PP2Ac) and regulatory (α4) subunits. Wright et al. show that this mutation retains B-box1 domain structure and E3 ligase activity (star) but blocks interaction with α4, indicating disruption of the MID1-α4-PP2Ac complex.
July 2017: FEBS Journal
https://www.readbyqxmd.com/read/28705101/abdominal-wall-expanding-system-obviates-the-need-for-lateral-release-in-giant-incisional-hernia-and-laparostoma
#5
Dietmar Eucker, Andreas Zerz, Daniel C Steinemann
BACKGROUND: In large incisional hernias and after laparostoma midline closure may be impossible. A novel abdominal wall expander system (AWEX) is proposed and evaluated. METHODS: In patients with large incisional hernias and laparostoma where primary midline closure was impossible, AWEX was used. Patients undergoing abdominal wall reconstruction using AWEX between May 2012 and December 2015 were included. Intraoperative the abdominal wall was stretched by attaching the midline fascia borders to a retraction system under tension for 30 minutes...
July 1, 2017: Surgical Innovation
https://www.readbyqxmd.com/read/28699050/-optic-nerve-hypoplasia-and-septo-optic-dysplasia
#6
R Lohmüller, A-S Gangloff, F Wenzel, W A Lagrèze
Optic nerve hypoplasia (ONH) is one of the most common causes of congenital visual impairment. It was first described in 1915 and represents a developmental disorder of the central nervous system. It is often associated with intracranial midline defects and is then referred to as septo-optic dysplasia (SOD). The symptoms of ONH range from minimal visual dysfunction to significant visual impairment with sensory defect nystagmus and even blindness. The ONH is often associated with further systemic, endocrinological and neurological abnormalities requiring a close interdisciplinary treatment of the patients...
July 11, 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/28691197/loss-of-dcc-in-the-spinal-cord-is-sufficient-to-cause-a-deficit-in-lateralized-motor-control-and-the-switch-to-a-hopping-gait
#7
Jimmy Peng, Julien Ferent, Qingyu Li, Mingwei Liu, Ronan Vinicius da Silva, Hanns Ulrich Zeilhofer, Artur Kania, Ying Zhang, Frédéric Charron
BACKGROUND: Humans with heterozygous mutations in the axon guidance receptor DCC display congenital mirror movements (MMs), which are involuntary movements of body parts, such as fingers, on one side of the body that mirror voluntary movement of the opposite side. In mice, the homozygous Dcc(kanga) mutant allele causes synchronous MM-like hindlimb movements during locomotion, resulting in hopping. In both human and mice, the neuroanatomical defect responsible for the deficit in lateralized motor control remains to be elucidated...
July 10, 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/28685259/extremely-large-sinus-pericranii-with-involvement-of-the-torcular-and-associated-with-crouzon-s-syndrome
#8
Felipe H Sanders, Bryan A Edwards, Matthew Fusco, Rod J Oskouian, R Shane Tubbs, James M Johnston
INTRODUCTION: Sinus pericranii is a rare vascular malformation that connects the intracranial dural sinuses to the extracranial venous drainage system and is caused by either trauma or congenital defects. Although the majority of these vascular structures are due to trauma, some are congenital. CASE REPORT: Herein, we report a 5-month-old patient with a very large and fluctuating subcutaneous mass over the occiput and the diagnosis of Crouzon's syndrome. The child presented with a large midline mass that on imaging, connected to the underlying torcular and was diagnosed as a sinus pericranii...
July 6, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28676569/vegf-a-and-neuropilin-1-nrp1-shape-axon-projections-in-the-developing-cns-via-dual-roles-in-neurons-and-blood-vessels
#9
Lynda Erskine, Urielle François, Laura Denti, Andy Joyce, Miguel Tillo, Freyja Bruce, Neil Vargesson, Christiana Ruhrberg
Visual information is relayed from the eye to the brain via retinal ganglion cell (RGC) axons. Mice lacking NRP1 or NRP1-binding VEGF-A isoforms have defective RGC axon organisation alongside brain vascular defects. It is not known whether axonal defects are caused exclusively by defective VEGF-A signalling in RGCs or are exacerbated by abnormal vascular morphology. Targeted NRP1 ablation in RGCs with a Brn3b(Cre) knock-in allele reduced axonal midline crossing at the optic chiasm and optic tract fasciculation...
July 1, 2017: Development
https://www.readbyqxmd.com/read/28665391/-tactics-of-reconstructive-closure-of-basal-defects-after-resection-of-midline-tumors-of-the-anterior-skull-base-a-literature-review
#10
D A Gol'bin, S N Mindlin
The purpose of this review is to analyze the techniques used to reconstruct midline defects in the anterior skull base after transcranial and endoscopic endonasal resection of skull base tumors. We provide brief information on the technique for preparing various flaps, indications and contraindications, and advantages and disadvantages. We analyze the international experience in the use of vascularized and non-vascularized flaps as well as the existing algorithms for reconstruction of complex defects of the midline structures of the skull base...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28652674/evolution-of-ventricular-outpouching-through-the-fetal-and-postnatal-periods-unabating-dilemma-of-serial-observation-or-surgical-correction
#11
REVIEW
Niraj Kumar Dipak, Sumitra Venkatesh, Shakuntala Prabhu, Sudha Rao
Ventricular outpouching is a rare finding in prenatal sonography and the main differential diagnoses are diverticulum, aneurysm, and pseudoaneurysm in addition to congenital cysts and clefts. The various modes of fetal presentation of congenital ventricular outpouching include an abnormal four-chamber view on fetal two-dimensional echocardiogram, fetal arrhythmia, fetal hydrops, and pericardial effusion. Left ventricular aneurysm (LVA)/nonapical diverticula are usually isolated defects. Apical diverticula are always associated with midline thoracoabdominal defects (epigastric pulsating diverticulum or large omphalocele) and other structural malformations of the heart...
July 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28634690/long-term-retromuscular-and-intraperitoneal-mesh-size-changes-within-a-randomized-controlled-trial-on-incisional-hernia-repair-including-a-review-of-the-literature
#12
P Rogmark, O Ekberg, A Montgomery
Purpose Ingrowth of fibroblasts in a polypropylene mesh may cause contraction and a later recurrence. We assessed mesh contraction in intraabdominal and retromuscular implantation after incisional hernia repair. METHODS: A cohort of patients within an RCT on laparoscopic (LHR) versus open hernia repair (OHR) had their mesh borders marked with metal clips. X-ray was performed on postoperative day 1 and after 1 year. Total length, width, and dislocation were measured. A tacker fixated large-pore polypropylene mesh was used in LHR, and a retromuscular small-pore heavy-weight mesh was sutured to the midline in OHR...
June 20, 2017: Hernia: the Journal of Hernias and Abdominal Wall Surgery
https://www.readbyqxmd.com/read/28617177/discordant-anomalies-with-combined-features-of-pentalogy-of-cantrell-and-oeis-complex-a-case-report-in-monochorionic-twins
#13
Nique Kunapinun, Jitsupa Treetipsatit
INTRODUCTION: Ventral body wall defects have various manifestations. Among others, pentalogy of Cantrell (PC) and omphalocele exstrophy imperforate anus spinal abnormalities (OEIS) complex are defects that involve upper and lower anterior midline of body wall, respectively. Although both entities are in a spectrum of ventral body wall defects, the combination of PC and OEIS complex has not been described. CASE REPORT: In this report, we describe an unusual case of congenital ventral body wall defect with combined features of PC and OEIS complex, which discordantly occurred in monochorionic monoamniotic twins...
June 15, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28615204/human-cytomegalovirus-ie2-protein-disturbs-brain-development-by-dysregulating-neural-stem-cell-maintenance-and-the-polarization-of-migrating-neurons
#14
Dasol Han, Sung-Hyun Byun, Juwan Kim, Mookwang Kwon, Samuel J Pleasure, Jin-Hyun Ahn, Keejung Yoon
Despite the high incidence of severe defects in the central nervous system caused by human cytomegalovirus (HCMV) congenital infection, the mechanism of HCMV neuropathogenesis and the roles of individual viral genes have not yet been fully determined. In this study, we show that the immediate-early 2 (IE2) protein may play a key role in HCMV-caused neurodevelopmental disorders. IE2-transduced neural progenitor cells gave rise to neurospheres with a lower frequency and produced smaller neurospheres than control cells in vitro, indicating reduction of self-renewal and expansion of neural progenitors by IE2...
June 14, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28610820/mandibular-symphyseal-midline-distraction-osteogenesis-for-micrognathia-associated-with-aglossia-and-situs-inversus-totalis
#15
X C Ren, Y F Li, Y Liu, S S Zhu
Aglossia is a rare congenital abnormality, often associated with micrognathia and limb defects. Situs inversus totalis is also a rare congenital abnormality, defined as a mirror-image reversal of all the asymmetric organs of the thorax and abdomen. The concurrence of these two abnormalities has only been reported in eight similar cases in the literature. Although micrognathia and malocclusion were observed in all of these cases, few treatments were performed for the patients' dentofacial deformities. This report describes the case of a 7-year-old boy suffering from micrognathia, aglossia, and situs inversus totalis simultaneously, and the treatment for his micrognathia by mandibular symphyseal midline distraction osteogenesis, guided by virtual surgical planning and a three-dimensional printed surgical template...
June 10, 2017: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28599093/clinical-and-molecular-cytogenetic-characterization-of-four-unrelated-patients-carrying-2p14-microdeletions
#16
Marie-Laure Mathieu, Caroline Demily, Sandra Chantot-Bastaraud, Alexandra Afenjar, Cyril Mignot, Joris Andrieux, Marion Gerard, Jaume Catala-Mora, Pierre Simon Jouk, Audrey Labalme, Patrick Edery, Damien Sanlaville, Massimiliano Rossi
We report the clinical and molecular cytogenetic characterization of four unrelated patients from France and Spain, carrying 2p14 microdeletions and presenting with intellectual disability and dysmorphisms. 2p14 microdeletions are very rare. Seven patients have been reported so far harboring deletions including 2p14p15 and encompassing OTX1, whose haploinsufficiency is frequently associated with genitourinary defects. To date, only one patient has been reported carrying a more proximal 2p14 microdeletion which does not include OTX1...
June 9, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28593555/genetic-analysis-of-irf6-a-gene-involved-in-craniofacial-midline-formation-in-relation-to-pituitary-and-facial-morphology-of-patients-with-idiopathic-growth-hormone-deficiency
#17
Eline Starink, Anita C S Hokken-Koelega, Theo J Visser, Janneke Baan, Robin P Peeters, Laura C G de Graaff
INTRODUCTION: Growth hormone is secreted by the pituitary gland, which forms part of the craniofacial midline. IRF6 encodes a transcription factor involved in the development of the craniofacial midline and mutations in IRF6 are known to disturb craniofacial development. Craniofacial and pituitary development are closely related. After whole exome sequencing revealed a new mutation in IRF6 in a family with Idiopathic Growth Hormone Deficiency (IGHD), we screened the remainder of our IGHD cohort for mutations in this gene and related their genotypes to pituitary and craniofacial morphology...
June 7, 2017: Pituitary
https://www.readbyqxmd.com/read/28583501/otorhinolaryngologic-manifestations-of-hartsfield-syndrome-case-series-and-review-of-literature
#18
Jeremie D Oliver, Deanna C Menapace, Shelagh A Cofer
Diagnosis of Hartsfield syndrome includes recognition of three distinct clinical anomalies: holoprosencephaly, ectrodactyly, and bilateral cleft-lip and palate syndrome. A family including three male siblings all affected by Hartsfield syndrome presented to our institution for care. An autosomal dominant variant in Fibroblast Growth Factor Receptor 1 (FGFR1) was identified. This report focuses on otorhinolaryngologic manifestationsof Hartsfield syndrome, previously undescribed, including midline defects of holoprosencephaly, bilateral cleft-lip and palate, retrognathia, gastroesophageal reflux disease, external ear anomalies, eustachian tube dysfunction, and midface abnormalities, in addition to multidisciplinary, long-term management strategies...
July 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28548391/structural-and-functional-observations-of-the-p151l-mid1-mutation-reveal-alpha4-plays-a-significant-role-in-x-linked-opitz-syndrome
#19
Katharine M Wright, Haijuan Du, Michael A Massiah
Mutations of human MID1 are associated with X-linked Opitz G Syndrome (XLOS), which is characterized by midline birth defects. XLOS-observed mutations within the MID1 B-box1 domain are associated with cleft lip/palate, wide-spaced eyes and hyperspadias. Three of the four XLOS-observed mutations result in B-box1 unfolding but the structural and functional effects of the P151L mutation is not characterized. Here, we demonstrate that the P151L mutation does not disrupt the overall tertiary structure of the B-box1 domain and the adjacent domains...
May 26, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28523383/anterior-sacral-meningocele-presenting-as-intracystic-bleeding
#20
Ignacio Javier Gilete-Tejero, Marta Ortega-Martínez, Jacinto Mata-Gómez, María Rico-Cotelo, Luis Miguel Bernal-García, Beatriz Yerga-Lorenzana, Ignacio Casado-Naranjo
PURPOSE: To report a case of anterior sacral meningocele with intralesional bleeding secondary to sacrococcygeal trauma. Likewise, there is a discussion about the physiopathology and the surgical approach to these types of lesions. METHODS: A 43-year-old man diagnosed with Marfan syndrome suffered sacrococcygeal trauma. He was admitted to the emergency room due to symptoms of headache, nausea, and lower limb subjective weakness. CT and MRI showed a large retroperitoneal mass with hemorrhagic content close to the sacrum...
May 18, 2017: European Spine Journal
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