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      A Rare Case of Giant Occipital Encephalocele With Thoracic Myelomeningocele: An Anesthetic Conundrum

      case-report

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          Abstract

          Encephalocele and myelomeningocele are congenital defects in the cranium and spine with herniation of contents into an extracranial and extraspinal sac, respectively. The occurrence of encephalocele and myelomeningocele in the same patient has rarely been described in the literature. The anesthetic management of such cases is associated with multiple challenges, which include difficulty in securing the airway, prone positioning, blood loss, electrolyte imbalance, hypothermia, cardiorespiratory disturbances, and perioperative care. The main aims are, to prevent hemodynamic fluctuations and excessive pressure on the sac to avoid premature rupture and manage a possible difficult airway due to the head and neck mass. We report such a rare case to highlight and share our experiences faced during perioperative management of a giant vascular occipital encephalocele with impending rupture and thoracic myelomeningocele requiring surgical excision and repair. Previous similar case reports were also reviewed, and potential perioperative complications were discussed.

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          Most cited references18

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          Genetic, epigenetic, and environmental contributions to neural tube closure.

          The formation of the embryonic brain and spinal cord begins as the neural plate bends to form the neural folds, which meet and adhere to close the neural tube. The neural ectoderm and surrounding tissues also coordinate proliferation, differentiation, and patterning. This highly orchestrated process is susceptible to disruption, leading to neural tube defects (NTDs), a common birth defect. Here, we highlight genetic and epigenetic contributions to neural tube closure. We describe an online database we created as a resource for researchers, geneticists, and clinicians. Neural tube closure is sensitive to environmental influences, and we discuss disruptive causes, preventative measures, and possible mechanisms. New technologies will move beyond candidate genes in small cohort studies toward unbiased discoveries in sporadic NTD cases. This will uncover the genetic complexity of NTDs and critical gene-gene interactions. Animal models can reveal the causative nature of genetic variants, the genetic interrelationships, and the mechanisms underlying environmental influences.
            • Record: found
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            Is Open Access

            Difficult tracheal intubation in neonates and infants. NEonate and Children audiT of Anaesthesia pRactice IN Europe (NECTARINE): a prospective European multicentre observational study

            Neonates and infants are susceptible to hypoxaemia in the perioperative period. The aim of this study was to analyse interventions related to anaesthesia tracheal intubations in this European cohort and identify their clinical consequences.
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              Associated malformations among infants with neural tube defects.

              Infants with neural tube defects (NTDs) often have associated congenital anomalies. The reported frequency and types of associated malformations vary between different studies. The purpose of this investigation was to assess the frequency and types of associated malformations among infants with NTDs in a geographically well-defined population from 1979 to 2008 of 402,532 consecutive births. Of the 441 infants with NTDs born during this period, 20.4% had associated malformations. Infants with associated malformations were divided into those with recognizable conditions [11 (2.5%) infants with chromosomal and 23 (5.2%) with non-chromosomal conditions], and those without recognizable conditions [56 (12.7%) infants with multiple malformations]. Associated malformations were more frequent among infants with encephalocele (36.8%) than those with anencephaly (11.5%) or spina bifida (23.8%). Oral clefts and malformations in the musculoskeletal, renal and cardiovascular systems were the most commonly observed associated anomalies. The frequency of associated malformations in infants with NTDs emphasizes the need for a thorough investigation of these infants. Routine screening for other malformations, especially facial clefts and musculoskeletal, renal and cardiac anomalies, may need to be considered in infants with NTDs, and referral of these infants for genetics evaluation and counseling seems warranted.

                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                26 September 2022
                September 2022
                : 14
                : 9
                : e29602
                Affiliations
                [1 ] Anesthesiology, Critical Care and Pain Medicine, All India Institute of Medical Sciences, Raipur, Raipur, IND
                Author notes
                Jitendra V. Kalbande drjitu222@ 123456gmail.com
                Article
                10.7759/cureus.29602
                9599913
                36321061
                5014f921-7b1d-4e5a-93af-679158876c70
                Copyright © 2022, Kalbande et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 23 September 2022
                Categories
                Anesthesiology
                Pediatrics
                Neurosurgery

                paediatric neurosurgery,paediatric anesthesia,encephalocele with myelomeningocele,neonates,multiple neural tube defect,myelomeningocele,encephalocele,difficult airway

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