Congenital Tumors
Asim Kurjak in CRC Handbook of Ultrasound in Obstetrics and Gynecology, 2019
Other pathological structures that should be differentiated from cystic hygroma are cervical meningomyelocele, encephalocele, and cystic teratoma of the neck. It is particularly important to distinguish encephalomeningocele and cystic hygroma on the basis of morphological criteria alone. These criteria have been described by Pearce et al.33 and serve as a useful guide for differential diagnosis. Cystic hygroma is usually characterized by the presence of typical multiple septa, contains only fluid, and is located in the posterolateral aspect of the neck. They may sometimes extend over the trunk, back, or extremities. In more than half of our cases, the cystic hygromas were larger than the fetal head. On the contrary, encephalocele is characterized by the presence of the large cyst posteriorly to the head. Although bony defect of the skull may not be seen in some cases, helpful signs which enable correct diagnosis are herniation of the brain tissue in the sac, smaller size of biparietal diameter (BPD), and presence of slight ventriculomegaly.
Fetal malformations detected with magnetic resonance imaging in the diabetic mother
Moshe Hod, Lois G. Jovanovic, Gian Carlo Di Renzo, Alberto de Leiva, Oded Langer in Textbook of Diabetes and Pregnancy, 2018
If defect of the neural tube closure occurs on the cranial end, it will result in meningocele or meningoencephalocele, as shown in Figure 42.15. Most of the time, the defect is on the occipital part of the skull. In meningoencephalocele or encephalocele, there is some brain tissue herniated in the protruded meningeal sac. Encephalocele is associated with a much worse neurological prognosis. Encephalocele can readily be detected on the ultrasound. MRI complements the ultrasound by its ability to detect the herniated brain with higher accuracy. This is particularly important in smaller or deeper lesions, such as lesion in the base of the skull. Associated anomalies of the posterior fossa can be evaluated with higher clarity, as shown in Figure 42.16.
CSF Leaks
John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie in Basic Sciences Endocrine Surgery Rhinology, 2018
Congenital CSF leaks in association with encephalocele or meningoencephaloceles are uncommon. These are well diagnosed on MRI and this scan is recommended when sinus opacity is demonstrated adjacent to a skull base defect. Brain tissue contained within the encephalocele is invariably non-functioning and can be removed as part of the surgical procedure. Congenital abnormalities of the inner ear such as the Mondini Dysplasia may present with substantial CSF leaks where the CSF has only briefly transversed the perilymphatic space. Such leaks, in addition to presenting as hearing loss or recurrent meningitis, may also present with CSF otorrhea or CSF oto-rhinorrhea (Figure 108.1).
Bone fusion in transcele reconstruction of frontoethmoidal meningoencephalocele
Published in British Journal of Neurosurgery, 2022
Samsul Ashari, Mohammad Saekhu, Kevin Gunawan, Amanda Aldilla, Nadya Zaragita, Setyo Widi Nugroho
Frontoethmoidal encephaloceles is a rare congenital malformation in Western countries but is not uncommon in Southeast Asia region. Various theories have been proposed regarding the pathogenesis of this malformation, one of them by De Klerk and De Villiers, who suggested that adhesions between the neuro- and cutaneous-ectoderm may prevent the normal ingrowth of mesoderm to form a normal skull.9 In 2007, Selçuki et al. did a research and found that in the anterior encephalocele there was a disruption in the closure of rostral neuropore into becoming glabella that caused the failure of superficial ectoderm separation with neuroectoderm, so that mesoderm in the area of closure of rostral neuropore was not formed, and bone defects occurred in the foramen cecum. From this study, it was concluded that apoptosis is needed for a perfect neurulation process, especially when the neural folds separate from the cutaneous ectoderm. Anterior encephalocele is not caused by bone development disorders.10
Association of Fetal MTHFR C677T Polymorphism with Susceptibility to Neural Tube Defects: A Systematic Review and Update Meta-Analysis
Published in Fetal and Pediatric Pathology, 2022
Razieh Sadat Tabatabaei, Neda Fatahi-Meibodi, Bahare Meibodi, Atiyeh Javaheri, Hajar Abbasi, Amaneh Hadadan, Reza Bahrami, Seyed Reza Mirjalili, Mojgan Karimi-Zarchi, Hossein Neamatzadeh
Neural tube defects (NTDs) are serious birth defects of the central nervous system (CNS) that originate during embryogenesis when the neural tube fails to close completely [1,2]. According to the reports, as morbidity and mortality from infectious diseases are decreasing globally, the contribution of birth defects to under-5 morbidity and mortality will continue to increase equally [3]. In low income countries, NTDs may account for 29% of neonatal deaths due to observable birth defects [4]. In 2016, Zaganjor et al., in a systematic review demonstrated a great variability in reported NTD prevalence estimates globally (range: 0.3–199.4 per 10,000 births) [3]. It is estimated that 1 in 1000 infants are born with a NTD annually in American Caucasians, and more are spontaneously aborted or electively terminated [5]. Open spina bifida (myelomeningocele) is the most common type of NTD and results from a failure of the closure of the neural tube along the body axis during fetal development [6,7]. Encephalocele is a type of NTD which is characterized by a meningeal sac which contains brain tissue, protruding from the skull [8,9].
Acute cerebral venous outflow obstruction during convexity meningioma resection
Published in International Journal of Neuroscience, 2021
Bo Shen, Changchen Hu, Liming Jia, Yanjun Hu
Cerebral venous outflow obstruction often refers to idiopathic intracranial hypertension (IIH), and the most common clinical condition related to IIH is dural venous sinus obstruction (stenosis). In these cases, the pathological process is often chronic with mild symptoms and rarely requires urgent surgical intervention [1]. However, acute cases of cerebral venous outflow obstruction during surgery develop an intractable intraoperative encephalocele. In clinical practice, the “intraoperative encephalocele (or intraoperative brain bulging)” is considered to be cerebral herniation. According to the classic definition, encephaloceles consist of brain tissue and meninges that have herniated through a skull defect, and although the pathology of an encephalocele involves abnormally displaced normal brain tissue, it is somewhat different from cerebral herniation. Cerebral herniation specifically refers to the displacement of brain tissue in a closed cranium, such as a subfalcine herniation, transtentorial herniation, or cerebellar tonsil herniation. However, an encephalocele is the displacement of brain tissue to the extracranial space through a skull defect [2].
Related Knowledge Centers
- Ataxia
- Microcephaly
- Neural Tube Defect
- Skull
- Spastic Quadriplegia
- Teratology
- Brain
- Biological Membrane
- Craniofacial Abnormality
- Hydrocephalus