3 Consent to Treatment
Judith Hendrick in Child Care Law for Health Professionals, 2018
In this case the baby was born prematurely suffering from a serious form of hydrocephalus. At 16 weeks she was terminally ill and had massive physical and mental handicaps with blindness, probable deafness and spastic cerebral palsy of all four limbs. Her ultimate prognosis was one of hopelessness as she was dying and the only question was how soon this would happen. The central issue before the court was what treatment should be given if, as sooner or later was inevitable, C suffered some infection or illness over and above the handicaps from which she was already suffering. For example, if it became impossible to feed her through a syringe should she be fed through a naso-gastric tube or intravenously? And should antibiotics be given if she developed an infection?
Familial Chordoma
Dongyou Liu in Handbook of Tumor Syndromes, 2020
There are approximately 300 new cases of chordoma in the United States each year [23]. In pediatric patients, there have been less than 300 cases reported in the literature [24]. The diagnosis of this tumor is made based on clinical, topographical, radiological, and histological findings [24]. Young children with an intracranial chordoma will often experience headaches as a clinical symptom before diagnosis. Infants and very young children, those below 5, diagnosed with this tumor often too will present with long tract signs, lower cranial nerve palsy, and torticollis. Sacrococcygeal chordoma is another form of chordoma in which patients will present clinically with perineal pain as well as potential bladder or bowel problems [24]. Lastly, vertebral column chordoma is often characterized by pain and deformity in the spine. More general symptoms like hydrocephalus occur in approximately one-third of patients [25]. Adult patients similarly present with symptoms related to location of their primary lesion [26].
Pre- and Perinatal Factors in the Etiology of Mental Retardation
Michele Kiely in Reproductive and Perinatal Epidemiology, 2019
The problem of outcome for perinatal factors is not simply definition and measurement of mental retardation. The identical processes which may impair the intellect, may also cause a cerebral palsy syndrome, and/or epilepsy. All pose problems of definition, classification, identification, and standardized measurement, and may be associated with apparently similar clinical states or processes. Infantile hydrocephalus illustrates many of the problems. It is a clinical, or at best a pathological entity which may arise in association with spina bifida as a neural tube defect, as a result of peri- and intraventricular hemorrhage before, during or after delivery, especially in immature babies, or after birth as a consequence of encephalitis. It may be of any degree, and may lead to cerebral damage, intellectual impairment, and/or multiple disabilities of any degree or none. It may be arrested naturally or by medical intervention in certain cases. What is cause and what is outcome is difficult to determine in this complex, variable, and multifactorial process.161
Prenatal and Postnatal Zika Intrauterine Infection: Diagnostic Imaging Techniques and Placental Pathology
Published in Fetal and Pediatric Pathology, 2023
Pedro Teixeira Castro, Heron Werner, Edward Araujo Júnior, Maria Paola Bonasoni, Gabriele Tonni
A 33-year-old primigravida woman, at 7th week of gestation (WGA), presented with fever and cutaneous rash. No other signs or clinical manifestations were present until 20th WGA. At this week, during routine second trimester ultrasound examination, head circumference (HC) was below the 5th centile for the expected value of gestational age [5]. This finding was associated with a moderate borderline bilateral ventriculomegaly defined as a lateral ventricular measurement of 13 mm [6–8]. Following genetic counseling, fetal karyotype and investigations for Toxoplasma gondii, rubella virus, cytomegalovirus, and herpes virus (TORCH) were carried out on the amniotic fluid. Karyotype demonstrated a 46,XY fetus, with no signs of congenital TORCH infection. At 37th WGA, the patient was referred to the Clínica Diagnóstico por Imagem, Rio de Janeiro-RJ, Brazil for thorough ultrasound examination and fetal MRI with a special focus on central nervous system (CNS). Ultrasound examination confirmed severe microcephaly (HC < 3rd centile for expected gestational age) [9] associated with mild hydrocephaly (lateral ventricles > 16 mm) [6]. The periventricular areas were hyperechogenic and calcifications were observed within the cerebral parenchyma (Fig. 1a). The posterior fossa was normal (Fig. 1b). The fetal MRI confirmed the ultrasound diagnosis of mild, bilateral hydrocephalus, with smoothness of the brain surface. The skin was folded on the occipital region, with significant microcelphaly. Posterior fossa was normal as well as the other organs (Figs. 2a, 2b, 2c, 2d).
Neuropathology Evaluation of in Utero Correction of Myelomeningocele and Complications of Late-Onset GBS Infection
Published in Fetal and Pediatric Pathology, 2023
Sarah Edminster, Tai-Wei Wu, Alexander Van Speybroeck, Jason Chu, Denise A. Lapa, Ramen H. Chmait, Linda J. Szymanski
Myelomeningocele (MMC) causes significant morbidity and mortality. It has been hypothesized that MMC is a progressive disease that begins with the initial failure of the neural tube to close. Further neurologic deficits ensue secondary to damage to the exposed spinal cord from irritants and toxins in amniotic fluid as well as direct trauma to the cord (the “two-hit” hypothesis) [1]. Recent efforts have been directed to correcting this defect in utero to decrease the need for treatment of progressive hydrocephalus and improve orthopedic outcomes. To date, various surgical approaches have been utilized to repair MMC defects – from hysterotomy to fetoscopy [2]. Different types of patches have been used, dermal matrix or biocellulose, as dural substitutes. The neuropathology literature on antenatally repaired MMC and associated complications remains limited. In this report, we describe the neuropathologic findings of a dermal matrix used as a dural substitute in a case where the MMC was repaired in utero. The case was complicated by late-onset group B streptococcus (GBS) infection and neonatal demise.
Symptomatic communicating hydrocephalus in a contemporary cohort of high grade glioma patients
Published in British Journal of Neurosurgery, 2018
Thomas Beez, Sven Burgula, Marcel Kamp, Marion Rapp, Hans-Jakob Steiger, Michael Sabel
After obtaining approval by the institutional review board (study no. 4587), we retrospectively identified all patients who underwent neurosurgical resection of a supratentorial HGG (i.e. gliomas of WHO grades III and IV) under the care of the senior author between 2007 and 2013. Patients had to have radiological (cranial MRI or CT) and clinical follow-up data available for ≥6 months following the last craniotomy for tumour resection. Patients presenting with obstructive hydrocephalus due to tumour growth, space-occupying tumour bed cysts, (potential) post-infectious hydrocephalus (i.e. hydrocephalus occurring after microbiologically proven infectious meningitis) or radiological evidence of leptomeningeal tumour spread were not included due to distinct underlying pathomechanisms. Demographic (age, gender), tumour (WHO grade, location) and treatment (number of craniotomies, intraoperative opening of ventricles, implantation of BCNU wafers, radiotherapy, chemotherapy) variables were extracted from hospital notes. In hydrocephalic patients, treatment variables prior to the diagnosis of hydrocephalus were deemed relevant to investigate risk factors.
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