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Skull base Child

Ajeet Kumar, Nitika Goel, Chandni Sinha, Abhishek Singh
Crouzon syndrome (CS) is an autosomal dominant genetic disorder characterized by craniofacial dysostosis. Premature fusion of skull base leads to midfacial hypoplasia, shallow orbit, mandibular prognathism, overcrowding of upper teeth, high-arched palate, and upper airway obstruction. It is important for anesthesiologists managing such patients to recognize and avoid potential airway complications. Here, we present a case of a 10-year-old child with CS posted for ptosis correction surgery. Use of peripheral nerve blocks to cut down opioid requirement, inhalational induction, and maintenance are key aspects in successful management of such cases...
January 2017: Anesthesia, Essays and Researches
Rabia Khan, Peter Oakes, Christian Fisahn, Brittni Burgess, Kristina M Kirkpatrick, Rod J Oskouian, R Shane Tubbs, Jeffrey P Blount
INTRODUCTION: Metaphyseal chondrodysplasia, Jansen type (JMD), is a rare form of endochondral ossification resulting in short limbs and dwarfism. CASE REPORT: A child presented with JMD and was found to have involvement of the cervical spine. Conservative treatment was given to the patient who at the long-term follow-up continues to have no neurological findings or cervical spine instability. CONCLUSIONS: To our knowledge, this case represents the first report of involvement of the superior cervical spine in a patient with JMD...
2017: Pediatric Neurosurgery
Peter Mudry, Ondrej Slaby, Jakub Neradil, Jana Soukalova, Kristyna Melicharkova, Ondrej Rohleder, Marta Jezova, Anna Seehofnerova, Elleni Michu, Renata Veselska, Jaroslav Sterba
BACKGROUND: Infantile myofibromatosis belongs to a family of soft tissue tumors. The majority of these tumors have benign behavior but resistant and malignant courses are known, namely in tumors with visceral involvement. The standard of care is surgical resection. Observations suggest that low dose chemotherapy is beneficial. The treatment of resistant or relapsed patients with multifocal disease remains challenging. Patients that harbor an actionable mutation in the kinase domain are potential subjects for targeted tyrosine kinase inhibitor therapy...
February 10, 2017: BMC Cancer
Maite Gárriz-Luis, Pablo Irimia, Juan M Alcalde, Pablo Domínguez, Juan Narbona
Background There are only four previous pediatric reports of the glossopharyngeal neuralgic form of the stylohyoid complex syndrome. Stylohyoid complex has merely been described as cases of glossopharyngeal neuralgia in children. Case Report A 12-year-old boy came to our hospital because of recurrent episodes of severe cranial pain (9/10) lasting for 5 to 15 minutes. Pain affected the right tonsillar fossa, ear, and mastoid region. Since the start at the age of 9 years, the frequency of painful episodes has progressively increased: when admitted to our clinics 3 years later, the child was having up to five episodes daily in spite of analgesic, antiepileptic, and antidepressant drugs; he had abandoned school and leisure...
February 2017: Neuropediatrics
K M H Ahanogbe, K Ibahioin, M Karkouri, M B Dianka, W Akpo, A El Azhari
INTRODUCTION: Osteosarcoma is a malignant mesenchymal tumor including cells that present an osteoblastic differentiation. On the skull, it has often extra-axial development associated with bone reaction. We report an atypical and rare case of intracranial or cerebral osteosarcoma underline the radiological and pathological diagnostic difficulties. CASE REPORT: Our case concerns a primary osteosarcoma without bone involvement in a 10-year old boy who was admitted for intracranial hypertension with progressive worsening and brachial monoparesis...
October 2016: Neuro-Chirurgie
Eliz Kilich, Reena Dwivedi, Shelley Segal, Sandeep Jayawant, Manish Sadarangani
We describe the youngest case to date of a 2 year old child who developed central skull base osteomyelitis (SBO) initially presenting with a fever, vomiting and sore throat. An extremely rare complication of mastoiditis following otitis media in children is SBO which can present with non-specific symptoms. This report describes the first case of symptomatic ischaemic stroke secondary to SBO in an immunocompetent child. We review the literature of the management and the potential cerebrovascular complications of central SBO in children secondary to otolaryngological infection...
October 2016: International Journal of Pediatric Otorhinolaryngology
Francesco Giovanni Sgulò, Pietro Spennato, Ferdinando Aliberti, Giuliana Di Martino, Daniele Cascone, Giuseppe Cinalli
Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined...
January 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Adi Reuveni-Salzman, Guy Rosenthal, Oded Poznanski, Yigal Shoshan, Mony Benifla
OBJECTIVE: The prevalence of skull fractures after mild head trauma is 2 % in children of all ages and 11 % in children younger than 2 years. The current standard management for a child diagnosed with an isolated skull fracture (ISF), in our institute, is hospitalization for a 24-h observation period. Based on data from the literature, less than 1 % of all minor head injuries require neurosurgical intervention. The main objective of this study was to evaluate the risk of neurological deterioration of ISF cases, in order to assess the need for hospitalization...
September 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Zhou Zhou, Binhui Jiang, Libo Cao, Feng Zhu, Haojie Mao, King H Yang
BACKGROUND AND OBJECTIVE: Studies on traumatic injuries of children indicate that impact to the head is a major cause of severe injury and high mortality. However, regulatory and ethical concerns very much limit development and validation of computer models representing the pediatric head. The purpose of this study was to develop a child head finite element model with high-biofidelity to be used for studying pediatric head injury mechanisms. METHODS: A newly developed 10-year-old (YO) pediatric finite element head model was limitedly validated for kinematic and kinetic responses against data from quasi-static compressions and drop tests obtained from an experimental study involving a child-cadaver specimen...
July 2016: Computer Methods and Programs in Biomedicine
Todd W Lyons, Anne M Stack, Michael C Monuteaux, Stephanie L Parver, Catherine R Gordon, Caroline D Gordon, Mark R Proctor, Lise E Nigrovic
BACKGROUND AND OBJECTIVE: Although children with isolated skull fractures rarely require acute interventions, most are hospitalized. Our aim was to safely decrease the hospitalization rate for children with isolated skull fractures. METHODS: We designed and executed this multifaceted quality improvement (QI) initiative between January 2008 and July 2015 to reduce hospitalization rates for children ≤21 years old with isolated skull fractures at a single tertiary care pediatric institution...
June 2016: Pediatrics
Mohamad G Bakhaidar, Fahad A Alghamdi, Saleh S Baeesa
Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma...
January 2016: Journal of Pediatric Neurosciences
Hyeoh Won Yu, Won Im Cho, Hye Rim Chung, Keun Hee Choi, Sumi Yun, Hwan Seong Cho, Choong Ho Shin, Sei Won Yang
Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD...
March 2016: Annals of Pediatric Endocrinology & Metabolism
Wei-Fang Chang, Yi-Chih Hsu, Yi-Der Wu, Chun-Lang Kuo, Guo-Shu Huang
Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors...
2016: EXCLI journal
Zhigang Li, Xiaoqiang Han, Hao Ge, Chunsheng Ma
To account for the effects of head realistic morphological feature variation on the impact dynamic responses to head injury, it is necessary to develop multiple subject-specific pediatric head finite element (FE) models based on computed tomography (CT) or magnetic resonance imaging (MRI) scans. However, traditional manual model development is very time-consuming. In this study, a new automatic method was developed to extract anatomical points from pediatric head CT scans to represent pediatric head morphological features (head size/shape, skull thickness, and suture/fontanel width)...
July 2016: Journal of the Mechanical Behavior of Biomedical Materials
M R Bogomil'sky, M M Polunin, E I Zelikovich, Yu L Soldatsky, O V Burova
This publication was designed to describe a rare case of development of apicalpetrositis in a child presenting with acute otitis mediafollowing chicken pox experienced in the preceding period. We carried out the study with the use of computed tomography (CT) that demonstrated destruction of the temporal bone, bones of the base of the skull and of the first cervical vertebra. The treatment strategy chosen for the management of this condition that included antibiotic therapy and expectant observation proved justified and can be recommended as an algorithm of choice taking into consideration the difficulty of surgical approach to the apex of the petrous pyramid...
2016: Vestnik Otorinolaringologii
Somnath Sharma, Bal Krishan Ojha, Anil Chandra, Sunil Kumar Singh, Chhitij Srivastava
An encephalocele is a protrusion of the brain and/or meninges through a defect in the skull. Based on the location of the skull defect they are classified into sincipital, basal, occipital or parietal varieties. Occurrence of more than one Encephalocele in a patient is very rare and very few cases of double encephalocele are reported. We report an interesting case where a parietal and an occipital encephalocele were present together. The patient was a 2 months boy who was brought to us with complaints of two swelling on the scalp since birth...
May 2016: European Journal of Paediatric Neurology: EJPN
M Chirat, L Dainese, S Fasola, V Couloigner, F Denoyelle, E-N Garabedian, N Leboulanger
INTRODUCTION: Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. The main head and neck locations are the base of the skull, nasopharynx, nasal cavity and orbit. An outer ear location is considered extremely rare. We present 3 cases of children, aged 6 to 14 years, presenting with auricular RMS. CASE REPORTS: The first child, aged 6, was managed by 4 chemotherapy cycles followed by surgical resection of the tumor bed, completed by 5 further cycles of chemotherapy...
February 2016: European Annals of Otorhinolaryngology, Head and Neck Diseases
Mary Dwyer
INTRODUCTION: Optimal management of paediatric patients with tumours may include radiation therapy. Proton therapy, in theory, should achieve superior outcomes. For children referred overseas, multiple factors are taken into consideration. AIM AND METHODS: The purpose of this article is to provide context to current decision making. The MEDLINE, EMBASE and PubMed databases were searched for relevant literature. RESULTS AND DISCUSSION: The delivery of proton therapy is in evolution...
February 2016: Journal of Medical Imaging and Radiation Oncology
Alistair R M Cobb, Michael Vourvachis, Jahangir Ahmed, Michelle Wyatt, David Dunaway, Richard Hayward
BACKGROUND: Patients with syndromic forms of craniosynostosis may experience functional problems such as raised intracranial pressure, proptosis, obstructive sleep apnoea and failure to thrive. The monobloc fronto-facial advancement with osteogenic distraction is increasingly used to correct these functional problems in one procedure as well as improve appearance. The authors report the phenomenon of post operative aberrant facial flushing - an unusual and previously unreported complication of the procedure...
October 2015: Journal of Cranio-maxillo-facial Surgery
Ankur Singh, Mridula Goswami, Gaurav Pradhan, Min-Su Han, Je-Yong Choi, Seema Kapoor
We report an unusual combination of features comprising delayed tooth eruption and closure of the anterior fontanel as the sole presenting features in a child with cleidocranial dysplasia (CCD). Radiological survey revealed the presence of wormian bones in the skull, pseudoepiphysis at the base of the bilateral second metacarpal, and midline ossification defects at pubic symphysis in the presence of essentially normal clavicles. DNA sequencing of the RUNX2 gene detected a novel nonsense mutation in exon1 (c...
July 2015: Molecular Syndromology
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