Malignant extrarenal rhabdoid tumor: a case report

2015 ◽  
Vol 4 (1) ◽  
Author(s):  
Tanja Stüber ◽  
Eric Frieauff ◽  
Joachim Diessner ◽  
Arnd Hönig ◽  
Monika Rehn
Keyword(s):  
Fetal Distress ◽  
Rhabdoid Tumor ◽  
First Year ◽  
Therapeutic Trial ◽  
Male Fetus ◽  
Preterm Baby ◽  
Histopathologic Analysis ◽  
The Right ◽  
First Year Of Life ◽  
Extrarenal Rhabdoid Tumor

AbstractThe fetal extrarenal rhabdoid tumor is a highly aggressive and rare neoplasm that mostly occurs in the first year of life. In this case, a remarkably enlarged right upper arm was diagnosed in a male fetus at 31+2 weeks of gestation. Because of a fast progressive tumor volume and signs of fetal distress, a cesarean section was performed at 34 weeks of gestation. The preterm baby showed a blue livid tumor reaching from the right elbow up to the shoulder that resulted in an extrarenal rhabdoid tumor on histopathologic analysis. A therapeutic trial with cyclophosphamide was not effective and the child died at the age of 26 days because of multi-organ failure.

AGE-RELATED CHANGES IN THICKNESS OF CORTEX AND ITS LAYERS IN THE PARAMEDIAN LOBULE OF THE CEREBELLUM IN CHILDREN

2020 ◽  
pp. 14-18
Author(s):  
Татьяна Александровна Цехмистренко ◽  
Аслан Батразович Мазлоев ◽  
Дмитрий Константинович Обухов
Keyword(s):  
Right Hemisphere ◽  
Molecular Layer ◽  
First Year ◽  
Age Related ◽  
Average Group ◽  
Computer Morphometry ◽  
The Right ◽  
First Year Of Life ◽  
Granular Layers ◽  
Age Related Changes

Цель - изучение возрастных изменений толщины коры и ее слоев в парамедианной дольке мозжечка у детей. Материал и методы. Работа выполнена на постмортальном материале (62 мозжечка), полученном от детей в возрасте от рождения до 12 лет, умерших в результате травм без повреждений головного мозга. С помощью компьютерной морфометрии на окрашенных методом Ниссля фронтальных гистологических срезах коры, взятой билатерально в области парамедианной (тонкой) дольки (HVIIB) на вершине листков мозжечка, измеряли толщину коры, а также толщину ее молекулярного и зернистого слоев. Анализ количественных данных проводили в годовых интервалах. Результаты. В парамедианной дольке мозжечка увеличение толщины коры происходит в четыре этапа: в правом полушарии - от рождения к 1, 3, 5 и 9 годам, в левом полушарии - к 1, 5, 7 и 9 годам. Левосторонняя асимметрия толщины коры мозжечка отмечается у детей 1 и 2 лет, толщины молекулярного слоя - у детей 3 лет жизни. Правосторонняя асимметрия характерна для толщины зернистого слоя у детей 3 лет и поперечника коры, в целом, у детей 6 лет. Толщина коры и слоев в области парамедианной дольки мозжечка по среднегрупповым показателям достигает уровня взрослых людей к 9 годам. Выводы. Толщина коры мозжечка и ее слоев в области дольки H VII B увеличивается гетерохронно и гетеродинамически в правом и левом полушариях мозжечка у детей на первом году жизни, а также в периоды раннего, первого и второго детства. Уменьшения поперечника коры и слоев в парамедианной дольке мозжечка у детей от рождения до 12 лет не обнаружено. Objective - to study the age-related changes in the thickness of the cortex and its layers in the paramedian lobule of the cerebellum in children. Material and methods. The work was performed on postmortem material (62 cerebellums) obtained from children aged from birth to 12 years who died from injuries but without brain damage. The thickness of the cortex, as well as the thickness of its molecular and granular layers, were measured using computer morphometry on the Nissl-stained frontal histological sections of the cortex taken bilaterally in the region of the paramedian (gracile) lobule (HVIIB) at the top of the folia of cerebellum. Analysis of quantitative data was performed at annual intervals. Results. In the paramedian lobule of the cerebellum, the increase in the thickness of the cortex occured in four stages: in the right hemisphere - from birth to 1, 3, 5 and 9 years, in the left hemisphere - to 1, 5, 7 and 9 years. Left-sided asymmetry of the cortical thickness of the cerebellum was observed in 1 and 2-year old children, the thickness of the molecular layer - in 3-year old children. Right-sided asymmetry was characteristic for the thickness of the granular layer in 3-year old children and a cross-section of the cortex in 6-year old children. The thickness of the cortex and layers in the area of the paramedian lobule of the cerebellum on the average group indicators reached the level of adults by 9 years. Conclusions. The thickness of the cerebellar cortex and its layers in the area of the lobule HVIIB increased heterochronically and heterogeneously in the right and left hemispheres of the cerebellum in children of the first year of life, and in the periods of early, first and second childhood. No reduction in the diameter of the cortex and layers in the paramedian lobule of the cerebellum of children from birth to 12 years was found.

scholarly journals Stridor in infant caused by subglottic hemangiome: case report

2020 ◽  
Vol 10 (3) ◽  
Author(s):  
Raquel Lot ◽  
Caroline Rosa ◽  
Camila Freitas ◽  
Gracinda Adnet ◽  
Luisa Costa ◽  
...  
Keyword(s):  
Beta Agonist ◽  
First Year ◽  
Vascular Neoplasms ◽  
Threatening Condition ◽  
Life Threatening ◽  
Diagnostic Hypothesis ◽  
Therapeutic Measures ◽  
The Right ◽  
First Year Of Life ◽  
Age Range

Subglottic hemagioma is a rare cause of stridor, but it is one of the most common vascular neoplasms of the airways in childhood. If the treatment is not promptly instituted, it becomes a life-threatening condition. The diagnosis should be suspected when infants outside the age range for acute laryngitis present with stridor associated with severe respiratory effort, without viral prodromes, with a condition that is not responsive to initial therapeutic measures considering the main diagnostic hypothesis. Infantile hemangiomas begin to proliferate during the first year of life (between the 1st and 2nd month of life). Involution usually occurs between 6 months and 12 months of life (most involution until 4 years). The case is a 5-month-old female infant, with sudden stridor associated with respiratory distress without viral prodromes or fever, with little response to inhaled short-acting beta-agonist, inhaled adrenaline, as well as corticosteroids inhalation/parenteral. Bronchoscopy showed a bulging of the submucosa to the right of the subglottis with slight vascularization, suggestive of subglottic hemangioma. Treatment with propranolol was initiated orally with the aim of regressing the hemangioma and after clinical stability, the infant was discharged with outpatient follow-up.

scholarly journals Transposition of Great Arteries

2021 ◽  
Author(s):  
Rita Prasad Verma
Keyword(s):  
Fetal Echocardiography ◽  
Arterial Switch Operation ◽  
Cardiac Anomaly ◽  
First Year ◽  
Term Outcome ◽  
Hemodynamic Pattern ◽  
Switch Operation ◽  
The Right ◽  
First Year Of Life

TGA is the commonest complex congenital cyanotic cardiac anomaly occurring during the first week of life. It is characterized by the unusual anomaly of ventriculoarterial discordance, with the aorta (A) originating from the right ventricle (RV) and the pulmonary artery (PA) from the left ventricle (LV). In the common Dextro form (DTGA), A is abnormally located to the right, anterior, and inferior of PA. The anatomic configuration results in the lethal hemodynamic pattern of 2 independent and parallel running circulatory circuits, which mandates creating a conduit to ensure the mixing of oxygenated and deoxygenated blood for survival. In the rare Levo form (LTGA), the aorta is placed anterior and to the left of PA with ventricular inversion. TGA is well tolerated in the fetus and is challenging to diagnose by fetal echocardiography unless the outflow tracts are specifically visualized. Postnatally the typical findings of murmur and cyanosis vary according to the associated cardiac defects and the degree of intercirculatory mixing. The arterial switch operation (ASO), which involves establishing ventriculoarterial concordance, is the standard surgical repair of D-TGA and has replaced the atrial switch procedures due to its superior long-term outcomes. The Rastelli procedure is used for complex DTGA cases. DTGA has a 90% mortality rate in the first year of life if untreated, while over 95% survive for 5 to 25 years after surgery. Post-surgical course may be complicated and require surgical revisions. The long-term outcome is associated with normal or mild to moderate neurodevelopmental disabilities, depending upon the type, complexity, and course of the disease. Expert follow-up of the patients into adulthood is an integral part of the management of TGA for best outcomes.

The Hypoplasic Mandible: What Makes it Different From the Healthy Child?

2020 ◽  
pp. 105566562097230
Author(s):  
Floriane Remy ◽  
Yves Godio-Raboutet ◽  
Guillaume Captier ◽  
Pierre Bonnaure ◽  
Philippe Burgart ◽  
...  
Keyword(s):  
Supervised Classification ◽  
Three Dimensional ◽  
Growth Period ◽  
P Value ◽  
First Year ◽  
Healthy Children ◽  
Mandibular Hypoplasia ◽  
The Difference ◽  
The Right ◽  
First Year Of Life

Objective: This study aimed to analyze the morphology of the hypoplasic mandible and its evolution during the growth period to better understand how it differs from the pediatric healthy mandible. Method: Three-dimensional mandibular models of hypoplasic and healthy children aged from 39 gestational weeks to 7 years old were analyzed with a morphometric method including data clustering. Morphological distinctions between pathological and healthy mandibles were highlighted. Bilateral and unilateral mandibular hypoplasia were distinguished. Results: The study sample was composed of 31 hypoplasic children and as many sex- and age-matched healthy children. Morphological distinctions between pathological and healthy mandibles were highlighted only from the first year of life. In bilateral hypoplasia, the overall mandibular dimensions were reduced while there was only a ramus asymmetry in unilateral mandibular hypoplasia (mean ± SD of the difference between the Grp03c and Grp03b subgroups: 6.80 ± 6.37 – P value = 1.64e–3 for the height of the left ramus versus 0.18 ± 4.18 – P value = .82 for the height of the right ramus). Supervised classification trees were built to identify the pathology and discriminate unilateral from bilateral mandibular hypoplasia (prediction rates = 81% and 84%, respectively). Conclusions: Based on a morphometric analysis, we demonstrated that mandibular hypoplasia significantly impacts the mandibular morphology only from the first year of life, with a distinction between bilateral and unilateral hypoplasia.

scholarly journals A RARE CASE OF HETEROTAXIA: FETAL AUTOPSY FINDINGS

2020 ◽  
Vol 28 (1) ◽  
Author(s):  
Daisy Dwivedi ◽  
Bal Chander
Keyword(s):  
Rare Case ◽  
Mirror Image ◽  
First Year ◽  
Male Fetus ◽  
Fetal Autopsy ◽  
Autopsy Findings ◽  
Situs Solitus ◽  
The Right ◽  
Thoracic And Abdominal ◽  
Asymmetrically Distributed

Visceral organs are asymmetrically distributed in thoracic and abdominal cavities but there is definite left right orientation along the midline. At times there may be disturbance of the same resulting from malrotation and/or movement during embryological development. We are presenting autopsy findings of a 20 weeks old male fetus wherein the stomach along with spleen and pancreas are on the right side. Rest of the thoracoabdominal viscera are in the anatomically correct locations. Such cases qualify for heterotaxia, denoting status somewhere between situs solitus which is the normal placement of organs and situs inversus which denotes mirror image of solitus. Heterotaxia can have a range of features. Such cases usually come to light within first year of birth but some cases remain asymptomatic till adulthood and are diagnosed incidentally. This article discusses the nomenclature with diagnostic criteria and points out the lacunae which make the present case difficult to classify.

scholarly journals GENERAL CHARACTERISTICS OF THE MAIN MORPHOMETRIC INDICATORS OF THE SHAPE AND ANGIOARCHITECTURE OF THE HUMAN GREATER OMENTUM

2019 ◽  
Vol 19 (4) ◽  
pp. 148-153
Author(s):  
I.L. Fedorchenko
Keyword(s):  
Blood Supply ◽  
Anterior Abdominal Wall ◽  
Individual Variability ◽  
Mesenteric Arteries ◽  
Greater Omentum ◽  
First Year ◽  
Linear Dimensions ◽  
The Right ◽  
First Year Of Life ◽  
Age Range

The review examines the age characteristics of the greater omentum, its metric and topographic parameters. It has been revealed that the shape of the greater omentum can be single-, two- and multi-lobular, which is determined by its loose part. The linear dimensions of the greater omentum are consistent with the stature type and age range. The length of the loose portion of the greater omentum varies widely and ranges from 20 to 43 cm (average 31.58 ± 0.8 cm); the area of the surface of the greater omentum in adults reaches an average of 0.81 m2, which is almost half the entire surface of the peritoneum and is on average 0.40 m2. The range of individual variability in the size of the greater omentum varies at different ages of the child's development. It is especially large at the end of the first year of life. In the next 2-4 years of life, the range of variability of the greater omentum narrows slightly, the largest growth of linear sizes of the greater omentum is observed during the first year of life of the child and they increase by 2 times, and the area – by 3 times. The asymmetry of the longitudinal dimensions of the greater omentum on the right and left also increases. The boundaries of the greater omentum are: liver, spleen, parietal peritoneum of anterior abdominal wall, small intestine, ascending and descending part of the colon. It has been revealed that the main source of blood supply of the human greater omentum is the abdominal and upper mesenteric arteries, the anterior duplication of the greater omentum receives nutrition from the short and long omental arteries, and numerous small arterial vessels are involved in the blood supply of the upper left part of the omentum; they are located in the thickness of the gastro-splenic ligament. Usually 5-13 branches ramify from the right gastrointestinal artery, and one branches off from the left one. The left gastrointestinal artery has a length of 7 to 21 cm and the right gastrointestinal artery – from 10 to 38 cm.

Prenatal detection of congenital malignant extrarenal rhabdoid tumor primarily involving the right upper extremity that ruptured in utero

2009 ◽  
Vol 29 (8) ◽  
pp. 819-821 ◽  
Author(s):  
Ja-Young Kwon ◽  
Kook In Park ◽  
Ki Seok Lee ◽  
Woo Ick Yang ◽  
Young-Han Kim
Keyword(s):  
Upper Extremity ◽  
In Utero ◽  
Rhabdoid Tumor ◽  
Prenatal Detection ◽  
The Right ◽  
Extrarenal Rhabdoid Tumor

Atypical teratoid rhabdoid tumor in the first year of life: the Canadian ATRT registry experience and review of the literature

2017 ◽  
Vol 132 (1) ◽  
pp. 155-162 ◽  
Author(s):  
Mary Fossey ◽  
Haocheng Li ◽  
Samina Afzal ◽  
Anne-Sophie Carret ◽  
David D. Eisenstat ◽  
...  
Keyword(s):  
Rhabdoid Tumor ◽  
First Year ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Review Of The Literature ◽  
Atypical Teratoid ◽  
First Year Of Life

scholarly journals The Importance of Physical Therapy in the Treatment of Unilateral Congenital Bell Paralysis - A Case Report

2018 ◽  
Vol 0 (0) ◽  
Author(s):  
Jelena Milosevic ◽  
Danijela Pavicevic ◽  
Katarina Parezanovic-Ilic ◽  
Zoran Milenkovic
Keyword(s):  
Facial Nerve ◽  
First Year ◽  
Nerve Lesion ◽  
Efficient Technology ◽  
The Face ◽  
Facial Nerve Lesion ◽  
The Right ◽  
First Year Of Life ◽  
First Time ◽  
Degree Of Recovery

Abstract Peripheral paralysis of facial nerve in the newly-born can be congenital and developed. In clinical sense, paralysis of facial nerve is characterised by paralysis of mimic face muscles that are controlled by a facial nerve. A female newly-born, delivered by Caesarean section was clinically diagnosed weakness on the right side of the face. Thirteen days after the birth the newly-born was examined by a physiatrist for the first time due to the weakness of the right facial side. During the first year of life a severe congenital lesion of facial nerve was diagnosed. Rehabilitation treatments were administered during the first year of life, with partial clinical improvement. The seriousness of facial nerve lesion has a significant influence on the degree of recovery. It is very important to identify the type of lesion by using efficient technology, since it is the only way to provide early and adequate therapy.

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