scholarly journals Poland’s syndrome

2017 ◽  
Vol 5 (1) ◽  
pp. 63-70
Author(s):  
Olga E. Agranovich ◽  
Igor A. Komolkin ◽  
Alyona Ju. Dimitrieva
Keyword(s):  
Surgical Treatment ◽  
Clinical Features ◽  
Clinical Presentation ◽  
Treatment Options ◽  
The Body ◽  
Poland’S Syndrome ◽  
Poland's Syndrome ◽  
Congenital Condition ◽  
The Right ◽  
Bone Abnormalities

Poland’s syndrome is a rare congenital condition classically characterized by partial or complete absence of chest muscles on one side of the body and usually webbing of the fingers of the hand on the same side. There may also be rib (aplasia or hypoplasia) and chest bone abnormalities, which may be noticeable due to less fat under the skin. Breast and nipple abnormalities may also occur, and underarm hair is sometimes sparse or abnormally placed. In most cases, the abnormalities in the chest area do not cause health problems or affect movement. Poland’s syndrome most often affects the right side of the body and occurs more frequently in males than in females. The etiology is unknown; however, interruption of the embryonic blood supply to the arteries that lie under the collarbone (subclavian arteries) is the prevailing theory. There are many methods of operative correction because of the polymorphic clinical features of this syndrome. We gathered data on the etiology, pathogenesis, and clinical presentation of Poland’s syndrome and reviewed the existing surgical treatment options.

scholarly journals Siblings with Bardet Beidl Syndrome

2013 ◽  
Vol 33 (3) ◽  
pp. 236-238
Author(s):  
Ram Peter ◽  
Priya Jose ◽  
MNG Nair
Keyword(s):  
Clinical Features ◽  
Autosomal Recessive ◽  
Clinical Presentation ◽  
The Body ◽  
Bardet Biedl Syndrome ◽  
Recessive Condition ◽  
Autosomal Recessive Condition

Bardet Biedl syndrome is an autosomal recessive condition affecting many parts of the body. Incidence of BBS is 1 in 100000. Its clinical features varies in person to person though from same family too. We are reporting two siblings with Bardet Beidl syndrome with different clinical presentation. DOI: http://dx.doi.org/10.3126/jnps.v33i3.8081   J. Nepal Paediatr. Soc. 2013;33(3):236-238

scholarly journals Congenital lobar emphysema: a rare cause of respiratory distress in a neonate

2018 ◽  
Vol 6 (1) ◽  
pp. 218
Author(s):  
Nishant Mittal ◽  
Ankit Parakh ◽  
Prashant Jain ◽  
N. K. Mittal
Keyword(s):  
Early Intervention ◽  
Respiratory Distress ◽  
Treatment Options ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Congenital Lobar Emphysema ◽  
Congenital Condition ◽  
Lobar Emphysema ◽  
Intervention Message ◽  
The Right

Congenital lobar emphysema (CLE) is a congenital condition characterized by distension and air trapping of the affected lobe of the lung. It is one of the causes of infantile respiratory distress, which may require surgical resection of affected lobe. Case characteristics: 3-day-old neonate with ventilation refractory respiratory distress. Imaging was suggestive of decreased lung tissue on the right side with ipsilateral mediastinal shift. Intervention/ outcome: Early surgical lobectomy was done to improve lung functions and the child improved dur to early intervention. Message: An early diagnosis with high index of suspicion helps patients with this rare congenital anomaly. Early intervention is the key to good long-term outcome. More awareness about the entity and treatment options available would greatly help improving the outcome and disease burden.

scholarly journals Spinal lipomas: clinical spectrum, embryology, and treatment

2007 ◽  
Vol 23 (2) ◽  
pp. 1-12 ◽  
Author(s):  
Michael A. Finn ◽  
Marion L. Walker
Keyword(s):  
Surgical Treatment ◽  
Clinical Presentation ◽  
Wide Spectrum ◽  
Spinal Dysraphism ◽  
Treatment Options ◽  
Conus Medullaris ◽  
Clinical Spectrum ◽  
Pathological Features ◽  
Occult Spinal Dysraphism ◽  
Pathological Mechanism

✓Spinal lipomas, particularly lipomas of the conus medullaris and terminal filum, are the most common form of occult spinal dysraphism and represent a wide spectrum of disease with regard to anatomy, clinical presentation, and treatment options. These lesions, however, are united by a similar embryology and pathological mechanism by which symptoms arise. Recently, the treatment of these lesions has generated much controversy, with some physicians advocating surgical treatment for all patients regardless of symptoms and others proposing that surgery be withheld until symptoms develop. The authors discuss lumbosacral spinal lipomas, with particular attention to the theories of their origin, anatomical and pathological features, and treatment options, including a review of current controversies.

A case of successful surgical treatment of patient with adrenal cyst using 3-d modeling

2020 ◽  
Vol 28 (4) ◽  
pp. 530-535
Author(s):  
Ivan Andreev ◽  
Alexander Kolsanov ◽  
Sergey Katorkin ◽  
Evgeniy Shestakov ◽  
Leonid Lichman
Keyword(s):  
Surgical Treatment ◽  
Surgical Resection ◽  
3D Modeling ◽  
Clinical Observation ◽  
Preoperative Planning ◽  
Clinical Presentation ◽  
Surgical Intervention ◽  
Adrenal Cyst ◽  
The Right ◽  
Medical University

Aim. Demonstration of potentials of preoperative planning and implementation of surgical resection in patients with adrenal cysts. A clinical observation of a successful surgical treatment of a rare pathology cyst of the right adrenal is presented. The choice of surgical treatment tactics is determined by the size of tumor and clinical presentation of the disease. The surgical treatment was accomplished laparoscopically which permitted to reduce the time of recovery and rehabilitation of the patient. In this clinical observation, the benefit of using 3D-modeling of the surgical area was shown for visualization of topographic and anatomic peculiarities and facilitation of the intraoperative navigation with the help of Avtoplan program developed by Samara State Medical University. Conclusion. Preoperative 3D-modeling permits to prepare to surgical intervention taking into account individual anatomic peculiarities of a patient, and to determine the optimal volume of the operation.

scholarly journals Non-Melanoma Skin Cancers: Biological and Clinical Features

2020 ◽  
Vol 21 (15) ◽  
pp. 5394
Author(s):  
Mauro Cives ◽  
Francesco Mannavola ◽  
Lucia Lospalluti ◽  
Maria Chiara Sergi ◽  
Gerardo Cazzato ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Clinical Features ◽  
Clinical Presentation ◽  
Treatment Options ◽  
Biological Evolution ◽  
Hedgehog Pathway ◽  
Merkel Cell ◽  
Innovative Treatment ◽  
Skin Cancers ◽  
Melanoma Skin

Non-melanoma skin cancers (NMSCs) include basal cell carcinoma (BCC), squamous cell carcinoma (SCC) and Merkel cell carcinoma (MCC). These neoplasms are highly diverse in their clinical presentation, as well as in their biological evolution. While the deregulation of the Hedgehog pathway is commonly observed in BCC, SCC and MCC are characterized by a strikingly elevated mutational and neoantigen burden. As result of our improved understanding of the biology of non-melanoma skin cancers, innovative treatment options including inhibitors of the Hedgehog pathway and immunotherapeutic agents have been recently investigated against these malignancies, leading to their approval by regulatory authorities. Herein, we review the most relevant biological and clinical features of NMSC, focusing on innovative treatment approaches.

scholarly journals Features of diagnostics and surgical treatment of right ventricular thrombosis complicated by pulmonary artery thromboembolism (clinical case)

2019 ◽  
pp. 67-71
Author(s):  
R. M. Vitovsky ◽  
P. M. Semeniv ◽  
A. O. Rusnak ◽  
Y. R. Ivanov ◽  
V. F. Onischenko
Keyword(s):  
Pulmonary Embolism ◽  
Surgical Treatment ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Young Patients ◽  
The Body ◽  
Blood Clots ◽  
The Right ◽  
Clinical Conditions

The case of differential diagnosis and treatment of a patient with pulmonary embolism (PE), the source of which was the thrombus formed in the right ventricle of the heart, is presented. The peculiarity of this case was the untimely diagnosis of the disease, which simulated pneumonia, the treatment of which did not improve the clinical condition of the patient. Tomography allowed to determine the thrombosis of the right branch of the pulmonary artery and to send the patient to the cardiac surgery center for further treatment. Diagnosis of a probable source of embolism occurred after echocardiography, which revealed a tumor-like lesion of the right ventricle of large size and dense consistency. The results of surgical treatment of the patient, during which extensive formation of the right ventricle was removed, a dense elastic consistency with signs of fragmentation confirmed the prediction of this particular source of pulmonary embolism. Removal of blood clots from the right branch of the pulmonary embolism showed their similar macrostructure with right ventricular formation. The appearance and macrostructure of the formation did not allow to determine with certainty its character. Only histological examination was able to determine the thrombogenicity of the origin of this formation. The recurrent nature of pneumonia, without the presence of risk factors, in young patients may be the basis for more thorough examination to identify atypical clinical conditions. The restoration of the source of the body is of great importancefor the prevention of its relapse. Finding the source of pulmonary embolism should necessarily include echocardiography to carefully examine possible lesions of intracardiac structures with the formation of blood clots that may be responsible for its occurrence.

scholarly journals PECULIARITIES OF CLINICAL PRESENTATION AND SURGICAL TREATMENT OF PATIENTS WITH OCCULT CARCINOMA OF GALLBLADDER

2018 ◽  
pp. 87-91
Author(s):  
V. I. Кolomiytsev ◽  
О. M. Syroid ◽  
О. V. Lukavetskiy
Keyword(s):  
Surgical Treatment ◽  
Liver Resection ◽  
Bile Ducts ◽  
Clinical Presentation ◽  
Distant Metastases ◽  
The Body ◽  
Treatment Result ◽  
Extrahepatic Bile Ducts ◽  
Calculous Cholecystitis ◽  
Carcinoma Of Gallbladder

141 patients with gallbladder cancer (GBC) were operated from 2003 to 2016. 29 (20.6 %) patients were diagnosed with occult GBC. In most (88.4–100 %) cases, GBC was accompanied by cholelithiasis; furthermore, cholelithiasis lasted much longer if patients had occult GBC (P = 0.032). The signs of acute or chronic calculous cholecystitis were clinically detected in patients with occult GBC. For this group of patients, tumours were more often located in the body or fundus of gallbladder (P < 0.05) and were less locally spread (P < 0.04); however, the level of differentiation (P > 0.3) and the presence of distant metastases (P > 0.4) were not statistically different from those in patients with manifest GBC. Cholecystectomy was performed in all patients with occult GBC and in 50 (44.6 %) patients with manifest GBC (P < 0.001). Liver resection and/or operation on the extrahepatic bile ducts was performed in 8 (27.6 %) and 50 (44.6 %) patients, respectively (P < 0.001). Postoperative complications were detected in 2 (6.9 %) patients with occult GBC and in 27 (24.1 %) patients with manifest GBC (P < 0.05). ІІІ–ІV stage of tumour was identified in 5 (17.2 %) patients with occult GBC. Treatment result in this case was less satisfactory.

scholarly journals PECULIARITIES OF CLINICAL PRESENTATION AND SURGICAL TREATMENT OF PATIENTS WITH OCCULT CARCINOMA OF GALLBLADDER

2018 ◽  
pp. 87-91
Author(s):  
V. I. Кolomiytsev ◽  
О. M. Syroid ◽  
О. V. Lukavetskiy
Keyword(s):  
Surgical Treatment ◽  
Liver Resection ◽  
Bile Ducts ◽  
Clinical Presentation ◽  
Distant Metastases ◽  
The Body ◽  
Treatment Result ◽  
Extrahepatic Bile Ducts ◽  
Calculous Cholecystitis ◽  
Carcinoma Of Gallbladder

141 patients with gallbladder cancer (GBC) were operated from 2003 to 2016. 29 (20.6 %) patients were diagnosed with occult GBC. In most (88.4–100 %) cases, GBC was accompanied by cholelithiasis; furthermore, cholelithiasis lasted much longer if patients had occult GBC (P = 0.032). The signs of acute or chronic calculous cholecystitis were clinically detected in patients with occult GBC. For this group of patients, tumours were more often located in the body or fundus of gallbladder (P < 0.05) and were less locally spread (P < 0.04); however, the level of differentiation (P > 0.3) and the presence of distant metastases (P > 0.4) were not statistically different from those in patients with manifest GBC. Cholecystectomy was performed in all patients with occult GBC and in 50 (44.6 %) patients with manifest GBC (P < 0.001). Liver resection and/or operation on the extrahepatic bile ducts was performed in 8 (27.6 %) and 50 (44.6 %) patients, respectively (P < 0.001). Postoperative complications were detected in 2 (6.9 %) patients with occult GBC and in 27 (24.1 %) patients with manifest GBC (P < 0.05). ІІІ–ІV stage of tumour was identified in 5 (17.2 %) patients with occult GBC. Treatment result in this case was less satisfactory.

scholarly journals Crossed renal ectopia with an unusual form of fusion (inverted U shape) managed by novel approach - laparoscopic ureterocalicostomy. A case report

2021 ◽  
Vol 6 (1) ◽  
pp. 71-75
Author(s):  
Osama Bani Hani ◽  
Omar Halalsheh ◽  
Yazeed Mohammad ◽  
Anas Bani Yaseen ◽  
Ruba Khasawneh ◽  
...  
Keyword(s):  
Case Report ◽  
Clinical Presentation ◽  
The Body ◽  
Published Data ◽  
Renal Ectopia ◽  
Novel Approach ◽  
Crossed Renal Ectopia ◽  
The Right ◽  
Tract Infections ◽  
Unusual Type

Herein we present a case of crossed renal ectopia with an unusual type of fusion, discovered incidentally in a 11-year-old girl presented with recurrent urinary tract infections. Both kidneys were located on the right side of the body fused in their upper poles only, forming an inverted U shape. After reviewing the published data on this topic, we found that most of the described anomalies were within the six well-known types of fusion anomalies. This child had an unusual clinical presentation of severe hydronephrosis of the orthotopic kidney. A unique surgical technique to correct the pathology to be able to preserve the residual mass of that kidney was performed.

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