scholarly journals Staged surgical treatment of brachioplexopathy in an adolescent with Klippel-Feil syndrome: a rare clinical case and literature review

2021 ◽  
Vol 18 (1) ◽  
pp. 6-13
Author(s):  
E. V. Petrova ◽  
O. E. Agranovich ◽  
M. V. Savina ◽  
E. L. Gabbasova ◽  
V. P. Snishchuk ◽  
...  

Klippel-Feil syndrome is a congenital malformation, the leading component of which is a violation of segmentation of the cervical vertebral bodies. The syndrome can be combined with other skeletal anomalies: skull asymmetry, scoliosis, high shoulder blades, and cervical ribs. Treatment of the syndrome is usually symptomatic; indications for surgical treatment are progressive neurological disorders and persistent pain syndrome, which usually develop due to instability of unblocked segments, or neurogenic pain. A clinical case of treatment of a 17-year-old patient with Klippel-Feil syndrome who developed a picture of severe upper limb monoparesis during three years due to compression of the brachial plexus associated with cervical ribs is presented. Decompression of the brachial plexus was performed, which led to rapid relief of pain syndrome and gradual partial regression of motor disorders. Due to incomplete restoration of the gripping function, tendon-muscle plasty of the right hand was performed, which significantly improved the possibility of self-care. The results of radiation and staged neurophysiological studies are described, as well as a review of the literature on the Klippel-Feil syndrome.

2020 ◽  
Vol 22 (1) ◽  
pp. 77-82
Author(s):  
A. A. Bimurzin ◽  
A. V. Kalinovsky

The study objective is to describe a clinical case of a rare disorder of the cerebellum, Lhermitte–Duclos disease.Clinical case. The Federal Neurosurgical Center (Novosibirsk) admitted a male patient, 40 years old. Medical history showed that in 2017 he underwent ventriculoperitoneal shunt due to hypertensive hydrocephalus syndrome caused by space-occupying mass of the right hemisphere of the cerebellum. In June of 2017, the patient was consulted by a neurosurgeon. Later, an increase in the mass, partial atrophy of the optic disks were observed. Due to the patient’s complaints of headache, ataxia, vertigo, as well as insufficient effect of the shunt, microsurgical removal of the space-occupying mass in the cerebellum by right paramedian suboccipital craniotomy was performed. Gangliocytoma removal was performed under constant neurophysiological control which allowed to fully resect the gangliocytoma inside intact brain tissue. Histological and immunohistological examinations allowed to diagnose grade I dysplastic gangliocytoma of the cerebellum (Lhermitte–Duclos disease). In the postoperative period, brain symptoms regressed fully, vestibulocerebellar syndrome persisted. The patient was discharged in satisfactory condition. No negative dynamics were observed in 7 months of follow-up.Conclusion. Due to the rareness of Lhermitte–Duclos disease, currently there are no established approaches to treatment, however, in symptomatic course there are indications for surgical removal of the gangliocytoma. Radicality of resection should be correlated with the risk of neurological defects. In our case, surgical treatment allowed to improve the patient’s condition, prevent further progression of the gangliocytoma and obtain an accurate histological diagnosis.


2021 ◽  
Vol 49 ◽  
Author(s):  
V. B. Grinevich ◽  
I. V. Gubonina ◽  
M. I. Shperling ◽  
S. I. Lapteva ◽  
T. V. Kolodin ◽  
...  

Familial Mediterranean fever (FMF) is a rare genetic autosomal recessive disease typical for special ethnic groups, such as Arabs, Greeks, Armenians, Jews, Turks and other ethnicities inhabiting the Mediterranean coast. The characteristic clinical signs and symptoms of the disease are recurrent episodes of fever, associated with polyserositis, in particular, with benign (aseptic) peritonitis. The disease is caused by mutation in the MEFV gene encoding the synthesis of the protein pyrine, which leads to an uncontrolled release of pro-inflammatory cytokines by granulocytes. The criterion for the severe course of the disease is AA-renal amyloidosis associated with an unfavorable prognosis.Here we present a clinical case of classic FMF with high fever and abdominal pain syndrome. A 26-year-old Armenian patient had a sudden attack of fever and severe pain in the right iliac area several months before his first hospital admission. Non-steroid anti-inflammatory drugs and antispasmodics were unable to relieve the symptoms, with the attack ceasing spontaneously after 3 days from the beginning. Thereafter, the attacks recurred 1 to 2 times per month within a year. During hospital stay at the peak of the attack, peritoneal symptoms at abdominal palpation, together with neutrophilic leukocytosis and a significant increase in acute phase parameters of inflammation (C-reactive protein, fibrinogen, erythrocyte sedimentation rate) were found. Based on the characteristics of the complaints, past history, laboratory and instrumental results, we excluded infections, autoimmune and surgical disorders and made a decision to perform genetic testing to detect the MEFV gene mutation. Confirmation of the mutation has led to the definitive diagnosis of FMF. This clinical case is remarkable for its late onset, which is not typical for FMF. As a result, the patient was prescribed continuous therapy with сolchicine. Six months of the follow up have demonstrated a positive trend with no attacks during this period.


2020 ◽  
Vol 13 (3) ◽  
pp. 214-226
Author(s):  
Yulia Aleksandrovna Stepanova ◽  
Aleksandr Anatolevich Gritskevch ◽  
Amiran Shotaevich Revishvili ◽  
Madina Valerevna Kadirova ◽  
Egor Sergeyevich Malyshenko ◽  
...  

ntroduction. A distinctive feature of kidney cancer is a frequent, compared with other tumors, spread of the tumor through the venous collectors (in the renal and inferior vena cava up to the right atrium), along the path of least resistance to invasive growth.The aim of the study was to present a clinical case of radical treatment of kidney cancer involving extensive IVC thrombosis.Materials and methods. The study describes a clinical case of radical treatment of patient M. with kidney cancer involving extensive IVC thrombosis, extending to the right atrium (written informed consent for patient information and images to be published was obtained prior to the study). During preoperative examination, the patient was diagnosed with renal cell carcinoma with non-occlusive hypervascular tumor thrombus of the renal vein, the inferior vena cava and the right atrium based on the findings of ultrasound examination (transabdominal and transthoracic, and transesophageal), multislice computed tomography (MSCT) and magnetic resonance imaging (MRI).Results and discussion. Surgical treatment remains the main method of treatment of renal cell cancer, moreover, the inferior vena cava thrombosis cannot serve as a cause for refusing surgical treatment. The thrombus spreading along the venous collectors is an important factor in determining the tactics of surgical treatment. The length of the tumor thrombus, as well as the degree of its fixation and ingrowth into the vein wall is of great significance for planning surgical techniques and predicting clinical outcomes. Based on various methods of radiological examination, patient M. was diagnosed with cancer of the right kidney, 3 stage T3cNxM0, IVC tumor thrombus, paraneoplastic syndrome (hyperthermia), right-sided nephrectomy with aortocaval lymphadenectomy, thrombectomy from the IVC, vascular isolation of the liver, resection of the IVC, thrombectomy from the right atrium combined with cardiopulmonary bypass.Conclusion. Despite the technical complexity of nephrectomy with thrombectomy from the IVC, especially in the presence of a massive supradiaphragmatic thrombus, these interventions have no alternatives if a radical treatment is to be achieved. Step-by-step support using radiological methods of investigation is an important aspect of patients preparation; this allowing determining the exact volume of the damage and non-invasively assessing clinical outcomes of surgical treatment.


2021 ◽  
Vol 20 (2) ◽  
pp. 144-148
Author(s):  
Vasily P. Gavrilyuk ◽  
Elena V. Donskaya ◽  
Maria I. Statina ◽  
D. A. Severinov ◽  
Margarita V. Dovbnya

Background. Rib osteomyelitis is extremely rare in childhood. This localization of purulent focus represents only 1% of all osteomyelitis cases. The typical manifestations of ribs osteomyelitis are fever, chest or back pain. However, these clinical signs are not always present, and the disease can manifest in other way.Clinical Case Description. Mother with 14 years old child K. have applied to the regional children's hospital admission department. The child had complains of right upper quadrant abdominal pain and in right part of the chest, difficulty in breathing. The disease began 2 days before admission with fatigue, weakness, and pyretic fever (39 °C). Complaints remained over time. The patient was hospitalized with the diagnosis: “Chest impact injury with severe pain syndrome? Right lung contusion?". The ultrasound examination of pleural cavities and the right anterior chest surface has revealed osteomyelitic phlegmon in VII intercostal space on the 3rd day of hospitalization. It was lanced and drained under general anesthesia, about 7 ml of liquid pus were obtained. The child received treatment according to clinical guidelines for the management of patients with such nosology. The final diagnosis was: «Acute hematogenous osteomyelitis of the VII rib, local form. Osteomyelitic phlegmon of VII intercostal space. Right pneumonitis. Bilateral mild hydrothorax». The boy was discharged on the 10th day since surgery in satisfactory condition.Conclusion. The described clinical case is interesting for pediatric surgeons, traumatologists, infectious disease specialist. Particular attention should be paid to the clinical features of the disease and anamnestic information in such unclear clinical cases. It will allow to exclude traumatic injuries and assume the local inflammatory process.


2019 ◽  
Vol 16 (1) ◽  
pp. 81-87
Author(s):  
D. V. Kuklin ◽  
D. G. Naumov ◽  
M. V. Belyakov ◽  
I. A. Sovpenchuk ◽  
M. S. Serdobintsev

The paper presents a rare clinical case of surgical treatment of a patient with a giant invasive schwannoma of the thoracolumbar spine. A single-stage en block resection of the tumor through a combined posteroanterior approach was performed followed by replacement of post-resection interbody diastasis with a carbon implant and by posterior instrumental fixation of the spine. The pain syndrome regressed from VAS scores 7 and 8 (back, lower limbs) to scores 4 and 1, respectively. The follow-up examination was conducted at 6 and 12 months after surgery: there were no signs of relapse. Publications on giant invasive spinal schwannomas were analyzed.


2021 ◽  
Vol 25 (2) ◽  
pp. 80
Author(s):  
K. A. Petlin ◽  
E. A. Kosovskikh ◽  
B. N. Kozlov ◽  
V. A. Tomilin

<p>Chest injury is an acute, extremely life-threatening condition. Among penetrating heart wounds, approximately 85% are stab wounds. In the case of stab and cut injuries, the lethality, according to various sources, reaches 90% and directly depends on the time of delivery of the victim to the hospital. In the overwhelming majority of cases, emergency operations for stab wounds of the chest are conducted in general surgical hospitals. As a rule, when providing emergency surgical care, only superficial heart wounds are sutured, excluding the correction of damage to the intra-cardiac structures. Aorto-right atrial fistula is a rather rare clinical condition that is formed due to the action of a traumatic factor and is characterised by the formation of a communication between the aorta and the right atrium with the discharge of arterial blood into the venous system. Considering that this pathology is accompanied by an increase in pressure in the right atrium, signs of stagnation are found in the systemic circulation, followed by compensatory dilatation of the right atrium and then dilatation of the right ventricle. The only effective method to treat this problem is surgery.<br />The presented clinical case demonstrates the surgical treatment of chronic post-traumatic aorto-right atrial fistula, pseudo-aneurysm between the non-coronary sinus and the right atrium and pseudo-aneurysm of the left coronary sinus 2 years after the stab wound. Echocardiography performed after surgery indicated a positive trend, i.e., a decrease in the size of the right- and left-sided heart and normalisation of pressure in the pulmonary artery. No inter-chamber shunts were found. This case shows that clinicians must remember that after providing emergency surgical care to patients with penetrating chest wounds, it is necessary to conduct an additional examination of the heart after stabilisation of the condition to detect hidden damage to the intra-cardiac structures.</p><p>Received 2 February 2021. Revised 2 March 2021. Accepted 3 March 2021.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p>


2021 ◽  
Vol 74 (1) ◽  
pp. 155-160
Author(s):  
Serhii O. Savvi ◽  
Alla Yu. Korolevska ◽  
Serhii Yu. Bityak ◽  
Yevhen A. Novikov

Using the example of a clinical case, to present the management features of a patient with complete esophageal obliteration as a chemical burn result, the surgical intervention features in case of a non-standard situation during the operation, and the treatment results analyze. It was described a clinical case of 41-year-old patient with thoracic esophagus obliteration due to extended post-burn cicatricial esophageal stricture, dysphagia of IV degree in very severe general condition. Stamm-Senn-Kader’s gastrostomy was performed as a first step of surgical treatment. Angiography and embolization of the right colic artery and it’s branches was performed in 8 months while preserving the middle colic artery. In 20 days the cologastroanastomosis and feeding colostomy on the right chest wall were performed. In 10 days after the colostomy was disattached from the chest wall, the end-to-side esophagocoloanastomosis was performed intrapleurally. In one month after the third surgery and restoration of the food passage by the natural way, closure of the contact gastrostomy was performed. During the observation over the patient (8 years) the postoperative complications were not observed. The patient survived. The proposed staged surgical treatment tactics of patients with complete esophageal obliteration due to post-burn esophageal stricture, dysphagia of IV degree presents effective treatment results and a significant improvement in the patient’s life quality.


2021 ◽  
Vol 12 ◽  
pp. 555
Author(s):  
Anton Konovalov ◽  
Oleg Sharipov ◽  
Oleg Shekhtman ◽  
Vadim Gadzhiagaev ◽  
Pavel Kalinin

Background: Central nervous system (CNS) aspergillosis is more often met in patients with expressed immune suppression. Still, in 50% of cases of meningitis caused by Aspergillus spp., it is observed in patients without expressed immune suppression. The prognosis of CNS aspergillosis is unfavorable with the general rate of lethality around 70%. Case Description: Clinical case of a 58-year-old man who developed an Aspergillus abscess in the chiasmosellar region and an associated mycotic aneurysm of the right middle cerebral artery (MCA) and intracerebral hemorrhage. Microsurgical clipping of the fusiform-ectatic aneurysm of the right MCA in the conditions of rupture was performed. An extra-intracranial micro anastomosis was formed on the right. An open biopsy of the neoplasm in the chiasmosellar region was made. The neoplasm was yellow and destroyed the bone plate of the skull base. Biopsy results: Mycotic lesion (aspergillosis). The analysis of surgical treatment for mycotic aneurysms in the acute period of hemorrhage in patients with aspergillosis revealed a high rate of lethality. The issue of the feasibility and effectiveness of complicated revascularization interventions in the patients with hemorrhage and aspergillosis remains unsolved. Conclusion: The lack of generally accepted tactics of the treatment of this pathology requires further studies and systemic analysis. A high risk of the lethal outcome in patients with invasive mycotic infection and rupture of mycotic aneurysm highlight the importance of timely diagnostics and the beginning of antimycotic therapy. WThe issue of the evaluation of the revascularization methods effectiveness in patients after surgical treatment of a mycotic aneurysm associated with cerebral aspergillosis remains poor.


2021 ◽  
Vol 9 (1) ◽  
pp. 101-106
Author(s):  
S.V. Leonchenko ◽  
◽  
V.N. Petyushkin ◽  
A.P. Motin ◽  
A.A. Dyomin ◽  
...  

In the article a clinical case of surgical treatment of peptic ulcer of gastroenteroanastomosis complicated with perforation and gastrointestinal bleeding, is described. The patient was observed with the diagnosis: cholelithiasis, chronic calculous cholecystitis, for which planned laparoscopic cholecystectomy with draining of the abdominal cavity was performed. According to the discharge record, the operation ran without peculiarities. Later the patient was rehospitalized with complaints of weakness, nausea, vomiting, pain in the upper parts of the abdomen; he was diagnosed with ulcer of gastroenteroanastomosis and continuing bleeding that were indications for the surgical intervention for life-saving indications. From the patient words, a part of the stomach was resected more than 20 years before for gastric ulcer. Until the latest time, the patient felt satisfactory, but within 6 months pain in the abdomen reappeared, however, on examination cholelithiasis was identified, and the pain syndrome was attributed to this pathology. Assumably, after the first operation performed in 1995, a complication developed in the early postoperative period in the form of obstruction of gastroenteroanastomosis (anastomositis?), and additional gastroenteroanastomosis was applied. Conclusion. Peptic ulcer of anastomosis is an actual problem of the gastric surgery which may not only appear long time after the operation, but may give the same complications as «essential» peptic ulcer, and really threaten the life of patients. It should be noted that in some cases the intraoperative picture, experience and sensations of a surgeon play a decisive role in the diagnosis of surgical pathology even if they differ from the data of additional methods of examination.


2021 ◽  
Vol 15 (1) ◽  
pp. 57-62
Author(s):  
Anatoliy V. Stukalov ◽  
Roman E. Lakhin ◽  
Evgeniy N. Ershov

This report presents a clinical case of combined anesthesia in a patient with videothoracoscopic atypical resection of the right lung. In the operating room, the patient underwent ultrasound-assisted blockade in the plane of the m. erector spinae (ESP block) using 20 mL of 0.375% levobupivacaine solution, after which general anesthesia was induced. Opioid consumption per surgery was 0.3 mg of fentanyl. In the postoperative period, high-quality multimodal pain relief was achieved without the use of opioids. Within 7 h after the operation, the patient could actively expectorate with minimal discomfort. The article demonstrates the possibility of the effective application of the ESP block to reduce the risk of respiratory complications during lung surgery and to prevent the development of neuropathic post-thoracotomy pain syndrome.


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