scholarly journals Scoliosis in Goldenhar syndrome with curve reversal during brace treatment: a case report

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Masashi Uehara ◽  
Shugo Kuraishi ◽  
Shota Ikegami ◽  
Hiroki Oba ◽  
Takashi Takizawa ◽  
...  

Abstract Background Goldenhar syndrome sometimes displays progressive scoliosis and other spinal deformities that require treatment. However, few reports exist on scoliosis correction in Goldenhar syndrome. We described the rare radiological outcomes of a patient with Goldenhar syndrome who received brace treatment for scoliosis. Case presentation A 4-year-old boy was diagnosed as having Goldenhar syndrome and referred to our hospital for scoliosis treatment. The deformity deteriorated gradually, and left convex scoliotic angle was 26 degrees (T3-L2) at 11 years of age. Unexpectedly during treatment with an orthopedic brace, the curve had reversed to 21 degrees (T5-L2) at 7 months of therapy. After another adjustment of the brace, his right convex scoliotic angle improved to 13 degrees (T4-L2) at 15 months of treatment. Conclusions Curve reversal may occur during brace treatment for scoliosis in Goldenhar syndrome. Clinicians may opt to periodically check curve correction despite the risk of increased radiation exposure.

2017 ◽  
Vol 11 (1) ◽  
pp. 1548-1557 ◽  
Author(s):  
Shu-Yan Ng ◽  
Xiao-feng Nan ◽  
Sang-Gil Lee ◽  
Nico Tournavitis

Introduction: Physiotherapeutic Scoliosis-Specific Exercises (PSSE) and bracing have been found to be effective in the stabilization of curves in patients with Adolescent Idiopathic Scoliosis (AIS). Yet, the difference among the many PSSEs and braces has not been studied. The present review attempts to investigate the role of curve correction in the outcome of treatment for PSSEs and braces. Material and Methods: A PubMed manual search has been conducted for studies on the role of correction in the effectiveness of PSSE and bracing. For the PSSEs, the key words used were “adolescent idiopathic scoliosis, correction, physiotherapy, physical therapy, exercise, and rehabilitation.” For bracing, the key words used were “adolescent idiopathic scoliosis, correction and brace”. Only papers that were published from 2001-2017 were included and reviewed, as there were very few relevant papers dating earlier than 2001. Results: The search found no studies on the role of correction on the effectiveness of different PSSEs. The effectiveness of different PSSEs might or might not be related to the magnitude of curve correction during the exercises. However, many studies showed a relationship between the magnitude of in-brace correction and the outcome of the brace treatment. Discussion: The role of correction on the effectiveness of PSSE has not been studied. In-brace correction, however, has been found to be associated with the outcome of brace treatment. An in-brace correction of < 10% was associated with an increased rate of failure of brace treatment, whereas an in-brace correction of >40-50% was associated with an increased rate of brace treatment success (i.e. stabilization or improvement of curves). Thus, in the treatment of AIS, patients should be advised to use highly corrective braces, in conjunction with PSSE since exercises have been found to help stabilize the curves during weaning of the brace. Presently, no specific PSSE can be recommended. Conclusion: Braces of high in-brace correction should be used in conjunction with PSSEs in the treatment of AIS. No specific PSSE can be recommended as comparison studies of the effectiveness of different PSSEs are not found at the time of this study.


2020 ◽  
pp. 1-5
Author(s):  
Anton Stift ◽  
Kerstin Wimmer ◽  
Felix Harpain ◽  
Katharina Wöran ◽  
Thomas Mang ◽  
...  

Introduction: Congenital as well as acquired diseases may be responsible for the development of a megacolon. In adult patients, Clostridium difficile associated infection as well as late-onset of Morbus Hirschsprung disease are known to cause a megacolon. In addition, malignant as well as benign colorectal strictures may lead to intestinal dilatation. In case of an idiopathic megacolon, the underlying cause remains unclear. Case Presentation: We describe the case of a 44-year-old male patient suffering from a long history of chronic constipation. He presented himself with an obscurely dilated large intestine with bowel loops up to 17 centimeters in diameter. Radiological as well as endoscopic examination gave evidence of a spastic process in the sigmoid colon. The patient was treated with a subtotal colectomy and the intraoperative findings revealed a stenotic stricture in the sigmoid colon. Since the histological examination did not find a conclusive reason for the functional stenosis, an immunohistochemical staining was advised. This showed a decrease in interstitial cells of Cajal (ICC) in the stenotic part of the sigmoid colon. Discussion: This case report describes a patient with an idiopathic megacolon, where the underlying cause remained unclear until an immunohistochemical staining of the stenotic colon showed a substantial decrease of ICCs. Various pathologies leading to a megacolon are reviewed and discussed.


Author(s):  
Josué Saúl Almaraz Lira ◽  
Alfredo Luis Chávez Haro ◽  
Cristian Alfredo López López ◽  
Remedios del Pilar González Jiménez

Introduction. Scorpion stings occur mainly in spring and summer, with an estimate of 1.2 million cases per year worldwide. About 300,000 poisonings occur within a year, primarily affecting children and adults older than 65 years. In 2019, Guanajuato (Mexico) ranked third in poisoning by scorpion sting with a total of 43,913 cases. The intoxication grades are three where the signs and symptoms are varied. There are two types of antivenom in the Mexican market, and we use Alacramyn® in our case. Case presentation. A 70-year-old female —with grade 1 scorpion sting poisoning, 30 minutes of evolution, with type 2 diabetes and high blood pressure— received two vials of antivenom according to current regulations. She presented transient vagal reaction and subsequent transient pain in the cervical region that radiates to the sacral region. At discharge, there are no data compatible with scorpion sting poisoning. Conclusions. Transient pain in the cervical region to the sacral region may be secondary to an anxiety crisis, hypersensitivity to IgG, or secondary reaction to administration in less time than recommended by the provider. The benefit was greater than the reactions that occurred.


2020 ◽  
Vol 30 (3) ◽  
pp. 56-59
Author(s):  
Jūratė Gudaitytė ◽  
Justina Jermolajevaitė ◽  
Martynas Judickas

Background and objectives: Acromegaly is endocri­nal disorder which results in changes involving ge­neral appearance as well as upper airway abnorma­lities, cardiovascular and metabolic disorders which can aggravate the anesthesia and can lead to compli­cations. We aim to discuss the challenges for anesthe­siologist that occurs facing patient with acromegaly and are necessary to investigate before performing any kind of intervention. Case Presentation: 79 years old male patient presen­ted the hospital with recently diagnosed acromegaly for rectal prolapse surgery. From anamnesis he had NYHAIII with cardiomyopathy, atrial fibrillation and arterial hypertension, also multiple old compressive fractures Th10 – L5. He was graded with Mallam­pati score IV and ASA class IV. The complemen­tary examinations were made to assess the possible complications. In induction of general anesthesia the intubation was performed using fibro- bronchoscope and anesthesia went without complications except hypotension which was managed. After surgery the patient was leaded to the postoperative room for furt­her monitoring. Discussion and Conclusion: Acromegalic patients have an increased risk of difficulty during anesthe­sia compared to general population due to difficult intubation, cardiovascular complications , OSA , alte­ration in intraoperative glucose intolerance and fluid regulation. Therefore profound investigation and as­sessment are necessary to predict and prepare for possible difficulties in the surgery room.


2020 ◽  
Vol 3 (1) ◽  
Author(s):  
Anne-Jet S. Jansen ◽  
Paul M. van Schaik ◽  
Jasper M. Martens ◽  
Michel M. P. J. Reijnen

Abstract Background This case report demonstrates the value of IMPEDE-FX plugs in an embolization procedure of a false lumen of an infrarenal post-dissection aneurysm. Case presentation A 69-year-old patient was treated with mitral valve replacement, complicated by a Stanford type-A dissection. After 9 years he presented with an enlarging infrarenal post-dissection aneurysm. The false lumen was embolized using multiple IMPEDE-FX plugs as part of the treatment in addition to embolization of the inferior mesenteric artery and overstenting of the re-entry in the right iliac artery. At 15 months the CTA showed a fully thrombosed false lumen and remodeling of the true lumen. Conclusions The false lumen of an infrarenal post-dissection aneurysm can successfully be embolized using IMPEDE-FX embolization plugs as part of the treatment strategy. Prospective trials on patients with non-thrombosed false lumina are indicated.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yi Zhang ◽  
Cheng Chen ◽  
Min Lin ◽  
Kan Deng ◽  
Huijuan Zhu ◽  
...  

Abstract Background Functional gonadotroph adenomas (FGAs) are rare adenomas that most commonly secrete FSH. However, solitary LH-secreting pituitary adenomas are unusual. Case presentation A 30-year-old woman with elevated LH and normal FSH presented with inability to conceive. An MRI revealed an enlarged sella turcica and an intrasellar mass. Treatment with transsphenoidal resection led to normalization of LH and estradiol, as well as successful pregnancy. And we reviewed 6 cases of LH-secreting pituitary adenomas from 1981 to 2020. Conclusions Our case is unique because of the LH-secreting pituitary adenoma without FSH hypersecretion. This case indicates that pituitary adenoma should be considered when other diseases causing infertility have been excluded.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Min Li ◽  
Gang Zhu ◽  
Hao Guo ◽  
Shun Nan Ge ◽  
Guo Dong Gao ◽  
...  

AbstractBackgroundCerebral fat embolism (CFE) syndrome at high altitude was rare complicated with paroxysmal sympathetic hyperactivity (PSH) syndrome and septic shock. It is a challenge to differential diagnosis and treatment at high altitude.Case presentationThis case presents a CFE with PSH and septic shock of a 23-year-old man occurred at high altitude of 3800 m above sea level, transferred by airplane successfully and cured in the department of neurosurgery, Xi’an Tangdu Hospital.ConclusionsIt is key that CFE with PSH can be rapid diagnosed and treatment bundles of septic shock should be initiated as soon as possible. Early neurological rehabilitation played an important role for good outcome.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Henry Robayo-Amortegui ◽  
Natalia Valenzuela-Faccini ◽  
Cesar Quecano-Rosas ◽  
Darlyng Zabala-Muñoz ◽  
Michel Perez-Garzon

Abstract Background The new coronavirus disease 2019 pandemic has spread throughout most of the world. Cerebral venous thrombosis is a rare thromboembolic disease that can present as an extrapulmonary complication in coronavirus disease 2019 infection. Case presentation We report the case of a Hispanic woman with Down syndrome who has coronavirus disease 2019 and presents as a complication extensive cerebral venous thrombosis. Conclusions Cerebral venous thrombosis is a rare thromboembolic disease that can present as an extrapulmonary complication in coronavirus disease 2019 infection. In the absence of clinical and epidemiological data, it is important to carry out further investigation of the risk factors and pathophysiological causes related to the development of cerebrovascular thrombotic events in patients with Down syndrome with coronavirus disease 2019 infection.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Zai-Qiang Zhang ◽  
Jia-Wang Ding

Abstract Background While the perforation of the atrial wall and aortic sinus after closure of an atrial septal defect (ASD) is rare, it’s life-threatening, with rapid progress and high mortality. To the best of our knowledge, 21 similar cases have been reported since 1976. Case presentation We report a 16-year-old male whose atrial septal defect (ASD) was closed using a 12-mm Amplatzer septal occluder (ASO). Atrial wall and aortic sinus perforation occurred 3 months after transcatheter closure, and the patient was discharged after emergency operation. He was discharged on the 12th postoperative day in good overall condition. Conclusions With this case report, we want to illustrate that although percutaneous closure of ASD is regarded as a routine procedure, we should not forget the potentially lethal complications, especially cardiac erosion. Therefore, we should carefully evaluate the risk of erosion before surgery, and careful lifelong follow-up is needed.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Ahalyaa Sivashangar ◽  
Lallindra Gooneratne ◽  
Barnaby Clark ◽  
David Rees ◽  
Saroj Jayasinghe ◽  
...  

Abstract Background Erythrocyte pyruvate kinase is expressed under the control of the PKLR gene located on chromosome 1q21. Pyruvate kinase catalyzes the final steps of the glycolytic pathway and creates 50% of the red cell total adenosine triphosphate. Pyruvate kinase deficiency is the commonest glycolytic defect causing congenital non-spherocytic hemolytic anemia inherited in an autosomal recessive trait in which homozygotes and compound heterozygotes are common. Over 200 mutations have been described in patients with pyruvate kinase deficiency. This case report identifies a new pathogenic variant in PKLR gene detected in a patient with severe pyruvate kinase deficiency. Case presentation A Sri Lankan Sinhalese girl who developed neonatal anemia and jaundice within 24 hours of birth with mild hepatomegaly. She was from a nonconsanguineous marriage and had two siblings who had no hematological disorders. She had repeated admissions due to similar illnesses and at the age of 8 years was found to have pyruvate kinase deficiency associated with a novel homozygous pathogenic variant c.507+1delG in the PKLR gene. Conclusions A novel genetic variant in PKLR gene, consistent with pyruvate kinase deficiency, was detected in a Sri Lankan girl. This genetic variant may be specific to the Asian population and requires further studies.


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