Multistage surgical repair for split notochord syndrome with neuroenteric fistula: case report

2020 ◽  
pp. 1-9
Author(s):  
Mansour Mathkour ◽  
Tyler Scullen ◽  
Brendan Huang ◽  
Cassidy Werner ◽  
Edna E. Gouveia ◽  
...  
Keyword(s):  
Surgical Repair ◽  
Surgical Techniques ◽  
Multiple Organ ◽  
Case Description ◽  
Review Of The Literature ◽  
Viable Option ◽  
Unique Case ◽  
Organ Systems ◽  
The Central Nervous System

Split notochord syndrome (SNS) is a rare congenital defect of the central nervous system and has been associated with several anomalies affecting multiple organ systems. One association has been communication with the gastrointestinal tract and the spine, previously identified as a neuroenteric fistula (NEF). Here, the authors describe the unique case of a female infant with SNS and NEF treated with a multistage surgical repair. The three-stage operative plan included a two-stage repair of the defect and temporary subgaleal shunting followed by delayed ventriculoperitoneal shunt placement. The infant recovered well postsurgery and over a 5-year follow-up. A case description, surgical techniques, and rationale are reported. Additionally, a systematic review of the literature utilizing the MEDLINE database was performed.Treatment of SNS with NEF using a multidisciplinary multistaged approach to repair the intestinal defect, close the neural elements, and divert cerebrospinal fluid to the peritoneum is shown to be a safe and viable option for future cases.

scholarly journals Attenuation of COVID-19-induced cytokine storm in a young male patient with severe respiratory and neurological symptoms

2021 ◽  
Author(s):  
Christian Muschitz ◽  
Anita Trummert ◽  
Theresa Berent ◽  
Norbert Laimer ◽  
Lukas Knoblich ◽  
...  
Keyword(s):  
Decision Making ◽  
Male Patient ◽  
Young Male ◽  
Standard Of Care ◽  
Multiple Organ ◽  
Cytokine Storm ◽  
Invasive Ventilation ◽  
Organ Systems ◽  
The Central Nervous System

SummarySevere acute respiratory syndrome coronavirus type 2 (SARS-CoV-2), the etiological agent of coronavirus disease 2019 (COVID-19), produces protean manifestations and causes indiscriminate havoc in multiple organ systems. This rapid and vast production of proinflammatory cytokines contributes to a condition termed cytokine storm. A 35-year-old, otherwise healthy, employed, male patient was tested positive for COVID-19. He was admitted to the hospital on disease day 10 due to retarded verbal reactions and progressive delirium. On account of these conditions and the need for noninvasive/invasive ventilation, a combination treatment with baricitinib and remdesivir in conjunction with standard of care was initiated. The cytokine storm was rapidly blocked, leading to a vast pulmonary recovery with retarded recovery of the central nervous system. We conclude that the rapid blockade of the COVID-19-induced cytokine storm should be considered of avail as a principle of careful decision-making for effective recovery.

scholarly journals A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

Comparison of Doty and McGoon techniques for surgical reconstruction of congenital supravalvular aortic stenosis

2021 ◽  
pp. 1-8
Author(s):  
Mehmet Biçer ◽  
Mehmet Dedemoğlu ◽  
Oktay Korun ◽  
Hüsnü F. Altın ◽  
Okan Yurdakök ◽  
...  
Keyword(s):  
Aortic Stenosis ◽  
Surgical Repair ◽  
Pulmonary Stenosis ◽  
Surgical Techniques ◽  
Supravalvular Aortic Stenosis ◽  
Surgical Reconstruction ◽  
Residual Stenosis ◽  
Post Operative Period ◽  
Surgical Methods

Abstract Background: We aimed to compare the results of two surgical methods for the treatment of congenital supravalvular aortic stenosis. Methods: From May 2004 to January 2020, 29 patients underwent surgical repair for supravalvular aportic stenosis in a single centre. The perioperative evaluation of the patients was retrospectively reviewed. Results: Fifteen (51.7%) and 14 (48.2%) patients were treated with the Doty and the McGoon methods, respectively. The median age of our cohort was 4.5 (3.0–9.9) years. Ten (34.5%) patients had Williams–Beuren syndrome, and pulmonary stenosis was observed in 12 (41.3%) patients. The median follow-up time was 2.5 (0.7–7.3) years. On follow-up, five patients had residual stenosis with the McGoon technique and one with the Doty technique (p = 0.05). One patient died early in the post-operative period in the Doty group, and three patients were re-operated on due to restenosis in the McGoon group. Freedom from re-operation in the Doty group at 1, 3, 5, and 10 years was 100%. In the McGoon group, freedom from re-operation rates at the 1-, 3-, and 7-year follow-up were 100, 88.9, and 44.4%, respectively (p = 0.08). Conclusion: Our results with both surgical techniques suggest that supravalvular aortic stenosis can be treated with good results. The Doty method provided better relief for the supravalvular aortic segment, considering the residual stenosis and the re-operation rates.

scholarly journals Central Nervous System Infection with Histoplasma capsulatum

2019 ◽  
Vol 5 (3) ◽  
pp. 70 ◽  
Author(s):  
James Riddell ◽  
L. Joseph Wheat
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Histoplasma Capsulatum ◽  
Central Nervous System Infection ◽  
Treatment Strategies ◽  
Multiple Organ ◽  
Filamentous Form ◽  
Disseminated Infection ◽  
Organ Systems ◽  
The Central Nervous System

Histoplasmosis is an endemic fungal infection that may affect both immune compromised and non-immune compromised individuals. It is now recognized that the geographic range of this organism is larger than previously understood, placing more people at risk. Infection with Histoplasma capsulatum may occur after inhalation of conidia that are aerosolized from the filamentous form of the organism in the environment. Clinical syndromes typically associated with histoplasmosis include acute or chronic pneumonia, chronic cavitary pulmonary infection, or mediastinal fibrosis or lymphadenitis. Disseminated infection can also occur, in which multiple organ systems are affected. In up to 10% of cases, infection of the central nervous system (CNS) with histoplasmosis may occur with or without disseminated infection. In this review, we discuss challenges related to the diagnosis of CNS histoplasmosis and appropriate treatment strategies that can lead to successful outcomes.

scholarly journals Neurological, neuropsychiatric and neurodevelopmental complications of COVID-19

2020 ◽  
pp. 000486742096147
Author(s):  
Christos Pantelis ◽  
Mahesh Jayaram ◽  
Anthony J Hannan ◽  
Robb Wesselingh ◽  
Jess Nithianantharajah ◽  
...  
Keyword(s):  
Organ Damage ◽  
Multiple Organ ◽  
Global Pandemic ◽  
Organ Systems ◽  
Future Challenges ◽  
The Central Nervous System ◽  
The Impact ◽  
Collaborative Efforts ◽  
The Brain

Although COVID-19 is predominantly a respiratory disease, it is known to affect multiple organ systems. In this article, we highlight the impact of SARS-CoV-2 (the coronavirus causing COVID-19) on the central nervous system as there is an urgent need to understand the longitudinal impacts of COVID-19 on brain function, behaviour and cognition. Furthermore, we address the possibility of intergenerational impacts of COVID-19 on the brain, potentially via both maternal and paternal routes. Evidence from preclinical models of earlier coronaviruses has shown direct viral infiltration across the blood–brain barrier and indirect secondary effects due to other organ pathology and inflammation. In the most severely ill patients with pneumonia requiring intensive care, there appears to be additional severe inflammatory response and associated thrombophilia with widespread organ damage, including the brain. Maternal viral (and other) infections during pregnancy can affect the offspring, with greater incidence of neurodevelopmental disorders, such as autism, schizophrenia and epilepsy. Available reports suggest possible vertical transmission of SARS-CoV-2, although longitudinal cohort studies of such offspring are needed. The impact of paternal infection on the offspring and intergenerational effects should also be considered. Research targeted at mechanistic insights into all aspects of pathogenesis, including neurological, neuropsychiatric and haematological systems alongside pulmonary pathology, will be critical in informing future therapeutic approaches. With these future challenges in mind, we highlight the importance of national and international collaborative efforts to gather the required clinical and preclinical data to effectively address the possible long-term sequelae of this global pandemic, particularly with respect to the brain and mental health.

A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

An Asymptomatic Left Ventricular Pseudoaneurysm found incidentally at 12 months post Myocardial Infarction: Case Report and Review of the Literature

2020 ◽  
Vol 20 ◽  
Author(s):  
Christian Brooks ◽  
Heather Cooke
Keyword(s):  
Myocardial Infarction ◽  
Surgical Repair ◽  
Left Ventricular ◽  
Mechanical Complication ◽  
Review Of The Literature ◽  
Limited Data ◽  
Left Ventricular Pseudoaneurysm ◽  
History Of ◽  
Multi Disciplinary Team

Highlights: Left ventricular pseudoaneurysms are a rare mechanical complication of myocardial infarction. If found acutely following infarction (within 2 weeks, with some advocating up to 3 months), surgical repair is recommended due to their high risk of rupture.Whilst associated with chest pain, dyspnoea and heart failure, some individuals are asymptomatic, with the diagnosis made incidentally on routine follow-up often months to years post infarction. Less is known about the natural history of these chronic pseudoaneurysms, with concerns around their propensity to rupture perhaps less than the mortality risk of surgical repair.We present the case of a 70 year-old asymptomatic man who was found to have a 1.6cm left ventricular pseudoaneurysm found incidentally on routine transthoracic echocardiogram. at 12-months post posterior myocardial infarctionThe consensus opinion of our institution's multi-disciplinary team regarding further management of this patient, with reference to the current limited data on chronic pseudoaneurysms, will be discussed.

scholarly journals Preliminary experience with tacrolimus in managing Henoch-Schönlein purpura featuring extrarenal symptoms refractory to conventional therapy

2020 ◽  
Author(s):  
Yan Chen ◽  
Haitao Bai ◽  
Yang Yang ◽  
Zhulan Lian
Keyword(s):  
Adverse Reaction ◽  
Multiple Organ ◽  
Screening Tests ◽  
Henoch Schonlein Purpura ◽  
Open Label ◽  
Organ Systems ◽  
Tacrolimus Therapy ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Abstract Background Henoch-Schönlein purpura (HSP) is the commonest vasculitis of childhood, affecting multiple organ systems. The extrarenal manifestations are prominent in some HSP cases, for which no standard treatment is available. It is interesting to examine the value of tacrolimus in such cases. Findings Seventeen children received tacrolimus to manage their refractory extrarenal symptoms of HSP in an open-label prospective study. Tacrolimus was administered at daily dose of 0.05–0.15 mg/kg. Tacrolimus was effective in all cases after treatment for 7–21 days. Tacrolimus treatment resulted in complete resolution of extrarenal symptoms in 15 cases, and partial resolution in the remaining 2 cases. During follow-up, skin purpura reoccurred in 2 cases, abdominal pain and loss of follow-up in 1 case each. Drug-related adverse reaction was not observed in any case. Abnormal renal function test was identified in 7 patients by urinary screening tests before tacrolimus therapy, 4 of them received renal biopsy. Only one patient showed evidence of renal impairment during follow-up. Conclusions Tacrolimus works quickly without drug-related adverse reaction in managing the HSP patients featuring recurrent extrarenal symptoms. Our preliminary experience suggests good clinical efficacy and safety of tacrolimus in such off-label use.

scholarly journals Neuroinvasion of SARS-CoV-2 in human and mouse brain

2021 ◽  
Vol 218 (3) ◽  
Author(s):  
Eric Song ◽  
Ce Zhang ◽  
Benjamin Israelow ◽  
Alice Lu-Culligan ◽  
Alba Vieites Prado ◽  
...  
Keyword(s):  
Cortical Neurons ◽  
Immune Cell ◽  
Multiple Organ ◽  
Type I ◽  
Organ Systems ◽  
The Central Nervous System ◽  
Interferon Responses ◽  
The Brain ◽  
Immune Cell Infiltrates

Although COVID-19 is considered to be primarily a respiratory disease, SARS-CoV-2 affects multiple organ systems including the central nervous system (CNS). Yet, there is no consensus on the consequences of CNS infections. Here, we used three independent approaches to probe the capacity of SARS-CoV-2 to infect the brain. First, using human brain organoids, we observed clear evidence of infection with accompanying metabolic changes in infected and neighboring neurons. However, no evidence for type I interferon responses was detected. We demonstrate that neuronal infection can be prevented by blocking ACE2 with antibodies or by administering cerebrospinal fluid from a COVID-19 patient. Second, using mice overexpressing human ACE2, we demonstrate SARS-CoV-2 neuroinvasion in vivo. Finally, in autopsies from patients who died of COVID-19, we detect SARS-CoV-2 in cortical neurons and note pathological features associated with infection with minimal immune cell infiltrates. These results provide evidence for the neuroinvasive capacity of SARS-CoV-2 and an unexpected consequence of direct infection of neurons by SARS-CoV-2.

scholarly journals Klippel-Trenaunay Syndrome Causing Life-Threatening GI Bleeding: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Salih Samo ◽  
Muhammed Sherid ◽  
Husein Husein ◽  
Samian Sulaiman ◽  
Margaret Yungbluth ◽  
...  
Keyword(s):  
Vascular Malformations ◽  
Multiple Organ ◽  
Gi Bleeding ◽  
Blood Products ◽  
Review Of The Literature ◽  
Gi Tract ◽  
Organ Systems ◽  
Life Threatening ◽  
Klippel Trenaunay Syndrome ◽  
Congenital Syndrome

Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome of vascular malformations and soft tissue and bone hypertrophy. Vascular malformations can affect multiple organ systems. Involvement of the gastrointestinal (GI) tract is uncommon in KTS, but it can be a source of life-threatening bleeding. We report a case of a 32-year-old male with a known diagnosis of KTS who presented with a life-threatening rectal bleeding and was treated with proctosigmoidectomy and massive blood products transfusion. He expired after a long hospitalization. We then review the literature on KTS and management of some of its complications.

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