Perioperative management for functional ganglioneuroma in a 2-year-old child with multiple congenital defects and COVID-19: A case report (Preprint)

2021 ◽  
Author(s):  
Pragadeshwaran Rajendran ◽  
Habib Karim ◽  
Vikramjit Singh ◽  
Shruti Bias
Keyword(s):  
Decision Making ◽  
Perioperative Management ◽  
Septal Defect ◽  
Hormone Secretion ◽  
Perioperative Period ◽  
Congenital Defects ◽  
Rare Entity ◽  
Palpable Mass ◽  
Life Threatening ◽  
Surgical Manipulation

UNSTRUCTURED Unlike neuroblastoma, ganglioneuroma is a rare entity in children younger than seven years of age. Further, these tumors are mostly inactive endocrinologically and present as abdominal pain or palpable mass. Unfortunately, when functional, they mimic or occasionally co-exist with pheochromocytoma. While perioperative management of pheochromocytoma has evolved a lot, very little is known for the management of functioning ganglioneuroma. Hormone secretion due to surgical manipulation and anesthesia can precipitate life-threatening complications. The risk further increases when associated with other congenital comorbidities. Furthermore, corona pandemic as well as COVID-19 infection in the perioperative period is another new challenge. We report perioperative management and outcome of a functioning retroperitoneal ganglioneuroma in a 2-year-old child, having a ventricular septal defect, spina bifida occulta, and COVID-19. The case also highlights decision-making challenges during the COVID-19 pandemic.

scholarly journals Perioperative management of a patient with primary hypoparathyroidism and severe hypocalcaemia for lumbar spine surgery

2016 ◽  
Vol 03 (01) ◽  
pp. 049-051
Author(s):  
Deepak Madankar ◽  
Sheetal Samel ◽  
Abhay Ganar ◽  
Neelesh Mathankar
Keyword(s):  
Perioperative Management ◽  
Muscle Power ◽  
Lumbar Discectomy ◽  
Medical Emergency ◽  
Lumbar Spine Surgery ◽  
Rare Entity ◽  
Ionised Calcium ◽  
Life Threatening ◽  
Severe Hypocalcaemia ◽  
Intravenous Calcium

AbstractPrimary hypoparathyroidism and consequent hypocalcaemia in the absence of iatrogenic cause are a rare entity. Serum ionised calcium concentrations < 0.50 mmol l−1 are more frequently associated with life-threatening complications and constitute a medical emergency that necessitates intravenous calcium therapy. The anaesthesiologist should carefully look for the effects of hypocalcaemia on the heart, circulation, muscle power and blood coagulation. We report perioperative management of a case of hypoparathyroidism and associated hypocalcaemia posted for lumbar discectomy in prone position and its anaesthetic implications.

scholarly journals A Safety Management Case of Laparoscopic Colectomy in a Patient With Paroxysmal Nocturnal Hemoglobinuria

2019 ◽  
Vol 104 (7-8) ◽  
pp. 333-337
Author(s):  
Hiroshi Matsuno ◽  
Taishi Hata ◽  
Hidekazu Takahashi ◽  
Naotsugu Haraguchi ◽  
Mamoru Uemura ◽  
...  
Keyword(s):  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Perioperative Management ◽  
Safety Management ◽  
Lower Abdominal Pain ◽  
Perioperative Period ◽  
Right Hemicolectomy ◽  
Complement Protein ◽  
Humanized Monoclonal Antibody ◽  
Hemolytic Crisis ◽  
Life Threatening

Paroxysmal nocturnal hemoglobinuria is a rare and life-threatening disorder of acquired hemolytic anemia. Surgery is one of the major clinical situations that trigger hemolytic attack. Eculizumab is a humanized monoclonal antibody that binds the complement protein C5 and prevents complement-mediated hemolysis via inhibition of the terminal complement cascade. A 76-year-old woman received a diagnosis of ascending colon cancer during the search for the cause of right lower abdominal pain. She had received a diagnosis of paroxysmal nocturnal hemoglobinuria and been followed for 26 years at our hospital. We planned to start eculizumab for perioperative management in order to reduce the risk of the patient developing hemolytic crisis as a result of surgery. We administered 600 mg of eculizumab on the 15th, 8th, and 1st preoperative days. The levels of serum complement and lactate dehydrogenase decreased with the first administration of eculizumab. Laparoscopic right hemicolectomy was performed successfully. The patient had good postoperative progress. We administered 600 mg of eculizumab on the 6th postoperative day and 900 mg of eculizumab on the 13th postoperative day. She was discharged from hospital on the 16th postoperative day. We started use of eculizumab before surgery for safety in the management of the operation and during the perioperative period. When we enforce the elective operation for patients with paroxysmal nocturnal hemoglobinuria who do not start treatment of eculizumab, we recommend the use of eculizumab for perioperative management of patients with paroxysmal nocturnal hemoglobinuria.

Percutaneous Treatment of Congenital Defects of the Inter-atrial Septum in Adults – Recent Advances and Persistent Pitfalls

2009 ◽  
Vol 4 (1) ◽  
pp. 76
Author(s):  
James Slater ◽  
Mark Fisch ◽  
◽  
Keyword(s):  
Atrial Septal Defect ◽  
Adult Patient ◽  
Patent Foramen Ovale ◽  
Septal Defect ◽  
Percutaneous Treatment ◽  
Atrial Septum ◽  
Congenital Defects ◽  
Foramen Ovale ◽  
Anatomy And Physiology ◽  
Specific Tissue

William Harvey was the first scientist to describe the heart as consisting of separate right- and left-sided circulations. Our understanding of the heart’s anatomy and physiology has grown significantly since this landmark discovery in 1628. Today, we recognise not only the importance of these separate systems, but also the specific tissue that divides them. Our growing understanding of the inter-atrial septum has allowed us to identify defects within this structure and develop effective percutaneous devices for closure of these defects in the adult patient. This article discusses the formation of a patent foramen ovale (PFO) and atrial septal defect (ASD). In addition, we describe the medical illnesses caused by these defects and summarise the indications and risks related to percutaneous closure of these defects. We also report the most up-to-date transcatheter therapeutic options for closure of these common congenital defects in the adult patient.

scholarly journals Perforation of the atrial wall and aortic sinus after closure of an atrial septal defect with an Atriasept occluder: a case report

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Zai-Qiang Zhang ◽  
Jia-Wang Ding
Keyword(s):  
Case Report ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Emergency Operation ◽  
Atrial Wall ◽  
Aortic Sinus ◽  
Case Presentation ◽  
Lethal Complications ◽  
Life Threatening

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.

Of Love and Laetrile: Medical Decision Making in a Child's Best Interests

1979 ◽  
Vol 5 (3) ◽  
pp. 269-294
Author(s):  
Eve T. Horwitz
Keyword(s):  
Decision Making ◽  
Medical Treatment ◽  
Burden Of Proof ◽  
State Intervention ◽  
Convincing Evidence ◽  
State Agency ◽  
Best Interests ◽  
Medical Decision ◽  
Appropriate Treatment ◽  
Life Threatening

AbstractTwo recent cases have raised important questions concerning the appropriateness of state intervention in parental choices of unorthodox medical treatment for children with life-threatening conditions. This Note first discusses whether, and if so, when, state intervention in a child's treatment selection by its parents is appropriate, and then analyzes the tests a court should apply in deciding upon an appropriate treatment. The Note recommends a decision-making approach that requires the appropriate state agency to prove, by clear and convincing evidence, that the parents' choice of medical treatment either is directly or is indirectly harming their child. Under this approach, if the state meets its burden of proof the court then must apply the ‘best interests’ test, rather than the ‘substituted judgment’ test, to choose an appropriate medical treatment for the child.

scholarly journals Physicians’ decreased tendency to choose palliative care for patients with advanced dementia between 1999 and 2015

2021 ◽  
Vol 20 (1) ◽  
Author(s):  
Saila Haapasalmi ◽  
Reetta P. Piili ◽  
Riina Metsänoja ◽  
Pirkko-Liisa I. Kellokumpu-Lehtinen ◽  
Juho T. Lehto
Keyword(s):  
Decision Making ◽  
Palliative Care ◽  
Legal Protection ◽  
Postal Survey ◽  
Advanced Dementia ◽  
Background Factors ◽  
Physician Decision Making ◽  
Care Approach ◽  
Life Threatening ◽  
The Impact

Abstract Background Physicians’ decision-making for seriously ill patients with advanced dementia is of high importance, especially as the prevalence of dementia is rising rapidly, and includes many challenging ethical, medical and juridical aspects. We assessed the change in this decision-making over 16 years (from 1999 to 2015) and several background factors influencing physicians’ decision. Methods A postal survey including a hypothetical patient-scenario representing a patient with an advanced dementia and a life-threatening gastrointestinal bleeding was sent to 1182 and 1258 Finnish physicians in 1999 and 2015, respectively. The target groups were general practitioners (GPs), surgeons, internists and oncologists. The respondents were asked to choose between several life-prolonging and palliative care approaches. The influence of physicians’ background factors and attitudes on their decision were assessed. Results The response rate was 56%. A palliative care approach was chosen by 57 and 50% of the physicians in 1999 and 2015, respectively (p = 0.01). This change was statistically significant among GPs (50 vs 40%, p = 0.018) and oncologists (77 vs 56%, p = 0.011). GPs chose a palliative care approach less often than other responders in both years (50 vs. 63% in 1999 and 40 vs. 56% in 2015, p < 0.001). In logistic regression analysis, responding in 2015 and being a GP remained explanatory factors for a lower tendency to choose palliative care. The impact of family’s benefit on the decision-making decreased, whereas the influence of the patient’s benefit and ethical values as well as the patient’s or physician’s legal protection increased from 1999 to 2015. Conclusions Physicians chose a palliative care approach for a patient with advanced dementia and life-threatening bleeding less often in 2015 than in 1999. Specialty, attitudes and other background factors influenced significantly physician decision-making. Education on the identification and palliative care of the patients with late-stage dementia are needed to make these decisions more consistent.

scholarly journals Perioperative management with biologics on severe aortic valve regurgitation caused by Behçet syndrome: the experience from a single center

2021 ◽  
Vol 12 ◽  
pp. 204062232110267
Author(s):  
Luxi Sun ◽  
Jinjing Liu ◽  
Xiufeng Jin ◽  
Zhimian Wang ◽  
Lu Li ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Perioperative Management ◽  
Perioperative Period ◽  
Valve Regurgitation ◽  
Aortic Valve Regurgitation ◽  
Behçet Syndrome ◽  
Reactive Protein ◽  
Behcet Syndrome ◽  
Sparing Effect ◽  
Median Interval

Background: To investigate the efficacy and safety of biologics in the perioperative management of severe aortic valve regurgitation (AR) caused by Behçet syndrome (BS). Methods: We retrospectively analyzed 20 patients with severe AR caused by BS who were all treated with biologics during the perioperative period of cardiac surgeries in our center between February 2016 and October 2020. Results: A total of 20 patients with severe AR were enrolled, including 19 males and 1 female, with a mean age of 39.1 ± 8.8 years and a median course of 8 [interquartile range (IQR) 5.25–10.00] years. Before biologic administration, 92.9% of the patients who underwent aortic valve replacement had failed conventional therapy and developed postoperative paravalvular leakage (PVL) at a median interval of 4 months. Biologics were administered with background glucocorticoids (GCs) and immunosuppressants during the perioperative period for 22 aortic valve surgeries, including preoperatively with a median interval of 3.5 (IQR 2.75–4.25) months in 13 cases and within 3 months postoperatively in 9 cases. After a median follow up of 21 (IQR 15–32) months, 2 out of 13 cases (15.4%) preoperatively, and 1 out of 9 cases (11.1%) postoperatively treated with biologics developed PVL, and the rest were event free. The Behçet’s Disease Current Activity Form score improved significantly (7 versus 0, median, p < 0.0001). Decrease of erythrocyte sedimentation rate [25.0 (IQR 11.00–36.25) mm/h versus 6.5 (IQR 4.0–8.8) mm/h, p < 0.001], and C-reactive protein [20.77 (IQR 7.19–29.58) mg/l versus 1.53 (IQR 0.94–2.92) mg/l, p = 0.001] were achieved rapidly and effectively. The GC dosage tapered from 40 (IQR 30–60) mg/d to 10 (IQR 5–11.25) mg/d, p < 0.0001. Immunosuppressants were tapered in number and dosage in 6 (30%) and 20 patients (100%), respectively. No serious adverse event was observed. Conclusion: Our study suggests that biologics were effective and well tolerated for the perioperative management of severe and refractory AR caused by BS, which significantly reduced the occurrence of postoperative PVL and had favorable GC- and immunosuppressant-sparing effect.

A critical reappraisal of MIB-1 labelling index significance in a large series of pituitary tumours: secreting versus non-secreting adenomas.

2002 ◽  
pp. 103-113 ◽  
Author(s):  
M L Jaffrain-Rea ◽  
D Di Stefano ◽  
G Minniti ◽  
V Esposito ◽  
A Bultrini ◽  
...  
Keyword(s):  
Operative Treatment ◽  
Hormone Secretion ◽  
Tumour Volume ◽  
Pituitary Tumour ◽  
Labelling Index ◽  
Pituitary Tumours ◽  
Life Threatening ◽  
The Mean ◽  
Monoclonal Antibody Mib 1 ◽  
Mib 1

Pituitary tumours are usually benign neoplasia, but may have a locally aggressive or malignant evolution. This study aimed to identify factors which mostly influence their proliferative activity, in order to clarify its value for clinical and research purposes. The proliferative index was determined in a prospective series of 132 pituitary tumours as the percentage of monoclonal antibody MIB-1-immunopositive cells and referred to as the MIB-1 labelling index (LI). Its distribution was analysed according to both univariate and multivariate models. A life-threatening pituitary tumour is presented separately. The mean LI was 1.24+/-1.59%, with significant differences between clinically secreting (CS) and clinically non-secreting (CNS) adenomas. In CS adenomas (n=65), LI was highly variable and markedly influenced by pre-operative pharmacological treatment (0.80+/-1.03 vs 2.06+/-2.39% in treated vs untreated cases, P=0.009); it decreased with patient's age (P=0.025, r=0.28) and increased with tumour volume and invasiveness. The influence of pre-operative treatment and macroscopic features on LI in this group was confirmed by multivariate analysis. In CNS adenomas (n=67), LI distribution was less variable than in CS adenomas (P<0.0001), it was age-independent and correlations with tumour volume, invasiveness or recurrence did not reach significance. In a rapidly growing parasellar tumour, the mean LI was 24% at first surgery and exceeded 50% at second surgery performed 4 months later. LI should be interpreted according to hormone secretion and pre-operative treatment. Unusually high LI values deserve particular attention.

Chylothorax Associated With Tricuspid Dysplasia and Atrial Septal Defect in a Bullmastiff

2009 ◽  
Vol 45 (2) ◽  
pp. 78-83 ◽  
Author(s):  
Alessia Diana ◽  
Carlo Guglielmini ◽  
Fabio Acocella ◽  
Flavio Valerio ◽  
Mario Cipone
Keyword(s):  
Pleural Effusion ◽  
Atrial Septal Defect ◽  
Ace Inhibitor ◽  
Septal Defect ◽  
Converting Enzyme ◽  
Pedicle Flap ◽  
Life Threatening ◽  
Chylous Leakage ◽  
Chylous Effusion ◽  
Omental Pedicle Flap

Transudate pleural effusion associated with tricuspid dysplasia and ostium secundum-type atrial septal defect was diagnosed in a 14-month-old bullmastiff. Following administration of furosemide and an angiotensin-converting enzyme (ACE) inhibitor, the dog remained free of pleural effusion for 10 months, until he showed severe dyspnea due to chylothorax. Medical therapy was unsuccessful to avoid recurrence of life-threatening pleural chylous effusion. Ligation of the thoracic duct and apposition of an omental pedicle flap were effective in the resolution of pleural chylous leakage.

scholarly journals Case Report: Ascending Aortic Pseudo-Aneurysm Following Ventricular Septal Defect Repair in a 4-Year-Old Girl

2021 ◽  
Vol 9 ◽  
Author(s):  
Xinya Li ◽  
Hong Zhou ◽  
Rui Zhang ◽  
Jing Zhao ◽  
Tian Li ◽  
...  
Keyword(s):  
Case Report ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Circulatory Arrest ◽  
Massive Hemorrhage ◽  
Purse String ◽  
Defect Repair ◽  
Life Threatening ◽  
Pseudo Aneurysm ◽  
Computed Tomograph

Pseudo-aneurysm is a fatal disease, and the main cause of death is massive hemorrhage secondary to the rupture of the aneurysm. This case report aims to evaluate the effects of pseudo-aneurysm excision procedure on the disease. A 4-year-old girl was readmitted on the 20th day after ventricular septal defect (VSD) closure procedure with a high fever of 40°C; aortic pseudo-aneurysm was suspected based on a spherical cystic echo (82 × 76 mm) of the ascending aorta which was detected by ultrasonic cardiogram, and the diagnosis was confirmed by an aortic computed tomograph angiography (CTA) examination and intraoperative findings. Treatment included emergency pseudo-aneurysm excision procedure and antibiotic therapy. The aortic pseudo-aneurysm was surgically removed under deep hypothermia and circulatory arrest. Antibiotics were applied according to the bacterial culture results. The pseudo-aneurysm was excised successfully, and the patient achieved a good recovery. Our case suggests that the postoperative ascending aortic pseudo-aneurysm was probably due to inappropriate purse-string suture and/or local or systematic infection, so extra precautions should be taken to avoid this life-threatening complication.

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