scholarly journals Scoliosis Surgery in Cystic Fibrosis: Surgical Considerations and the Multidisciplinary Approach of a Rare Case

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
George I. Mataliotakis ◽  
Athanasios I. Tsirikos ◽  
Karen Pearson ◽  
Don S. Urquhart ◽  
Carolyn Smith ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Enzyme ◽  
Deformity Correction ◽  
Posterior Spinal Fusion ◽  
Major Surgery ◽  
Scoliosis Surgery ◽  
Total Blood ◽  
Caucasian Girl ◽  
Postoperative Intensive Care ◽  
Scoliosis Correction

Spinal deformity in patients with cystic fibrosis (CF) is usually mild requiring no treatment. These patients are rarely considered as surgical candidates for scoliosis correction, as the pulmonary condition and other comorbidities increase the risk of general anaesthesia and recovery. This paper reviews all the literature up to date with regard to scoliosis in patients with CF and reports this unique case of a 14-year-old Caucasian girl with progressive scoliosis, who was treated surgically at the age of 17. She underwent a posterior spinal fusion T2-L3 with the use of unilateral segmental instrumentation. Preoperative workup included respiratory, cardiac, anaesthetic, endocrine, and dietician reviews, as well as bone density optimisation with zoledronic acid and prophylactic antibiotics. Surgical time was 150 minutes and intraoperative blood loss was 47% of total blood volume. Postoperative intensive care included noninvasive ventilation, antibiotic cover, pain management, chest physiotherapy, pancreatic enzyme supplementation, and nutritional support. She was discharged on day 9. At follow-up she had a good cosmetic outcome, no complaints of her back, and stable respiratory function. Multidisciplinary perioperative care and meticulous surgical technique may reduce the associated risks of major surgery in CF patients, while achieving adequate deformity correction and a good functional outcome.

scholarly journals Brachial Plexus Injury due to Central-line Insertion during Kypho-scoliosis Deformity Correction

2021 ◽  
Vol 11 (7) ◽  
Author(s):  
Hitesh N. Modi ◽  
Shakti A. Goel ◽  
Arvind Sharma ◽  
Udit Patel
Keyword(s):  
Brachial Plexus ◽  
Brachial Plexus Injury ◽  
Venous Pressure ◽  
Deformity Correction ◽  
Central Line ◽  
Sensory Loss ◽  
Scoliosis Surgery ◽  
Central Line Insertion ◽  
Line Insertion ◽  
Scoliosis Correction

Introduction: Internal Jugular Venous (IJV) cannulation or central-line insertion is frequently performed during kyphoscoliosis deformity correction surgery or spine surgery with high risk. This helps monitor central venous pressure and administer medicines when required. Although many complications of IJV cannulation have been reported in the literature, its effect on brachial plexus is not known. The objective of this paper was to report a rare complication of IJV during scoliosis surgery. Case Presentation: We reported a case of 27-year-old male who was operated for severe kyphoscoliosis correction where preoperatively IJV cannulation was done. Repeated attempts were done during IJV cannula insertion due to altered anatomy. Eventually, cannula insertion was done using ultrasound modality and surgery for correction was done. Postoperatively patient developed right upper extremity weakness and sensory loss although the clinical result of kyphoscoliosis correction was acceptable. EMG-NCV study proved it brachial plexus injury. The patient was treated with intravenous steroid and physiotherapy. The patient recovered completely within 6 months of surgery. Conclusion: We reported a case of kyphoscoliosis deformity corrective surgery where IJV cannulation led to brachial plexus injury and was eventually recovered with medications and physiotherapy. Keywords: Scoliosis correction, Central-line insertion, Brachial plexus injury.

scholarly journals Dandy-Walker Syndrome Associated with Scoliosis: Clinical Presentation, Preoperative Assessment, and Treatment

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Athanasios I. Tsirikos ◽  
Sarah J. Wordie
Keyword(s):  
Skeletal Maturity ◽  
Surgical Techniques ◽  
Deformity Correction ◽  
Preoperative Assessment ◽  
Posterior Spinal Fusion ◽  
Postoperative Recovery ◽  
Uneventful Recovery ◽  
Scoliosis Correction ◽  
Assessment And Treatment ◽  
Cranial Fossa

Dandy-Walker syndrome (DWS) affects the posterior cranial fossa resulting in characteristic dysmorphic facial and body features. Scoliosis is not typically reported as an extracranial manifestation of this condition. We present a 12-year-old female patient who developed a right thoracic scoliosis measuring 60° with increased lumbar lordosis. Scoliosis correction was indicated to alleviate back pain, improve cosmesis, and prevent respiratory complications. A multidisciplinary preoperative assessment included cardiac, respiratory, anaesthetic, and neurology reviews. She underwent a posterior spinal fusion from T2 to L3 with pedicle hook/screw and rod instrumentation and a combination of locally harvested autologous and allograft bone. This resulted in excellent deformity correction and a balanced spine in the coronal and sagittal planes. The patient made an uneventful recovery and returned gradually to her normal level of activities. She was monitored in clinic until she completed her growth (4 years after surgery); the satisfactory surgical outcome was maintained at follow-up and was associated with high patient satisfaction. Scoliosis can occur in children with DWS with resemblance to adolescent idiopathic scoliosis in regard to type of coronal deformity, age at presentation, surgical techniques, and postoperative recovery. Early identification of scoliosis in patients with DWS can allow preoperative planning and prompt surgical management in order to reduce the risk of significant morbidity which can occur if the scoliosis is allowed to deteriorate. Excellent deformity correction can be achieved and maintained beyond skeletal maturity in order to improve physical appearance, as well as preserve level of function and quality of life.

scholarly journals Decreased Wait Time and Increased Satisfaction With Bedside Pancreatic Enzyme Dosing for the Inpatient Adolescent With Cystic Fibrosis: A Quality Improvement Project Comparing Enzyme Self-Administration to Nurse Administration

2021 ◽  
Vol 8 ◽  
pp. 237437352098149
Author(s):  
Brandi Middour-Oxler ◽  
Margaret Gettis ◽  
Betsy Dye
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Enzyme ◽  
Wait Time ◽  
Self Administration ◽  
Nurse Satisfaction ◽  
Improvement Project ◽  
Hospital Unit ◽  
Pediatric Inpatient ◽  
Inpatient Adolescent ◽  
Enzyme Delivery

For children with cystic fibrosis (CF), enzymes are essential with meals to absorb nutrients and ensure adequate growth. When hospitalized, CF patients typically rely on nurse-administered medications. Recently, a pediatric hospital unit began allowing adolescents with CF enzymes at the bedside. Postimplementation, a satisfaction questionnaire was administered to participating patients and nurses measuring patient and nurse satisfaction with access to bedside enzymes versus nurse administration and overall time for enzyme delivery. The survey utilized a 5-point Likert scale. The wait time for pancreatic enzymes decreased for self-administered enzymes when compared to those that were nurse administered. All (11/11) patients and 86% (12/14) of nurses preferred the self-administration of enzymes. Hospitalized pediatric CF patients and nurses had higher levels of satisfaction with enzyme self-administration. Immediate access to enzymes in room safes impact patient autonomy, reflecting home self-care practices. Decreases in wait times optimize nutritional growth and healing while hospitalized. As a result, a new limited scope policy allowing patient-administered enzymes is now in place in the pediatric inpatient CF unit.

scholarly journals Efficacy and Safety of a New Formulation of Pancrelipase (Ultrase MT20) in the Treatment of Malabsorption in Exocrine Pancreatic Insufficiency in Cystic Fibrosis

2010 ◽  
Vol 2010 ◽  
pp. 1-7 ◽  
Author(s):  
Michael W. Konstan ◽  
Theodore G. Liou ◽  
Steven D. Strausbaugh ◽  
Richard Ahrens ◽  
Jamshed F. Kanga ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
Gastrointestinal Symptoms ◽  
Signs And Symptoms ◽  
Exocrine Pancreatic Insufficiency ◽  
Efficacy And Safety ◽  
Protein Nitrogen ◽  
Enzyme Replacement ◽  
New Formulation

Background. Pancreatic enzyme replacement therapy is the standard of care for treatment of malabsorption in patients with cystic fibrosis (CF) and exocrine pancreatic insufficiency (PI).Aim. To evaluate efficacy and safety of a new formulation of pancrelipase (Ultrase MT20) in patients with CF and PI. Coefficients of fat absorption (CFA%) and nitrogen absorption (CNA%) were the main efficacy parameters. Safety was evaluated by monitoring laboratory analyses, adverse events (AEs), and overall signs and symptoms.Methods. Patients (n=31) were randomized in a crossover design comparing this pancrelipase with placebo during 2 inpatient evaluation periods (6-7 days each). Fat and protein/nitrogen ingestion and excretion were measured from food diaries and 72-hour stool collections. CFA% and CNA% were calculated for each period and compared.Results. Twenty-four patients provided analyzable data. This pancrelipase increased mean CFA% and CNA% (+34.7% and +25.7%, resp.,P<.0001for both), reduced stool frequency, and improved stool consistency compared with placebo. Placebo-treated patients reported more AEs, with gastrointestinal symptoms being the most frequently reported AE.Conclusions. This pancrelipase is a safe and effective treatment for malabsorption associated with exocrine PI in patients with CF.

scholarly journals Study of the effectiveness of supplementary feeding in patients with inherited enzyme deficiency

2019 ◽  
Vol 81 (2) ◽  
pp. 239-244
Author(s):  
L. V. Ulyanova ◽  
V. S. Ledneva ◽  
N. S. Burdina ◽  
M. I. Talykova ◽  
A. S. Ivannikova ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Pancreatic Enzyme ◽  
Diet Therapy ◽  
Enzyme Deficiency ◽  
Control Group ◽  
Anthropometric Parameters ◽  
Group 2 ◽  
The Right ◽  
Supplementary Nutrition

The successful provision of optimal nutrition in children with serious diseases depends on the right diet, as well as the addition of specialized mixtures for its correction. The aim of the study was to study the effectiveness of supplemental nutrition in hereditary fermentopathy (cystic fibrosis) in children. The work included a three-year follow-up of 69 children aged 3 to 15 years suffering from cystic fibrosis. Patients were divided into two groups: 37 patients with cystic fibrosis group 1, receiving a modified version of treatment with additional enteral nutrition and 32 patients with cystic fibrosis group 2, receiving traditional treatment. The study conducted a comparative analysis of changes in trophic status in patients using nutritional support mixture produced in the Russian Federation – "Nutrien-standard"in diet therapy. Laboratory, functional and anthropometric parameters were monitored once a month. A significant increase in the physical development of patients was obtained only in the second year of use in the diet of supplementary nutrition, p<0.05. At the 3rd year of treatment, the results of positive dynamics of nutritional status in both groups were confirmed, while the increase in body mass index in the main group was 6.7%, and in the control group-only 1% (p<0.05). It is noted that the use of this mixture allows to achieve positive dynamics of nutritional status in 27% of patients and in 73% of cases to completely eliminate it. The obtained results prove the expediency of additional use of "Nutrient-standard" mixture in the complex therapy of patients with hereditary pancreatic enzyme deficiency, cystic fibrosis

scholarly journals Coefficients of Fat and Nitrogen Absorption in Healthy Subjects and Individuals with Cystic Fibrosis

2007 ◽  
Vol 12 (1) ◽  
pp. 47-52
Author(s):  
Drucy Borowitz ◽  
Michael W. Konstan ◽  
Anna O'Rourke ◽  
Morty Cohen ◽  
Leslie Hendeles ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Healthy Subjects ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
High Protein Diet ◽  
Fat Absorption ◽  
Nitrogen Absorption ◽  
High Fat ◽  
Food Dye ◽  
Median Test

BACKGROUND We sought to compare the differences of coefficient of fat absorption (CFA) and coefficient of nitrogen absorption (CNA) in healthy individuals and those with cystic fibrosis (CF) and to study the precision of CFA and CNA. METHODS Sixteen healthy and 23 subjects with CF and pancreatic insufficiency ate a high-fat, high-protein diet for 72 hours; stool was collected between blue food dye markers to determine CFA and CNA. Subjects with CF withheld pancreatic enzymes. Tests were repeated on 5 of the CF and 10 of the healthy subjects. RESULTS In healthy subjects, mean CFA was 93.5% ± 2.7%; mean CNA was 88.1% ± 5%. Median test-retest in 10 healthy subjects was +0.7% CFA (range, −8.1% to + 5.9%) and +0.9% CNA (range, −14.6% to +6.8%). For subjects with CF, mean CFA was 38.5% ± 14.7% and mean CNA was 52.2% ± 11.4%. Median test-retest change in 5 subjects with CF was −6.9% CFA (range, −19.7% to +42.8%) and +14.7% CNA (range, −6.4% to +42.8%). CONCLUSIONS CFA and CNA have inconsistent precision in CF. The limitations of CFA as a measure of steatorrhea correction in CF should be recognized in studies of pancreatic enzyme supplements.

scholarly journals The role of nutrition and pancreatic enzyme replacement therapy in children with cystic fibrosis

2021 ◽  
Vol 4 (2) ◽  
pp. 84-93
Author(s):  
Muzal Kadim ◽  
William Cheng
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
Nutritional Intervention ◽  
Enzyme Replacement ◽  
Pancreatic Enzyme Replacement Therapy

Background Cystic fibrosis (CF) is an inherited genetic disorder with high mortality and morbidity. CF is strongly correlated with malnutrition due to higher energy losses, pancreatic insufficiency, chronic inflammation, higher resting energy expenditure, and feeding problems. Malnutrition in CF patients associated with worse survival. Thus, appropriate and prompt nutritional intervention should be addressed to reduced malnutrition in CF patients. Methods The literature search was performed on 9 August 2021 in four major databases such as MEDLINE, EBSCOhost, Cochrane Reviews, and Web of Sciences to find the role of nutrition and pancreatic enzyme replacement therapy in pediatrics population with cystic fibrosis. Recent findings In recent decades, early nutritional management and pancreatic enzyme replacement therapy (PERT) have been shown to improve CF patient’s outcomes. Nutrition should be given in higher calories compared to healthy individuals with close and regular nutritional status monitoring. High protein and fat diets are essential for CF patient’s overall survival. Adequate level of micronutrients should be ensured to avoid morbidity caused by micronutrients deficiency. Regular pancreatic insufficiency screening should be done annually in order to start PERT early.  Further research focusing on body composition, growth chart, protein intake, and PERT are needed to further improve the management of CF patient. Conclusion Nutritional intervention and PERT play an important role in prolonging CF patient survival. Both treatments should be initiated early with nutritional status close monitoring and tailored to each individual. Collaboration with parents and children is critical to warrant that CF patients followed the dietary advice.

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