|Year : 2008 | Volume
| Issue : 6 | Page : 866
Child With Cystic Fibrosis And Pyopneumothorax For Emergency Thoracotomy For Decortication
Rakesh Garg1, Ashwani Kumar1, Mridu Paban Nath1, Dilip Shende2, Rajib Bhattacharyya3
1 Sr.Resident, Department of Anaesthesiology and Intensive Care, All India institute of Medical Sciences, Ansari Nagar, NewDelhi-110029, India
2 Additional Professor, Department of Anaesthesiology and Intensive Care, All India institute of Medical Sciences, Ansari Nagar, NewDelhi-110029, India
3 Associate Prof. Silchar Medical College, Department of Anaesthesiology and Intensive Care, All India institute of Medical Sciences, Ansari Nagar, NewDelhi-110029, India
|Date of Acceptance||25-Aug-2008|
|Date of Web Publication||19-Mar-2010|
Department of Anaesthesiology and Intensive Care, All India Institute of Medical Sciences, Ansari Nagar, NewDelhi-110029
Source of Support: None, Conflict of Interest: None
We describe a child of cystic fibrosis for emergency thoracotomy. A 7 year female diagnosed as cystic fibrosis, pyopneumothorax with pneumonia was posted for emergency left thoracotomy. On examination, child had decreased air entry on left side of chest and bilateral crepts. Anaesthesia was induced with fentanyl, propofol, vecuronium and sevoflurane in oxygen/air. Airway was secured with tracheal tube. Central venous line and caudal epidural catheter was placed. There were multiple episodes of desaturation and airway pressures were high. Child was shifted to ICU with tube in situ and put on ventilator and gradually weaned. Epidural infusion was initiated. Child had an uneventful recovery. Patient with cystic fibrosis for emergency thoracotomy requires proper choice of anaesthetic techniques along with perioperative pain relief.
Keywords: Cystic fibrosis, Thoracotomy, Pyopneumothorax, Anaesthesia, Analgesia
|How to cite this article:|
Garg R, Kumar A, Nath MP, Shende D, Bhattacharyya R. Child With Cystic Fibrosis And Pyopneumothorax For Emergency Thoracotomy For Decortication. Indian J Anaesth 2008;52:866
|How to cite this URL:|
Garg R, Kumar A, Nath MP, Shende D, Bhattacharyya R. Child With Cystic Fibrosis And Pyopneumothorax For Emergency Thoracotomy For Decortication. Indian J Anaesth [serial online] 2008 [cited 2020 Oct 21];52:866. Available from: https://www.ijaweb.org/text.asp?2008/52/6/866/60704
| Introduction|| |
Cystic fibrosis (CF) is a chronic, progressive autosomal recessive disease with an incidence of 1 in 2000 births caused by mutations in the CF transmembrane regulator protein gene ,,, . Earlier, 80% of affected infants died during the first year of life but survival and longevity have improved considerably now and anaesthesiologists encounter patients with CF, presenting either with complications of the disease or for unrelated surgery , . We describe a child of cystic fibrosis for emergency thoracotomy for pyopneumothorax and decortication.
| Case report|| |
Seven year 18 kg female, a known case of cystic fibrosis, presented in the paediatric casuality with the chief complaints of fever, cough along with an episode of blood tinged sputum for last 20 days and left sided chest pain with respiratory distress since one day. A diagnosis of pyopneumothorax with pneumonia was made. Emergency left thoracotomy and decortication was planned.
Patient had a history of multiple hospitalization for recurrent pneumonia since birth and managed with antibiotics, nebulisation, mucolytic agents and other conservative management. Parents gave history of inspissated mucus plugs in the sputum along with the cough. The younger sibling 4 year old female child was also a diagnosed case of CF.
At the age of 3 years, sweat test for diagnosis of CF revealed chloride concentration of 113.3 and 86.3mmol/L (total amount of sweat collected was 148 and 167mg respectively). The IgE for aspergillus fumigates was positive, 1.91 kUA/L. The absolute eosinophila count was 2666/mL (normal 100-1000/ml). IgE level was 2232 IU/mL (normal 0-52 IU/mL). Direct fluorescence for AFB in the BAL sample was negative.
On examination, child was sick looking, pulse rate148 beats /min, respiratory rate 36 breaths/min. Intercostal, subcostal, suprasternal recession was present. Auscultation of chest revealed decreased air entry on left side and bilateral crepts (Left>Right). Investigation revealed haemoglobin 11.1g/dL, TLC 19000 /mm 3 , blood sugar 98 mg/dL, chest X ray- left upper zone cavitatory lesion with air fluid levels, with left lower lobe collapse and right mid zone consolidation[Figure 1]a. Computed tomographic scan chest revealed left hydropneumothorax with loculations and collapse of left lower lobe along with cylindrical and varicose bronchiectasis with presence of impacted mucus in right lung bronchus (finger in glove appearance)[Figure 1]b.
In the operation room, monitors including pulse oximeter, electrocardiography, noninvasive blood pressure were applied. Oxygen saturation was 93% on room air. After preoxygenation, anaesthesia was induced with fentanyl 40 mcg, propofol 40 mg, sevoflurane in oxygen/air. Airway was secured with cuffed tracheal tube size 5.5 mm ID after achieving neuromuscular blockade with 1.8 mg of vecuronium. Peripherally inserted central venous line was inserted via left basilic vein. Caudal epidural catheter was placed in the left lateral position and fixed at 7 cm (epidural space at 1.5 cm). 0.125% bupivacaine with 1 mg of morphine in 12 ml of NS was administerd epiduarlly. Patient was positioned in the right lateral position for surgery. Anaesthesia was maintained on isoflurane in oxygen and air along with top ups of vecuronium and fentanyl. There were multiple episodes of desaturation upto 82% during manipulation of the lung but was restored to normal after releasing the manipulation and compression. The pulse rate was on higher side throughout the surgery (150-170 beats/min) inspite of adequate hydration and analgesia. The blood pressure ranged as systolic 90-100 mm Hg and diastolic 50-60 mm Hg intraoperatively. The EtCO 2 was on the higher side (48-58 mm Hg ) even on increasing the minute ventilation. The airway pressures were high (30-40 mm Hg). After the draining of pyopneumothorax, and left sided decortication, chest tube drains were put. The airway pressure and EtCO 2 settled down to 20-22 mm Hg and 40-44 mm Hg respectively after the surgical procedure. The total surgical duration was 135 minutes and blood loss was about 75 ml which was replaced with lactated Ringer solution. Fluid administration was guided by the CVP which was maintained at about 12-14 mm Hg, though there were some variations in the CVP value during surgical retraction during thoracotomy. Urine output was also monitored and was >2 mL/kg/hr (total urine output intraoperatively - 100 mL). The trachea was not extubated and child was shifted to the pediatric ICU and put on pressure control mode of ventilation. Epidural infusion with 0.125% bupivacaine with 3mcg/mL of fentanyl @ 2 mL/hour was initiated. Midazolam infusion was started @ 0.5 mg/hr. The child received nebulisation with 6% NaCl, salbutamol, ipratropium, and n-acetyl cysteine along with antibiotics. The child was weaned off to CPAP in next 8-10 hours and extubated after 36 hours when the CXR showed reinflation of the collapsed lung. Child had an uneventful recovery and was discharged on 12 th postoperative day with an advice to follow up in paediatrics clinics.
| Discussion|| |
Cystic fibrosis (CF) is characterized by a progressive loss of lung function (impaired mucociliary clearance in the airways leading to chronic bacterial colonization, Staphylococcus aureus and Pseudomonas aeruginosa, airway inflammation, and finally lung destruction), exocrine pancreatic insufficiency (obstruction of the pancreatic duct followed by pancreatic fibrosis leads to pancreatic insufficiency with malabsorption and maldigestion), liver disease (chronic inflammation and periportal fibrosis resulting in liver cirrhosis), intestinal motility disorder, male infertility, and high concentrations of sweat electrolytes , . The exocrine glands are dysfunctional, resulting in thick tenacious secretions , . Prevalence of diabetes mellitus is low in the first decade but impaired glucose tolerance with about 25% being insulin dependent develops by adulthood  .Airway related symptoms include sinusitis, nasal polyps, chronic bronchitis, pneumonia, bronchiectasis, lung fibrosis, emphysema, pneumothorax, haemoptysis, pulmonary hypertension and cor pulmonale  .
Sweat test for diagnosis of cystic fibrosis ( using Gibson Cook Method for pilocarpine Iontophoresis and titration method) have elevated sweat chloride concentration (>60mmol/L)
The therapeutic regimen includes antibiotic, physiotherapy, bronchial hygiene , mucolytic agents, bronchodilators, anti-inflammatory agents, nutritional support, oxygen supplementation, management of diabetes and other organ complications , .
Our patient had predominantly respiratory symptoms on clinical evaluation and we continued physiotherapy and airway secretions clearance techniques in the perioperative period to prevent mucus plugging and thus avoided further respiratory compromise ,, . Hypertonic saline, salbutamol, ipratropium, N-acetylcysteine nebulisation was given that increases ciliary transport, improves the rheological properties of the sputum, and improves the hydration of the surface of the airways  . Dextran exhibits antiadhesive properties in airway epithelial cells and acts as an antimicrobial agent in preventing the Pseudomonas infection.
Prevention of mucus plug formation and removing secretions by the above measures probably prevented in having another episode of pneumothorax in our child. Spontaneous pneumothorax due to increased transpulmonary pressure differences because of check valve phenomenon from mucus retention is reported in patients with CF  . We avoided nitrous oxide and used oxygen air mixture because of presence of preexisting pneumothorax in our patient.
Hemoptysis as a complication of lung disease resulting from erosion of abnormal bronchial vessels or coagulopathy secondary to vitamin K deficiency in such patients  . Conservative treatment such as bed rest, antibiotics, vitamin K, blood transfusion if necessary, and temporary discontinuation of physical therapy is sufficient to control the hemoptysis. Percutaneous bronchial artery embolization is effective method of controlling severe hemoptysis. Our child had a single episode of blood tinged sputum and was managed conservatively.
Surgical procedures required in patients with CF include nasal polypectomy, bronchoscopy, pleural stripping, lung transplantation, liver transplantation, laparotomy for bowel obstruction and enteral feeding procedures  . Short-acting anaesthetic agents are recommended because they allow rapid recovery. We used propofol and sevoflurane for induction of anaesthesia in our child. Ketamine is avoided because of its propensity to increase bronchial secretions and bronchorrhoea. Agents that irritate the respiratory tract, such as isoflurane or desflurane, should be avoided during the induction of anaesthesia. If nasal intubation is indicated, then the turbinate should be examined preoperatively for polyps, nevertheless, nasal intubation should usually be avoided because it may carry infections from the nasal cavity to the lungs  .Aminoglycoside antibiotics used to treat infections may prolong the neuromuscular blocking effect. Relaxants should be administered when necessary and only at minimum doses, and a nerve stimulator should routinely be used to guide administration and assess recovery of muscle function.
CT findings in our child were suggestive of chronic airway disease predominantly bronchitis type with hyperinflation, left upper lobe disease can be explained as partly focal collapse and partly bronchocele with dense impacted secretions and probable fungal colonization. In CF, severe airway obstruction and inflammatory bronchiectatic processes results in sputum retention, an increase in breathlessness, hyperinflation, ventilation perfusion mismatch, a decrease in respiratory muscle strength, and an inability to maintain arterial oxygenation. Nocturnal NIV with bilevel positive airway pressure can significantly improve nocturnal hypoventilation, chest symptoms, exertional dyspnoea, oxygenation and peak exercise capacity , . NIV has also been used clinically as an adjunct to airway clearance techniques  . CPAP therapy probably aided in mobilizing retained secretions and in preventing atelectasis after thoracic surgery in our child.
Postoperative pain relief is vital to allow mobilization and compliance with physiotherapy. Incisional pain resulting in hypoventilation and hypoxaemia is one of the immediate postoperative problems in patients who undergo thoracotomy and can be deleterious in such already compromised child. Regional analgesia techniques (epidural opioids, intercostal nerve blocks, local infiltration) are effective in preserving pulmonary function while adequately treating postoperative pain 3 . We provided analgesia with caudal epidural catheter using bupivacaine and fentanyl infusion in the postoperative period and probably helped us in effective physiotherapy and preventing stagnation of secretions and early reinflation of the collapsed lung.
Patients undergoing thoracotomy have a 28% incidence of acid gastroesophageal reflux, mandating aspiration prophylaxis  .
Intraoperatively, advanced haemodynamic monitoring may be indicated, depending on the patient's condition and the surgical procedure  . Patients with CF should be continuously monitored in intensive care unit postoperatively.
To conclude, patients with cystic fibrosis for emergency thoracotomy for pyopneumothorax and decortication requires vigilance in the perioperative period with proper choice of anaesthetic agents and techniques along with perioperative pain relief and chest physiotherapy.
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