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Year : 2008  |  Volume : 52  |  Issue : 6  |  Page : 866 Table of Contents     

Child With Cystic Fibrosis And Pyopneumothorax For Emergency Thoracotomy For Decortication

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 Acceptance25-Aug-2008
Date of Web Publication19-Mar-2010

Correspondence Address:
Rakesh Garg
Department of Anaesthesiology and Intensive Care, All India Institute of Medical Sciences, Ansari Nagar, NewDelhi-110029
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Source of Support: None, Conflict of Interest: None

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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 2021 Mar 4];52:866. Available from: https://www.ijaweb.org/text.asp?2008/52/6/866/60704

   Introduction Top

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 [1],[2],[3],[4] . 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 [1],[3] . We describe a child of cystic fibrosis for emergency thoracotomy for pyopneumothorax and decortication.

   Case report Top

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 Top

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 Scientific Name Search  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 [2],[4] . The exocrine glands are dys­functional, resulting in thick tenacious secretions [2],[4] . Prevalence of diabetes mellitus is low in the first decade but impaired glucose tolerance with about 25% being insulin dependent develops by adulthood [2] .Airway re­lated symptoms include sinusitis, nasal polyps, chronic bronchitis, pneumonia, bronchiectasis, lung fibrosis, em­physema, pneumothorax, haemoptysis, pulmonary hy­pertension and cor pulmonale [2] .

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 [2],[4] .

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 [5],[6],[7] . 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 [4] . 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 [8] . 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 [8] . 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 [1] . 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 [1] .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 [5],[8] . NIV has also been used clinically as an adjunct to airway clear­ance techniques [7] . 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 [9] .

Intraoperatively, advanced haemodynamic moni­toring may be indicated, depending on the patient's condition and the surgical procedure [1] . 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.

   References Top

1.Rocca GD. Anaesthesia in patients with cystic fibrosis. Curr Opin anaesthesiol 2002;15:95-101.  Back to cited text no. 1      
2.Staab D. Cystic fibrosis -therapeutic challenge in cys­tic fibrosis children. Eurpoean Journal of Endocrinol­ogy 2004;151:77-80.  Back to cited text no. 2      
3.Cameron AJD, Skinner TAJ. Management of a parturi­ent with respiarory failure secondary to cystic fibrosis. Anaesthesia 2005;60:77-80.  Back to cited text no. 3      
4.Dalcin PTR, Silva FAA. Cytic fibrosis in adults and thera­peutic aspects. J Bras Pneumol 2008;34:107-117.  Back to cited text no. 4      
5.Young ACY, Kotsimbos TC, Naughton MT. Randomised placebo controlled trial of non invasive ventilation for hypercapnia in cystic fibrosis. Thorax 2008;63:72-77.  Back to cited text no. 5      
6.Tannenbaum E, Prasad SA, Dinwiddie R, Main E. Chest physiotherapy during anaesthesia for children with cys­tic fibrosis: effects on respiratory function. Pediatr Pulmonol 2007; 42:1152-8.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
7.Holland AE, Denehy L, Ntoumenopoulos G, Naughton MT, Wilson JW. Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax 2003;58:880-84.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.Hulsey and Leslie L. Clark Patrick A. Flume, Charlie Strange, Xiaobu Ye, Myla Ebeling, Thomas. Pneumotho­rax in cystic fibrosis.Chest 2005;128;720-8.  Back to cited text no. 8      
9.Agnew NM, Kendall JB, Akrofi M, et al. Gastroesoph­ageal reflux and tracheal aspiration in the thoracotomy position: should ranitidine premedication be routine? AnesthAnalg 2002;95:1645-9.  Back to cited text no. 9      


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