Preventing postoperative complications in the adult cystic fibrosis patient

International Elsevier

PEDOT

Journal of Pediatric

Otorhinolaryngology,

18 (1990) 263-269

263

00618

Preventing postoperative complications in the adult cystic fibrosis patient Randolph

R. Cole

1 and

Robin

T. Cotton

*

’ Department of OtoLaryngotogV - Head and Neck Surgery, The Umversrty of Texas Health Science Center at Houston, Houston, TX (U.S.A.) and ’ Department of Otolatyngologv and Maxillofacral Surgery, Division oJ Pediatrrc Otolatyngologv, University of Cincinnati Medical Center, Cincinnati, OH (U.S.A ) (Received 31 May 1989) (Accepted 6 August 1989)

Key words: Cystic

fibrosis;

Anesthesia;

Complication

Abstract Herein we report on a 27-year-old cystic fibrosis (CF) patient who developed bronchospasm, secretory plugging of the trachea, and pneumothorax following general endotracheal anesthesia for intranasal polypectomy and bilateral Caldwell-Luc procedures at an adult facility. Intranasal polypectomy and paranasal sinus procedures are the most common surgical procedures performed on cystic fibrosis patients, making the otolaryngologist a frequent member of the cystic fibrosis team [8]. As survival improves, the pediatric otolaryngologist will find himself following CF patients into their 20’s and 30’s. Adult care facilities may not be as familiar with these patients as the surgeon would like. The otolaryngologist’s familiarity with the unique perioperative requirements of the CF patient can prove invaluable in such a setting. Preoperative assessment should identify any acute pulmonary changes, assess nutritional status. assure good control of blood glucose levels, and rule out clotting abnormalities. Good perioperative hydration and meticulous attention to pulmonary toilet are of foremost importance in the surgical care of the cystic fibrosis patient. Chest physiotherapy and suctioning of the tracheobronchial tree should precede arousal from anesthesia and extubation. By insisting on appropriate anesthetic and perioperative care, the knowledgeable otolaryngologist may circumvent potential postoperative complications in the cystic fibrosis patient.

Presented at the Annual Scientific Session of The American Society of Pediatric Otolaryngology, Kiawah Island, SC, April 20-24, 1988. Correspondence: RR Cole. Department of Otolaryngology-Head and Neck Surgery. The Universtty of Texas Medical School, P.O. Box 20708, Houston, TX 77225, U.S.A.

0165-5876/90/$03.50

0 1990 Elsevier Science

Publishers

B.V.

264

Introduction Cystic fibrosis (CF) is the most common fatal hereditary disease of Caucasians. Inherited as an autosomal recessive trait with variable penetrance, CF occurs with a frequency of 1 in 2000 live births [7]. The cystic fibrosis gene is thought to reside on the long arm of chromosome 7 [ll]. Exocrine and eccrine gland dysfunction stems from abnormal transmembrane ion and water transport. Thick secretions lead to chronic progressive lung disease, intestinal obstruction, hepatobiliary and pancreatic dysfunction, sterility and diagnostically important increase in sweat chloride. Other sequelae of this disease include an extremely high incidence of chronic and recurrent sinusitis and a greater than 10% incidence of nasal polyposis [1,2,5]. Today, 50% of CF patients live beyond 27 and survival continues to improve (Fig. 1) [3,12]. It is increasingly likely that otolatyngologists will be treating CF patients in adult facilities. Care outside a specialized pediatric center could involve the utilization of personnel without special interest or training in the care of the CF patient. In this setting, the risk of complications is increased.

Case report A 27-year-old CF patient was scheduled for left intranasal polypectomy and bilateral Caldwell-Luc procedures at an adult hospital. A chest X-ray taken prior to surgery showed the typical features of mild CF: chronic focal left lower lobe atelectasis and scarring, and bilateral apical cystic changes. No acute changes were noted (Fig. 2). Complete blood count, electrolyte and coagulation profiles, serum protein levels, and blood glucose were normal. Preoperative benzodiazepine was given. Induction utilizing atropine, thiopental sodium and muscle relaxants was uneventful. The 1.5 h surgical procedure was similarly uneventful. Total blood loss approached 150 ml. Upon extubation, the patient developed bronchospasm, experi-

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YEAR

Fig. 1. Mean survival of CF patients [3,12].

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Fig. 2. Preoperative

chest X-ray showing features typical of mild CF. Note the chronic lobe atelectasis and scarring, and bilateral apical cystic changes.

focal left lower

enced oxygen desaturation, and could not be adequately ventilated using a mask. The patient was immediately reintubated. Repeated endotracheal suctioning with irrigation and chest percussion resulted in the return of copious amounts of very thick, blood tinged, tenacious, creamy white secretions. A chest X-ray obtained on the operating room table showed marked infiltration of the right lung fields with a slight right-sided volume loss and associated atelectasis (Fig. 3). The patient was transported to the intensive care unit where she remained intubated and where aggressive pulmonary toilet was continued. Antibiotics and steroids were initiated. Pseudomonas eventually grew from sputum cultures. Repeat chest X-ray shortly after arrival in the ICU revealed the presence of a right apical 20% pneumothorax (Fig. 4). Chest tube placement reduced the size of the pneumothorax, but an air leak continued for several days. Because of the persistent air leak and a history of previous spontaneous pneumothorax on the same side the patient underwent right

Fig.

3. A chest

X-ray

taken in the OR demonstrates marked right-sided volume loss and associated

thoracotomy and pleural one week later.

abrasion

5 days after initial

infiltration atelectasis.

surgery.

of right

lung

fields

with

She was discharged

Discussion Cystic fibrosis is a multidisciplinary disease. The non-pulmonary aspects of CF, like the pulmonary aspect, are highly variable. Deficiencies of pancreatic enzymes and bile can lead to malabsorption of proteins and fats. CF patients, therefore, often have steatorrhea and typically consume large quantities of food in an effort to obtain an adequate number of calories. Many require replacement enzymes with meals. CF patients poorly absorb vitamins A, E, and K and may need to take water-soluble preparations of these vitamins with meals. The empiric administration of preoperative parenteral vitamin K is therefore an acceptable practice in CF patients and should be considered. In addition to deficiencies in serum proteins and vitamin K-related clotting factors, hepatobiliary involvement can, in extreme cases,

Fig. 4. An early chest X-ray in the ICU reveals a right apical pneumothorax

lead to portal hypertension, hypersplenism, and esophageal varicies. Diabetes is also common in CF patients, occurring with a frequency of 7-15s [7,8]. Hyperglycemia in the CF patient is almost always responsive to insulin therapy. Communication between the surgeon and the patient’s primary care physician will give the surgeon an idea of the severity of the patient’s disease and will help determine whether preoperative hospitalization is indicated. Consultations with a pulmonologist, gastroenterologist, and/or endocrine specialists may be needed. A preoperative visit to the anesthesiologist should always be obtained to alert the anesthesiologist that a CF patient is scheduled for surgery and give him the opportunity to counsel the patient [4,6,10]. The majority of postoperative complications in CF patients are pulmonary [8,9]. Special attention should be directed toward assessing and optimizing their respiratory status. Progressive deterioration of pulmonary function stems from chronic and recurrent obstruction of segmental and subsegmental bronchi and bronchioles by thick secretions. Stagnation leads to inflammation, infection, bronchiectasis, and fibrosis. Obstructive emphysema, ventilation-perfusion mismatches, mucosal metaplasia and loss of the mucociliary blanket result. An increasing alveolar-

268

arteriolar oxygen gradient and a rising arterial CO, level accompany these changes. Cor pulmonale with liver congestion is usually present at death in CF patients. A preoperative chest X-ray is mandatory both to evaluate chronic pulmonary changes and to rule out any acute disease. Electrocardiogram, pulmonary function tests, and arterial blood gases can assist in the preoperative assessment of the patient’s cardiopulmonary status and should be selectively utilized. The use of chest physiotherapy (CPT) and postural drainage, with or without bronchodilators, is already a daily routine in many of these patients. The importance of these procedures should be emphasized. Patients not on a home regime of CPT should initiate such a program prior to surgery. Preoperative hospitalization is appropriate in many cases to ensure intensive pulmonary toilet. Good perioperative hydration is mandatory and will help to keep secretions as thin as physiologically possible. Patients should be encouraged to drink clear liquids up to 4 h prior to surgery. Alternatively, intravenous hydration should be initiated in patients who have been hospitalized preoperatively. Low-grade pulmonary infections from Staph. aureus, Pseudomonas, Hemophilus influenzae, Proteus, E. coli, and Klebsiella are common in CF patients. The use of perioperative prophylactic antibiotics, directed at these pathogens, should therefore be considered. The use of atropine during induction, once a controversial subject, is now generally felt to be safe and beneficial. The cardiac protection provided by this drug outweighs the risk of thickened secretions, especially in the properly hydrated patient. A prolonged period may be needed to achieve the desired anesthetic level if inhalation agents are used due to ventilation-perfusion mismatching. Similarly, it will take longer for the patient to awaken. The closed circuitry of the anesthesia machine is designed to spare humidity. In patients with severe pulmonary disease, an in-line humidifier can be incorporated into the anesthetic circuit to ensure hydration of inspired gases. Emphysematous bullae are at risk for rupture if the patient is aggressively ventilated. At the end of the procedure, but before the patient is awakened, it is very important to thoroughly clean the tracheobronchial tree. The surgeon must insist on this. Knowledgeable anesthesiologists will usually incorporate chest percussion and endotracheal irrigation into their pre-extubation suctioning routine for CF patients. Postoperatively, the patient’s inspired air should be humidified. CPT with postural drainage should be continued throughout hospitalization. Nutritional needs must be reassessed and met, and proper hydration maintained. All aspects of this multiple organ system disease take on heightened importance during the recovery and healing stages that follow surgery. Do not hesitate to call in consultants if needed. In the case presented, complications arose which required vigorous ventilation and aggressive pulmonary toilet. This resulted in the rupture of a bulla and subsequent pneumothorax. Whether closer attention to the patient’s preoperative pulmonary status and pre-extubation pulmonary toilet would have avoided these complications, is speculative. At Children’s Hospital Medical Center, Cincinnati, we treat many CF patients. In this setting, pediatric cystic fibrosis specialists and anesthesiologists knowledgeable in the disease automatically become involved in the

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patient’s care. Complications are extremely rare. This experience may have lulled us into a false sense of security with our patient who was treated at an adult facility. We are writing this report to heighten awareness of the special problems involved in dealing with the CF patient, and to encourage the surgeon to become knowledgeable in the perioperative care of these patients. Such awareness and knowledge may prove invaluable when care is provided in a non-specialized center.

References 1 Cepero, R., Smith, R.J., Catlin, FL, Bressler, K.L., Furuta, G.R. and Shandera, K.C., Cystic Fibrosis - an otolaryngologic perspective, Otolaryngol. Head Neck Surg., 97 (1987) 356-360. 2 Crockett, D.M., McGill, T.J., Healy, G.B., Friedman, E.M. and Salkeld, L.J., Nasal and paranasal sinus surgery in children with cystic fibrosis, Ann. Otol. Rhinol. Laryngol., 96 (1987) 367-372. 3 Cystic Fibrosis Foundation, Patient Registry Analyses, Bethesda, MD, 1986. 4 Doershuk, C.F., Reyes, A.L., Regan, A.G. and Matthews, L.W., Anesthesia and surgery in cystic fibrosis, Anesth. Analg., 51 (1972) 413-421. 5 Jaffe, B.F., Strome, M., Khaw, K. and Shwachman, H., Nasal polypectomy and sinus surgery for cystic fibrosis - A IO-year review, Oto Clin. N. Am., 10 (1977) 81-90. 6 Lamberty, J.M. and Rubin, B.K., The management of anesthesia for patients with cystic fibrosis, Anesthesia 40 (1985) 448-459. 7 Matthews, L.W. and Drotar, D., Cystic fibrosis - a challenging long-term chronic disease, Pediatr. Clin. N. Am., 31 (1984) 133-151. 8 Olsen, M.M., Gauderer, W.L., Gin, M.K. and Izant, R.J., Surgery in patients with cystic fibrosis, J. Pediatr. Surg., 22 (1987) 613-618. 9 Reilly, B.J., Featherby, E.A. and Weng, T.R., The correlation of radiological changes with pulmonary function in cystic fibrosis, Radiology, 98 (1971) 218-285. 10 Smith, R.M., Anesthetic management of patients with cystic fibrosis, Anesth. Analg., 44 (1965) 143-146. 11 Wheeler, W.B. and Colten, H.R., Cystic fibrosis: current approach to diagnosis and management. Pediatr. Rev. 9 (1988) 241-248. 12 Wilmott, R.W., Tyson, S.L., Dinwiddie, R. and Matthew, D.J., Survival rates in cystic fibrosis. Arch. Dis. Child., 58 (1983) 835-836.