Kyphoscoliosis

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Kyphoscoliosis

Anatomic Alterations of the Lungs

Kyphoscoliosis is a combination of two thoracic deformities that commonly appear together. Kyphosis is a posterior curvature of the spine (humpback). In scoliosis the spine is curved to one side, typically appearing as an S or C shape. Its appearance is most obvious in the anterior-posterior plane.

In severe kyphoscoliosis the deformity of the thorax compresses the lungs and restricts alveolar expansion, which in turn causes alveolar hypoventilation and atelectasis. In addition, the patient’s ability to cough and mobilize secretions also may be impaired, further causing atelectasis as secretions accumulate throughout the tracheobronchial tree. Because kyphoscoliosis involves both a posterior and a lateral curvature of the spine, the thoracic contents generally twist in such a way as to cause a mediastinal shift in the same direction as the lateral curvature of the spine. Severe kyphoscoliosis causes a chronic restrictive lung disorder that makes it more difficult to clear airway secretions. Figure 24-1 illustrates the lung and chest wall abnormalities in a typical case of kyphoscoliosis.

The major pathologic or structural changes of the lungs associated with kyphoscoliosis are as follows:

Etiology and Epidemiology

Kyphoscoliosis affects approximately 2% of the people in the United States—mostly young children who are going through a growing spurt. Kyphoscoliosis rarely develops in the adult—unless it is a worsening condition that started in childhood and was not diagnosed or treated. Kyphoscoliosis may also develop in adults from a degenerative joint condition in the spine. Although the precise cause of kyphoscoliosis is unknown, it is commonly associated with the following general conditions:

Other possible causes include hormonal imbalance, trauma, extraspinal contractures, infections involving the vertebrae, metabolic bone disorders (e.g., rickets, osteoporosis, osteogenesis imperfecta), joint disease, and tumors.

Depending on the child’s age at the time of onset, idiopathic scoliosis is classified as infantile, juvenile, or adolescent. In infantile scoliosis the curvature of the spine develops during the first 3 years of life. In juvenile scoliosis the curvature occurs at 4 years of age to the onset of adolescence. In adolescent scoliosis the spinal curvature develops after the age of 10. Adolescent scoliosis is the most common. Early signs (i.e., appearing when a child is approximately 8 years of age) of scoliosis include uneven shoulder height, prominent shoulder blade(s), uneven waist height, elevated hips, and leaning to one side. Risk factors include the following:

Diagnosis

Scoliosis is diagnosed by means of the patient’s medical history, physical examination, x-ray evaluation, and curve measurement. Clinically, scoliosis is commonly defined according to the following factors related to the curvature of the spine:

• Shape (nonstructural scoliosis and structural scoliosis)—A nonstructural scoliosis is a curve that develops side-to-side as a C– or S-shaped curve. This form of scoliosis results from a cause other than the spine itself (e.g., poor posture, leg length discrepancy, pain). A structural scoliosis is a curvature of the spine associated with vertebral rotation. A structural scoliosis involves the twisting of the spine and appears in three dimensions.

• Location—The curve of the spine may develop in the upper back area where the ribs are located (thoracic), the lower back area (lumbar), or in both areas (thoracolumbar).

• Direction—Scoliosis can bend the spine left or right.

• Angle—A normal spine viewed from the back is zero degrees—a straight line. Scoliosis is defined as a spinal curvature of greater than 10 degrees (i.e., bending toward the ground when in the upright position). The degree of the lateral curvature is expressed by the Cobb angle, which is calculated from a radiograph as shown in Figure 24-2.

image OVERVIEW of the Cardiopulmonary Clinical Manifestations Associated with Kyphoscoliosis

The following clinical manifestations result from the pathophysiologic mechanisms caused (or activated) by Atelectasis (see Figure 9-8) and Excessive Airway Secretions (see Figure 9-12)—the major anatomic alterations of the lungs associated with kyphoscoliosis (see Figure 24-1).

CLINICAL DATA OBTAINED AT THE PATIENT’S BEDSIDE

The Physical Examination

CLINICAL DATA OBTAINED FROM LABORATORY TESTS AND SPECIAL PROCEDURES

Pulmonary Function Test Findings Moderate to Severe Kyphoscoliosis (Restrictive Lung Pathology)

FORCED EXPIRATORY FLOW RATE FINDINGS

FVC FEVT FEV1/FVC ratio FEF25%-75%
N or ↓ N or ↑ N or ↓
FEF50% FEF200-1200 PEFR MVV
N or ↓ N or ↓ N or ↓ N or ↓

image

LUNG VOLUME AND CAPACITY FINDINGS

VT IRV ERV RV  
N or ↓  
VC IC FRC TLC RV/TLC ratio
N

image

General Management of Scoliosis

The treatment of scoliosis largely depends on the cause of the scoliosis, the size and location of the curve, and how much more growing the patient is expected to do. In most cases of scoliosis (less than 20 degrees), the degree of abnormal spine curvature is relatively small and requires only observation to ensure that the curve does not worsen. Observation is usually recommended in patients with a spine curvature of less than 20 degrees. In young children who are still growing, observation checkups are usually scheduled at 3- to 6-month intervals. When the curve is determined to be progressing to a more serious degree (more than 25 to 30 degrees in a child who is still growing), the following treatments options are available:

Braces

A brace device is usually recommended as the first line of defense for growing children who have a spinal curvature of 25 to 45 degrees. The mechanical objective of the brace is to hyperextend the spine and to limit forward flexion. It does not reverse the curve. Although a brace does not cure scoliosis (or even improve the condition), it has been shown to prevent the curve progression in more than 90% of patients who wear it. Bracing is not effective in congenital or neuromuscular scoliosis. The therapeutic effects of bracing are also less helpful in infantile and juvenile idiopathic scoliosis. Today a number of braces are available, including the Boston brace, Charleston bending brace, and Milwaukee brace (Figure 24-4). The type of brace is selected according to a the patient’s age, the specific characteristics of the curve, and the willingness of the patient to tolerate a specific brace.

The Boston brace (also called a thoracolumbosacral orthosis [TLSO], a low-profile brace, or an underarm brace) is composed of plastic that is custom-molded to fit the patient’s body. The Boston brace extends from below the breast to the top of the pelvic area in front, and from below the scapula to the coccyx in the back. The Boston brace is typically used for curves in the lumbar (low-back) or thoracolumbar sections of the spine. The Boston brace is worn about 23 hours a day but can be taken off to shower, swim, or engage in sports.

The Charleston bending brace (also known as a part-time brace) is worn only for 8 to 10 hours at night, when the human growth hormone (HGH) level is at its highest. The Charleston bending brace is molded to conform to the patient’s body when the patient bends toward the convexity—or outward bulge—of the curve. This brace works to overcorrect the curve while the patient is asleep. In order for the Charleston brace to be effective, the patient’s curve must be in the 20- to 40-degree range and the apex of the curve needs to be below the level of the scapula. The Charleston bending brace works on the principle that the spine should be bent to grow in the correct direction during the time of day that most growing occurs. Many studies have shown that the Charleston nighttime brace is as effective as the braces that need to be worn for 23 hours.

The Milwaukee brace (also known as a cervicothoracolumbosacral orthosis [CTLSO]) is used for high thoracic (mid-back) curves. The Milwaukee brace is a full-torso brace with a neck ring that serves as a rest for the chin and for the back of the head. It extends from the neck to the pelvis. It consists of a specially contoured plastic pelvic girdle and a neck ring that is connected by metal bars in the front and back of the brace. The metal bars work to extend the length of the torso, and the neck ring keeps the head centered over the pelvis. The Milwaukee brace is used less frequently now that more form-fitting plastic braces are available.

Surgery

In general, surgery is performed to correct unacceptable deformity and prevent further curvature. Surgery is usually recommended in patients who have curvatures of the spine greater than 40 to 50 degrees. As a general rule, even the best surgical techniques do not completely straighten the patient’s spine. Also, surgery often does not improve ventilatory function. Surgical procedures include the following.

Rod Instrumentation

Rod instrumentation involves the insertion of metal rods (e.g., Harrington rod), hooks, screws, and wires to prevent the curve from moving for 3 to 12 months and to allow the fusion to become solid (Figure 24-5). The system provides disruption to the concave side of the spine and compression to the convex side. This action enhances stabilization, reduces any rotational tendency, and applies force to the spine to correct the curvature. Up to 50% improvement of the curvature may occur in patients who elect to have this procedure.

Respiratory Care Treatment Protocols

Oxygen Therapy Protocol

Oxygen therapy is used to treat hypoxemia, decrease the work of breathing, and decrease myocardial work. The hypoxemia that develops in kyphoscoliosis is commonly caused by atelectasis and pulmonary shunting. Hypoxemia caused by capillary shunting is often refractory to oxygen therapy. In addition, when the patient demonstrates chronic ventilatory failure during the advanced stages of kyphoscoliosis, caution must be taken not to overoxygenate the patient (see Oxygen Therapy Protocol, Protocol 9-1).

CASE STUDY

Kyphoscoliosis

Admitting History

A 62-year-old woman began to develop kyphoscoliosis when she was 6 years old. She lived in the mountains of Virginia all her life, first with her parents and later with her two older sisters. Although she wore various types of body braces until she was 17 years old, her disorder was classified as severe by the time she was 15 years old. Her doctors, who were few and far between, always told her that she would have to learn to live with her condition the best she could, and as a general rule she did.

She finished high school with no other remarkable physical or personal problems. She was well liked by her classmates and was actively involved in the school newspaper and art club. After graduation she continued to live with her parents for a few more years. At 21 years of age, she moved in with her two older sisters, who were buying a large farmhouse near a small but popular tourist town. All three sisters made various arts and crafts, which they sold at local tourist shops. The woman’s physical disability and general health were relatively stable until she was about 40 years old. At that time, she started to experience frequent episodes of dyspnea, coughing, and sputum production. As the years progressed, her baseline condition was marked by increasingly severe dyspnea.

Because the sisters rarely ventured into the city, the woman’s medical resources were poor until she was introduced to a social worker at a nearby church. The church had just become part of an outreach program based in a large city nearby. The social worker was charmed by the woman and fascinated by the beauty of the colorful quilts she made.

The social worker, however, also was concerned by the woman’s limited ability to move because of her severe chest deformity. In addition, the social worker thought that the woman’s cough sounded serious. She noted that the woman appeared grayish-blue, weak, and ill. The sisters told the social worker that their sibling had had a bad “cold” for about 6 months. After much urging, the social worker persuaded the woman to travel, accompanied by her sisters, to the city to see a doctor at a large hospital associated with the church outreach program. The woman was immediately admitted to the hospital. The sisters stayed in a nearby hotel room provided by the hospital.

Physical Examination

Although the patient appeared to be well nourished, the lateral curvature of her spine was twisted significantly to the left. She also demonstrated anterior bending of the thoracic spine. She appeared older than her stated age, and she was in obvious respiratory distress. The patient stated that she was having trouble breathing. Her skin was cyanotic. She had digital clubbing, and her neck veins were distended, especially on the right side. The woman demonstrated a frequent but adequate cough. During each coughing episode she expectorated a moderate amount of thick, yellow sputum.

When the patient generated a strong cough, a large unilateral bulge appeared at the right anterolateral base of her neck, directly posterior to the clavicle. The patient referred to the bulge as her “Dizzy Gillespie pouch.” The doctor thought that the bulge was a result of the severe kyphoscoliosis, which had in turn stretched and weakened the suprapleural membrane that normally restricts and contains the parietal pleura at the apex of the lung. Because of the weakening of the suprapleural membrane, any time the woman performed Valsalva’s maneuver for any reason (e.g., for coughing), the increased intrapleural pressure herniated the suprapleural membrane outward. Despite the odd appearance of the bulge, the doctor did not consider it a serious concern.

The patient’s vital signs were as follows: blood pressure 160/100, heart rate 90 bpm, respiratory rate 18/min, and oral temperature 36.3° C (97.4° F). Palpation revealed a trachea deviated to the right. Dull percussion notes were produced over both lungs; crackles and rhonchi were heard over them as well. There was 2+ pitting edema below both knees. A pulmonary function test (PFT) conducted that morning showed vital capacity (VC), functional residual capacity (FRC), and residual volume (RV) of 45% to 50% of predicted values.

Although the patient’s electrolyte levels were all normal, her hematocrit was 58%, and her hemoglobin level was 18 g%. A chest x-ray examination revealed a severe thoracic and spinal deformity, a mediastinal shift, an enlarged heart with prominent pulmonary artery segments bilaterally, and bilateral infiltrates in the lung bases consistent with pneumonia and atelectasis. The patient’s arterial blood gas values (ABGs) on room air were as follows: pH 7.52, Paco2 58, image 46, and Pao2 49. Her oxygen saturation measured by pulse oximetry (Spo2) was 88%. The physician requested a respiratory care consultation and stated that mechanical ventilation was not an option at this time per the patient’s request and his knowledge of the case. On the basis of these clinical data, the following SOAP was documented.

Respiratory Assessment and Plan

S “I’m having trouble breathing.”

O Well-nourished appearance; severe anterior and left lateral curvature of the spine; cyanosis, digital clubbing, and distended neck veins—especially on the right side; cough: frequent, adequate, and productive of moderate amounts of thick yellow sputum; 2+ pitting edema below both knees; vital signs: BP 160/100, HR 90, RR 18, T 36.3° C (97.4° F); trachea deviated to the right; both lungs: dull percussion notes, crackles, and rhonchi; PFT: VC, FRC, and RV 45% to 50% of predicted; Hct 58%, Hb 18 g%; CXR: severe thoracic and spinous deformity, mediastinal shift, cardiomegaly, and bilateral infiltrates in the lung bases consistent with pneumonia or atelectasis; ABGs (room air): pH 7.52, Paco2 58, image 46, Pao2 49; Spo2 88%

P Initiate Oxygen Therapy Protocol (HAFOE at Fio2 0.28; be careful not to overoxygenate the patient). Bronchopulmonary Hygiene Therapy Protocol (obtain sputum for culture; C&DB instructions and oral suction prn). Lung Expansion Therapy Protocol (incentive spirometry qid and prn). Aerosolized Medication Protocol (aerosolized Xopenex 0.5 mL in 1.5 mL 10% acetylcysteine q4h). Notify physician of admitting ABGs and impending ventilatory failure. Place mechanical ventilator on standby. Monitor closely.

10 Hours after Admission

The patient’s condition had not improved, and she was transferred to an intensive care unit. The physician had trouble titrating the cardiac drugs and decided to insert a pulmonary artery catheter, a central venous catheter, and an arterial line. Because of the woman’s cardiac problems, several medical students, respiratory therapists, nurses, and doctors were constantly in and out of her room, performing and assisting in various procedures. As a result, working with the patient for any length of time was difficult, and the intensity of respiratory care was less than desirable. Eventually, the patient’s cardiac status stabilized, and the physician requested an update on the woman’s pulmonary condition.

The respiratory therapist working on the pulmonary consultation team found the patient in extreme respiratory distress. She was sitting up in bed, appeared frightened, and stated that she was extremely short of breath. Both of her sisters were in the room; one sister was putting cold towels on the patient’s face while the other sister was holding the patient’s hands. Both sisters were crying softly. The woman’s skin appeared cyanotic, and perspiration was visible on her face. Her neck veins were still distended. She demonstrated a weak, spontaneous cough. Although no sputum was noted, she sounded congested when she coughed. Dull percussion notes, crackles, and rhonchi were still present throughout both lungs. Her vital signs were as follows: blood pressure 180/120, heart rate 130 bpm, respiratory rate 26/min, and rectal temperature 37.8° C (100° F).

Several of the patient’s hemodynamic indices were elevated: CVP, RAP, PA, RVSWI, and PVR.* Her oxygenation indices were as follows: increased image and O2ER and decreased Do2 and image. Her image and image were normal. No recent chest x-ray film was available. Her ABGs on an Fio2 of 0.28 were as follows: pH 7.57, Paco2 49, image 44, and Pao2 43. Her Spo2 was 87%. On the basis of these clinical data, the following SOAP was documented.

Respiratory Assessment and Plan

S Severe dyspnea; “I’m extremely short of breath.”

O Extreme respiratory distress; cyanosis and perspiration; distended neck veins; weak, spontaneous cough; sounds of congestion but no sputum produced; bilateral dull percussion notes, crackles, and rhonchi; vital signs: BP 180/120, HR 130, RR 26, T 37.8° C (100° F); hemodynamics: increased CVP, RAP, PA, RVSWI, and PVR; oxygenation indices: increased image, and O2ER and decreased Do2 and image; image and image normal; ABGs; pH 7.57, Paco2 49, image 44, Pao2 43; Spo2 87%

A 

P Up-regulate Oxygen Therapy Protocol (HAFOE at 0.35). Up-regulate Bronchopulmonary Hygiene Therapy Protocol (add CPT and PD qid). Up-regulate Aerosolized Medication Protocol (increase med. nebs. to q2h). Contact physician regarding possible ventilatory failure. Discuss therapeutic bronchoscopy with physician. Continue to keep mechanical ventilator on standby. Monitor and reevaluate in 30 minutes.

24 Hours after Admission

At this time the respiratory care practitioner found the patient watching the morning news on television with her two sisters. The woman was situated in a semi-Fowler’s position eating the last few bites of her breakfast. The patient stated that she felt “so much better” and that “finally I have enough wind to eat some food.”

Although her skin still appeared cyanotic, she did not look as ill as she had the day before. On request, she produced a strong cough and expectorated a small amount of white sputum. Her vital signs were as follows: blood pressure 140/85, heart rate 83 bpm, respiratory rate 14/min, and temperature normal. Chest assessment findings demonstrated crackles, rhonchi, and dull percussion notes over both lung fields. The rhonchi were less intense, however, than they had been the day before.

Although the patient’s hemodynamic and oxygenation indices were better than they had been the day before, she still had room for improvement. Her hemodynamic parameters, still abnormal, revealed an elevated CVP, RAP, PA, RVSWI, and PVR. All other hemodynamic indices were normal. Her oxygenation indices still showed an increased image and O2ER and a decreased Do2 and image. Her image and image were normal. The patient’s chest x-ray film, taken earlier that morning, showed some clearing of the pneumonia and atelectasis described on admission. Her ABGs on an Fio2 of 0.35 were as follows: pH 7.45, Paco2 73, image 49, and Pao2 68. Her Spo2 was 94%. On the basis of these clinical data, the following SOAP was recorded.

Respiratory Assessment and Plan

S “I feel so much better. I finally have enough wind to eat some food.”

O Cyanotic appearance; cough: strong, small amount of white sputum; vital signs: BP 140/85, HR 83, RR 14, T normal; crackles, rhonchi, and dull percussion notes over both lung fields; rhonchi improving; hemodynamic and oxygenation indices improving, but still an elevated CVP, RAP, PA, RVSWI, and PVR and still an increased image and O2ER and a decreased Do2 and image; CXR: improvement of the bilateral pneumonia and atelectasis; ABGs: pH 7.45, Paco2 73, image 49, Pao2 68; Spo2 94%

A 

P Down-regulate Oxygen Therapy Protocol, Bronchopulmonary Hygiene Therapy Protocol, and Aerosolized Medication Protocols. Continue to monitor and reevaluate (ABGs on reduced Fio2). Recommend pulmonary rehabilitation and patient and family education (cuirass respiratory ventilation, possibly rocking bed, BIPAP, or positive expiratory pressure [PEP]).

Discussion

This case provides an excessive amount of extraneous historical and personal material. This was done to demonstrate, in part, how the respiratory care worker must cut through to the core of the case in the SOAP notes. Care of the patient with symptomatic advanced kyphoscoliosis consists of (1) treatment of the conditions that can complicate it (e.g., bronchitis, pneumonia, atelectasis, pleural effusion) and (2) treatment of the underlying condition itself.

In the first assessment, the SOAP documented excessive bronchial secretions and a likely infection because of the thick yellow sputum and recent history. The patient had a good ability to mobilize the secretions as charted by a strong cough. The chest radiograph confirmed atelectasis and consolidation. Although acute alveolar hyperventilation on top of chronic ventilatory failure was present, the possibility of impending ventilatory failure was real. The therapist’s decision to oxygenate the patient with a low Fio2 (0.28), administer bronchial hygiene and mucolytic aerosols, and be prepared for ventilator support were all appropriate. The patient’s secondary polycythemia and cor pulmonale would have been expected to improve as overall oxygenation improved, although this improvement could take some time. The digital clubbing and cor pulmonale itself suggested that the hypoxemia was long-standing.

At the time of the second assessment, the intensity of the patient’s respiratory distress was increasing. This was verified by the continued observation of the high pulse and respiratory rate, excessive bronchial secretions, dull percussion notes, acute alveolar hyperventilation on top of chronic ventilatory failure with moderate to severe hypoxemia, atelectasis on the chest x-ray film, and poor response to oxygen therapy. Undoubtedly, impending ventilatory failure was more likely. Atelectasis is often refractory to oxygen therapy, suggesting that therapeutic bronchoscopy might have been worthwhile. At that point in time, the up-regulation of the Oxygen Therapy Protocol (Protocol 9-1), Bronchopulmonary Hygiene Therapy Protocol (Protocol 9-2), and Aerosolized Medication Protocol (Protocol 9-4) were all justified by the clinical indicators.

In the last assessment, the clinical manifestations associated with the patient’s disorder had all decreased substantially. The down-regulation of the Oxygen Therapy Protocol, Bronchopulmonary Hygiene Therapy Protocol, and Aerosolized Medication Protocol was appropriate. The recommendation of pulmonary rehabilitation and family education was appropriately considered. The ABGs were most likely at the patient’s baseline level, because the pH was in the normal range. In fact, according to the pH (normal but on the alkalotic side of normal) the patient’s usual Paco2 was most likely somewhat higher than the last assessment value.

Comparison with baseline values (if available) would be appropriate at such a time, and consideration of cuirass ventilation, a rocking bed, or positive expiratory pressure (PEP) to assist nocturnal ventilation might be in order. Oxygenation easily can be assessed by oximetry at home. This case is an excellent example of the value of hemodynamic monitoring (specifically the normal PCWP) in differentiating left-sided from right-sided cardiac failure.

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