116: Tietze Syndrome

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CHAPTER 116

Tietze Syndrome

Marta Imamura, MD; Satiko T. Imamura, MD

Synonyms

Parasternal chondrodynia

Costochondral junction syndrome

Thoracochondralgia

Chondropathia tuberosa

Costal chondritis

ICD-9 Code

733.6  Tietze’s disease; costochondral junction syndrome and costochondritis

ICD-10 Code

M94.0  Chondrocostal junction syndrome (Tietze)

Definition

Tietze syndrome is a benign, self-limited, nonsuppurative localized painful swelling of the upper costal cartilages of unknown etiology [110]. It affects the costochondral, costosternal, or sternoclavicular joints [2,57,9]. The manubriosternal and xiphisternal joints are less frequently affected [5,10]. First described in 1921 by a German surgeon in Breslau, Alexander Tietze, it is different from the costosternal syndrome [512]. Tietze syndrome is a rare cause of benign anterior chest wall pain associated with local swelling of the involved costal cartilages (Fig. 116.1) [5,6,12]. It is typically described in young adults and is a disease of the second and third decades of life [10,12]. Although it is not common, Tietze syndrome may also appear in children, infants [10,13], and elderly people [14]. It affects both men and women in a 1:1 ratio [46,9,10,15]. Lesions are unilateral and single in more than 80% of patients [4,10], and the second and third costal cartilages are most commonly involved [1,4,6,912]. Costosternal syndrome, a frequent cause of benign anterior chest wall pain, is not associated with a local swelling of the involved costal cartilages [512]. Costosternal syndrome usually occurs during and after the fourth decade of life, more frequently in women in a rate of 2 to 3:1 [9,10]. Multiple costal cartilages are involved in 90% of patients with costosternal syndrome [5,9,10].

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FIGURE 116.1 Schematic representation of the area of Tietze syndrome (costal cartilage, costosternal and costochondral joints) and anatomic relations with the mediastinal structures and other anterior chest wall structures.

The pathogenesis of Tietze syndrome is unknown [14,6, 810]. Recurrent functional overloading or microtrauma to the costal cartilages from severe coughing, heavy manual work, and sudden movement of the rib cage as well as malnutrition, sprain of the intra-articular sternocostal ligament, and respiratory tract infections may influence the development of Tietze syndrome [2,5,6,810,12,15]. Costal swelling may be due to focal enlargement [4,7], ventral angulation or irregular calcification of the affected costal cartilage [4,16], and thickening of overlying muscle [16,17]. Tietze syndrome may mimic a variety of life-threatening clinical entities [4,6,15] and must be considered in the differential diagnosis of any painful mass in the peristernal area. Clinical awareness of this syndrome and of its benign course may minimize performance of invasive diagnostic procedures [13].

Symptoms

Clinical manifestations include the sudden or gradual onset of pain of variable intensity [3,4,10,15] in the upper anterior chest wall in association with a fusiform and tender swelling of the involved costal cartilage [4,15]. Despite descriptions that pain may radiate to the shoulder, arm, and neck [3,4,12], its distribution usually occurs within the segment innervated by the afferent fibers carrying the painful impulse [2]. It is often aggravated by motion of the thoracic wall, sneezing, coughing, deep breathing, bending, exertion [18,15], and lying prone or over the affected side [10]. Some patients report inability to find a comfortable position in bed and have pain on turning over in the bed [1]. Weather change, anxiety, worry, and fatigue may exacerbate the pain [4]. Symptoms are usually unilateral, with no preferential side [3]. There is no reported association with sternotomy.

Physical Examination

On physical examination, a slight firm swelling is noted at the involved site [14,15]. Systemic manifestations [4,6,10,13,15] and inflammation are usually absent [1,4,5,7,10,15], but there may be local heat [15]. Pain is reproduced with active protraction or retraction of the shoulder, deep inspiration, and elevation of the arm [12] (Fig. 116.2). A unique visible, spherical, nonsuppurative, tender tumor of elastic-hard and pasty consistency can be palpated, usually over the second and third costochondral joints. Local palpation with firm pressure over the localized tender swelling reproduces the spontaneous pain complaint [12] (Fig. 116.3). Physical examination findings of the musculoskeletal and neurologic systems of reported cases from the literature are usually normal except for the local findings [46,13,15,17]. Muscle strength and upper limb range of motion may be decreased because of pain. Dermatomal and subcutaneous hyperalgesia (Fig. 116.4) and hyperemia (Fig. 116.5) may be present in the involved thoracic spinal segments. The adjacent intercostal [12], sternal, and pectoralis major and minor muscles may be tender to palpation [14].

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FIGURE 116.2 Reproduction of the spontaneous pain complaint during arm elevation in a patient diagnosed with Tietze syndrome.
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FIGURE 116.3 Local palpation with firm pressure over the localized tender swelling reproduces the spontaneous pain complaint.
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FIGURE 116.4 Subcutaneous hyperalgesia during the pinch and roll maneuver at the thoracic level.
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FIGURE 116.5 Hyperemia localized at the involved thoracic spinal segments after the pinch and roll maneuver.

Functional Limitations

The disability produced by Tietze syndrome is usually minor, although it can be severe with activity restriction involving the trunk and upper limbs. Activities such as lifting, bathing, ironing, combing and brushing hair, and other activities of daily living can be problematic. Patients who have physically vigorous jobs may need to be put on light duty for weeks and avoid physical efforts of the upper limbs and trunk [1]. Functional limitations may also be due to chronic pain [18]; however, even of those patients who continue to have pain after 1 year, most lead a life without disability.

Diagnostic Studies

Diagnosis of Tietze syndrome is essentially made on a clinical basis: anterior chest wall pain confirmed by palpation of a tender swelling at the second or third costochondral junction that reproduces the patient’s complaint in the absence of another definite diagnosis [35,79,11,15]. Results of laboratory analysis including inflammatory and immunologic parameters are usually normal [49,15]. Some cases may show a slight increase in the erythrocyte sedimentation rate [7,13,15].

Chest, rib, and sternum plain films and conventional tomograms of the costochondral junction are generally normal [6,17,19

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