Deformities of the chest wall are not unusual in children and infants. Localized abnormalities of the ribs are the most common deformity; more extensive bony abnormalities such as pectus excavatum and pectus carinatum are often evident at birth and present few diagnostic problems at their first evaluation. Tumours of the chest wall may be benign or malignant, although the latter are rare.

Superficial lesions such as haemangiomas and lymphangiomas can cause concern when first seen and are extensive at times, as in the Klippel–Trenaunay syndrome. The extent of these benign lesions may lead to a malignant course because of their local effects on the lungs or on movement of the chest wall. When they extend into the mediastinum or neck and compress the trachea they can be symptomatic at delivery or soon after. Endotracheal intubation and assisted ventilation should be performed until the extent of the lesion has been determined and a plan of excision devised.

Cystic hygromas confined to the thorax may present at any age. When they are symptomatic in children they are usually massive, associated with respiratory distress, fever, and, occasionally, with spontaneous chylothorax. These large cysts can apparently enlarge rapidly because of bleeding within the mass, causing presentation with acute respiratory symptoms. Complete excision of the cystic elements should be undertaken. Although tedious in the extreme, attempts should be made to preserve normal adjacent structures to minimize morbidity. All cysts should be drained, and the sites of lymphatic drainage should be closed to diminish the likelihood of a prolonged chylous leak. If a major lymphatic leak persists, reoperation is indicated if a trial of either intravenous hyperalimentation and bowel rest or elemental enteral feeding with medium-chain triglycerides fails. At the time of reoperation the thoracic duct should be ligated, and all areas of lymphatic leak should be closed. Remaining cysts that cannot be removed safely may be injected with sclerosing agents such as 50 per cent dextrose. Although cystic hygromas are benign lesions from a neoplastic point of view, many have a malignant course, especially if the lesion is extensive and is complicated by a high-volume lymphatic leak.

Hamartomas of the chest wall have been described under a number of different names (Table 1) 576. These lesions may be huge; they usually present at birth or in infancy as a large chest wall mass and are a histologically benign focal ‘overgrowth’ of normal skeletal elements. Their size can cause problems such as cardiorespiratory compromise, and the presenting symptoms are usually respiratory. Physical examination and plain chest radiographs usually show a calcified mass arising from the ribs and compressing adjacent normal lung. Treatment should be en bloc excision of all of the involved tissue with a good margin; less than complete excision leads to local recurrence. Chest wall reconstruction is feasible, but scoliosis is a problem in the long term.

Although infections of the chest wall are usually easily diagnosed, osteomyelitis of a rib can occasionally be seen as an isolated lesion. The distinction between infection and Ewing's tumour may require examination of a biopsy specimen. Erythema, pain, tenderness, bony destruction, and mass effect are seen with both diseases. Actinomycosis or an echinococcus cyst presenting as a chest wall mass is usually suspected from the clinical history. Both of these processes are unusual in children and are almost unheard of in infants. The radiographic appearance on plain radiographs, ultrasound examinations, and CT scanning help to establish the correct diagnosis.

Malignant lesions of the chest wall are rare. The patient's outcome is dependent on correct diagnosis, aggressive surgical treatment, and adjuvant chemotherapy or radiotherapy as indicated. Most of the lesions are sarcomas. Although their histological features are similar to those of sarcomas of the extremities, the risk of local recurrence is higher than for the latter, and the propensity for distant metastasis seems to be lower.

The separation of Askin's tumour and Ewing's sarcoma of the ribs has been called into question. These entities may be unified as a single tumour complex because of the similar biological behaviour and the need for similar treatment. These tumours represent the majority of chest wall malignancies. The presence of distant metastases at diagnosis usually predicts a fatal outcome despite intensive chemotherapy.

Aggressive local treatment is indicated because of the high rate of local recurrence. Following a diagnostic biopsy, the combination of preoperative chemotherapy or radiotherapy followed by complete surgical resection is reasonable for treatment of large lesions. Chemotherapy using Adriamycin, vincristine, actinomycin D, and cytoxan has been successful when used in addition to radical local resection, with or without radiotherapy to control local disease.

Deformities of the ribs and sternum defined as pectus carinatum (pigeon breast) or pectus excavatum (funnel chest) are often present at birth. Some patients, however, show sudden change in the contour of the chest wall over a period of a few weeks during a pubertal growth spurt.

The presence of a pectus deformity should alert the surgeon to a number of associated syndromes and potential problems, the most common and potentially most lethal of which is Marfan's syndrome. Patients with Marfan's disease should be evaluated for co-existing cardiac and aortic problems and should be counselled concerning their condition. Avoidance of stressful physical exertion such as competitive sport is probably wise in order to decrease the chances of sudden death. If Marfan's syndrome is suspected, full cardiac evaluation should be undertaken before any decisions are made regarding surgical procedures on the chest wall. Ligamentous and ocular problems are not always present, even when severe valvular heart disease, congestive heart failure, or aortic enlargement can be demonstrated.

Cardiac problems have been associated with chest wall deformities in patients with conditions other than Marfan's syndrome. The aetiology of cardiac dysrhythmias such as atrial fibrillation and paroxysmal supraventricular tachycardia in patients with pectus excavatum is unclear. Clearly, however, there is an increased incidence (up to 4 per cent) of Wolff-Parkinson-White syndrome in patients with pectus excavatum. Many patients with pectus excavatum also have echocardiographic evidence of mitral valve prolapse; this may resolve after correction of the chest wall deformity.

Scoliosis is present in about 20 per cent of patients with pectus deformities. Between 14 and 18 per cent of patients with combined pectus excavatum and scoliosis require treatment for the scoliosis, ranging from bracing of the thorax to arthrodesis of the vertebrae. The degree of the pectus deformity and of physiological impairment are difficult to quantify from examining published reports on the subject. Nearly all children with pectus deformity have no symptoms related to the abnormal chest wall. Mild cardiorespiratory dysfunction may be evident on exercise, but not at rest; these impairments may improve after repair of the deformity. The degree of sternal depression present before and after the surgery is usually not quantified in these studies. Some studies, however, have failed to show any physiological benefit following correction of the chest wall deformity, although the degree of deformity in these patients has rarely been quantified.

A proposed mechanism by which maximal cardiac output and exercise tolerance improves suggests that the left and right atria are compromised by the displaced position of the heart into the left chest. One can imagine that with increasing degrees of sternal depression, the area of expansion of the atria would become more and more limited. At rest the cardiac output is not likely to be affected; however, during exercise there may be space limitations to atrial filling and therefore a limitation to maximal cardiac output. Improvement in the aeration of the lower lobes of the lungs is probably less of a factor in improved cardiac output and exercise tolerance, but may be contributory, especially if some degree of ventilation–perfusion mismatch is present because of less than maximal excursion of the chest wall during maximal ventilation.

Many techniques for repair of the chest wall deformity in pectus excavatum and carinatum have been described before and since Ravitch elucidated the essentials of the repair. Most techniques used today involve resecting the deformed lower costal cartilages in a subperichondrial plane to free the sternum (Fig. 1) 2214. The sternum is then elevated to a normal or even to an ‘over-corrected’ position, usually with the assistance of an anterior osteotomy below the level of the manubrium and resection of the xiphoid process. Rotational deformities are corrected by performing the osteotomy at an oblique angle to correct the depression and rotation simultaneously (Fig. 2) 2215.

Complex sternal deformities may require a second osteotomy for full correction. The sternum is then secured in place using the pectoral muscles and fascia or internal stents of either metal or autologous bone graft from a flat rib (Fig. 3) 2216. The need for internal fixation is a matter of debate, good results being reported with all techniques. Metal struts usually need to be removed by a second procedure; this is usually performed in an outpatient setting, occasionally under local anaesthesia.

Other techniques have been reported to correct the deformed chest wall. Sternal eversion with resection of the deformed costal cartilages has been advocated. Plastic surgical techniques have been used to treat the depression deformity by filling the subcutaneous space with silicone implants or vascularized pedicle flaps; these may give acceptable cosmetic results without correcting any of the physiological problems.

In some patients with co-existing pectus excavatum and heart disease, closing the chest following corrective cardiac surgery may necessitate elevation of the sternum to prevent pressure on the right ventricle and pulmonary outflow tract or on surgically constructed conduits. Repair of the chest wall deformity at the time of cardiac surgery is possible if adequate preparation is made. The chest wall should ideally be repaired before any cardiac surgery to prevent difficulties with bleeding into the mediastinum following costal cartilage resection at the time of cardiac surgery. The accepted techniques for pectus repair do not usually prohibit a median sternotomy being performed after healing has occurred.

Poland's syndrome is often easily distinguished from simple pectus excavatum. The constellation of anomalies in this syndrome include absence of the pectoralis major and pectoralis minor muscles, hand anomalies, absence of ribs, chest wall depression, athelia (absent nipples) and/or amastia, absence of axillary hair, and limited subcutaneous fat on the chest. A single patient may have any number of the anomalies and the defects range from relatively mild to very severe.

Surgical intervention is not indicated in patients with hypoplasia of the ribs. The chest wall problem that leads to the most symptoms and most severe deformity is the absence of ribs. Absence of even small areas of ribs in infants can lead to a functional ‘flail chest’, causing increased respiratory work, lung herniation, atelectasis, and recurrent pneumonia. In addition, the contralateral chest wall often has a carinate deformity, and the sternum is usually rotated. Such patients are best treated with repairing the rotational defect of the sternum, by resecting the deformed costal cartilages, and by stabilizing the chest wall defect due to the absent ribs, using autologous rib from the opposite side. The rib is harvested leaving the periosteum intact to allow for regeneration. The rib graft may be split longitudinally to allow the deformity to be corrected while removing a minimum amount of tissue from the normal area. The chest wall deformity is usually repaired prior to any breast reconstruction, although techniques using the ipsilateral latissimus dorsi muscle as a flap along with rib grafts may allow breast reconstruction as a single operation. In boys with absent ribs use of the latissimus dorsi muscle should probably be avoided because it provides much of the shoulder stability in these patients with absent pectoralis muscles.

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