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Pectus excavatum: studiously ignored in the United Kingdom?
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  1. ROBERT WHEELER, Consultant Paediatric Surgeon
  1. Wessex Regional Centre for Paediatric Surgery
  2. Southampton General Hospital, Southampton SO16 6YD, UK
  3. Royal Hampshire County Hospital
  4. Winchester, Hampshire, UK
    1. KEITH FOOTE, Consultant Paediatrician
    1. Wessex Regional Centre for Paediatric Surgery
    2. Southampton General Hospital, Southampton SO16 6YD, UK
    3. Royal Hampshire County Hospital
    4. Winchester, Hampshire, UK

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      Pectus excavatum describes a malformation of the anterior chest wall characterised by a hollowing over the sternum and an associated prominence of the costochondral junction. The resulting depression in the chest wall, the opposite situation to pigeon chest (pectus carinatum), is variable in severity, ranging from a mere indentation to an extreme form where the sternum lies within a few centimetres of the vertebral column. The reported incidence is eight per 1000 population, more commonly in boys. It might be anticipated that such a deformity would have significant implications for cardiorespiratory function and pose a cosmetic challenge.

      Patients with pectus excavatum have a mild restrictive ventilatory defect,1 but functional impairment is difficult to demonstrate, appearing at only the extreme limit of exercise tolerance.2 Despite an increase in the intrathoracic volume postoperatively, there is no substantial associated improvement in pulmonary function.3

      The North American and [continental] European literature abound with references to various aspects of this condition: the possible benefits of surgical treatment, the complications of such operations, and the psychological burden associated with the condition. Such literature reveals that pectus surgery is commonplace in these societies, with series of many hundreds of cases being reported.

      The British literature is strangely silent, contributing fewer than 5% of articles cited in medline in the past 10 years. Equally, the referral rate to paediatricians and paediatric/thoracic surgeons appears to be very low, although we are currently conducting a survey of paediatricians with a respiratory interest in Wessex and the South West to quantify this.

      It is undoubtedly true that, unlike their North American colleagues, British paediatric surgeons see very few children with chest wall deformities and there is an overall impression that patients are simply advised to put up with their deformity.

      While obviously disfiguring, even the most trenchant pectus surgeons recognise that correction of the deformity will not usually give significant physiological benefit. The fact that in the face of this North American surgeons are prepared to perform extensive surgery with significant complications implies that they recognise the psychosocial burden4 of such an obvious abnormality. While formerly, the cynic might have pointed to a fee for case arrangement as a motivating factor, modern risk management would have curtailed such activities—but on the contrary, pectus surgery is flourishing.

      The surgery of gynaecomastia in adolescence bears comparison. This condition is known to resolve spontaneously in the vast majority of cases, but the psychosocial burden it places on the child makes subcutaneous mastectomy a recognised necessity in many cases. Pectus, on the other hand will tend to worsen throughout childhood and with the pubertal growth spurt, yet is apparently treated by camouflage rather than correction.

      Historically, the enormity of the necessary surgery may have encouraged surgeons to discourage all but the most severely affected from undergoing an operation. The traditional surgical approach involves a submammary transverse incision, followed by elevation of the skin, subcutaneous tissues, and pectoral muscles to give access to the thoracic cage. The defect is usually corrected by resection of costochondral junctions and multiple osteotomies, although some surgeons advocate physical disconnection of the sternum from all of its cartilage joints, and replacement of the sternum back to front.5

      The refashioned anterior chest wall is then held in place with sutures, struts or rods. All of this occurs through a substantial incision, with significant blood loss and postoperative pain. There have been attempts to minimise the incision and the associated trauma, but this remains major surgery with abundant complications.

      At least 15% of patients will get recurrent excavatum, the more immediate complications include the iatrogenic perforation of any feasible local intrathoracic structures during surgery. Later, migration of supporting metal work into adjacent sites is reported, together with the significant problem of an asphyxiating osteodystrophy, particularly if the surgery is too extensive or performed at too early an age.6

      Therefore, one can sympathise with the reluctance to refer or operate on a child for a “cosmetic” indication. This has led to alternative approaches, such as merely filling the defect with a subcutaneous mould of silicone to abolish the hollow contour.

      However, the “beach” societies such as USA and Australia have not been reluctant to operate for this indication—although in the meantime, have searched for an alternative to a major intervention. This has particularly been driven by the fear of asphyxiating osteodystrophy, where the segment of thoracic cage that has been resected fails to grow, and acts as a constricting band in the mid-zone of the developing chest.

      It appears that a solution has been found.7 Through 2.5 cm incisions on each side of the chest wall, a curved steel bar, moulded to the anticipated anterior thoracic contour is passed between the posterior aspect of the sternum and the pericardium, using direct vision from a thoracoscope. Once in place, the bar is rotated to its final position, forcing the concavity of the sternum anteriorly and abolishing the deformity.

      As with all procedures there are associated complications of infection and postoperative pain, but these are not significantly different from the conventional surgery. However, the lack of any costochondral resection removes the fear of late osteodystrophy. The procedure is minimally invasive taking less operating time than the conventional technique and leaving insignificant scarring. Furthermore, should the procedure fail to achieve the desired cosmetic result, or the deformity recur at a later date, the option of conventional surgery remains.

      It can only be hoped that this advance persuades those caring for patients with pectus excavatum to reconsider the management options. The excuse for persuading a young person that keeping his shirt on at the swimming pool is a better alternative than facing surgery is fading.

      References