Page 26 - 66 thorax49-55_opt

Chest Wall Deformities 335

1/0 polypropylene sutures guided by thoracoscopy. In the past, two by using the bar reverse bender. Once reasonably loose, it is pulled
stabilizers were used, one on either side. This practice has been out from the right side of the chest. The bar should not be forcibly
abandoned by most because, as the chest wall grows, the patient can extracted. In the event of difficulty, any residual scar tissue impinging
develop an hourglass deformity due to restriction in lateral growth, a on the bar should be excised before removal. Bar removal is generally
problem that does not occur with the use of a stabilizer on only one side. uncomplicated. A postoperative chest x-ray should be obtained to rule
The subcutaneous tissues and skin are closed. The lungs are expanded out pneumothorax. Pneumothorax following bar removal usually is
with positive pressures, and pneumothorax is relieved by putting a tube self-limiting and does not require any intervention. 7
underwater through a thoracoscopy port site. 7 Outcomes
A chest x-ray is obtained on day 1 postoperatively (Figure The long-term cosmetic results from the Nuss procedure are as fol-
53.9). Analgesia is maintained with epidural or a patient-controlled lows: excellent, 86%; good, 10.3%; fair, 2.4%; and failed, 1.3% (see
morphine infusion in combination with nonsteroidal anti-inflammatory Figure 53.10). 7
drugs (NSAIDS). A graded programme of incentive spirometry
and physiotherapy is commenced postoperatively. The epidural or Poland’s Syndrome
morphine infusion is usually stopped after the third postoperative Introduction
day. Oral NSAIDS and codeine may be required for up to 3 weeks Poland’s syndrome is a rare congenital malformation involving the
postoperatively. Patients are advised to avoid sporting activity for 3 chest wall and variable severity of other defects involving the areola,
months postoperatively. This allows sufficient scar tissue to develop subcutaneous tissues, muscles, ribs, hand, and heart. The extent of
around the bar, thus fixing it in place and preventing displacement. these defects varies significantly from the absent sternocostal head of
Complications the pectoralis major and/or minor with normal breast and underlying
In experienced hands, surgical complications, summarised in Table ribs to complete absence of anterior portions of second to fifth ribs and
53.1, are uncommon. The majority of early postoperative complica- cartilages. Breast involvement is frequent and is a disfiguring defect in
7
tions can be managed conservatively. Late postoperative complications girls. The hand deformity on the side of the defect is also associated in
also are uncommon (see Table 53.1). Bar displacement is caused by variable frequency from syndactyly to hypoplastic fingers. 6
inadequate fixation of the bar. Hence, it is recommended that the bar be Demographics
fixed by using a bar stabiliser and pericostal sutures. Persistent postop- The reported incidence of Poland’s syndrome is low (1 in 30,000) and
erative pain should be investigated for bar or stabiliser displacement, sporadic in nature. The exact aetiology of this defect is unknown. The
a tight or too long bar, sternal or rib erosion, infection, and bar allergy proposed aetiology is a disruption in the subclavian arterial blood sup-
(i.e., allergy to nickel). 7 ply of the limb bud during the 6th foetal week. 9,10
Bar Removal Clinical Features
The bar is generally removed after 3 years. Under general anaesthesia,
The anatomical abnormalities of Poland’s syndrome are usually unilat-
both lateral incisions containing the stabilizer bar is reopened. All vis-
eral. Clinically, these patients have an absent anterior axillary fold with
ible sutures around the stabiliser are excised. The bar is straightened
the posterior axillary fold being easily visible from the front. The nipple
and areola may be hypoplastic or absent with deficient subcutaneous
Table 53.1: Early and late postoperative complications following bar insertion. 6
tissues. The chest is depressed on the affected side due to hypoplasia
Early Pneumothorax small; most common, conservative treatment or absence of the underlying 2–4 or 3–5 ribs and cartilages. Rarely, the
postoperative Pneumothorax large; chest drainage lung may herniate through the defect in the chest wall, giving a flail
complications
Horner’s syndrome; transient, epidural related segment. This may cause respiratory distress in the newborn period.
Stitch site or wound infection Dextroposition of the heart is common in Poland’s syndrome, rather
than dextrocardia.
Pneumonia
Haemothorax Surgical Options
Pericarditis (postcardiomyotomy syndrome); oral indomethacin The surgical reconstruction options depend on the age of the patient
Pleural effusion; chest drainage and the extent of the defect. In the neonate, a flail chest may require
reconstruction in order to provide a rigid support to counteract the
Late Bar displacement. Major displacement revision required
postoperative Overcorrection paradoxical movement. Split rib grafts harvested from the contralateral
complications unaffected ribs are generally preferred for the replacement of the miss-
Bar allergy ing medial aplastic ribs. The grafts are then attached to the lateral bor-
Recurrence der of the sternum. A mesh sheath can also be used to help bridge large
Skin erosion defects. In older patients, a latissimus dorsi muscle flap can be used to
correct the defect in muscle mass or anterior axillary fold. For girls with
breast hypoplasia, myocutaneous flaps or silicone implants can be used
for post pubertal breast reconstruction. Various combinations of pro-
cedures may have to be used to achieve a satisfactory cosmetic result.
Jeune’s Syndrome
Introduction
Jeune’s syndrome, also known as asphyxiating thoracic dystrophy, is
a rare autosomal recessive disorder. It is characterised by dwarfism,
foreshortened horizontally placed ribs, and short limbs. Thoracic cage
abnormalities (osteochondro dystrophy) result in a markedly small
chest with severe restriction of expansion, pulmonary hypoplasia, and
severe respiratory distress. Its characteristic feature is a “bell-shaped”
chest and a protuberant abdomen.
Figure 53.10: Long-term outcome after removal of pectus bar (Nuss bar).