Elsevier

The Annals of Thoracic Surgery

Volume 74, Issue 6, December 2002, Pages 2218-2225
The Annals of Thoracic Surgery

Review
Poland’s syndrome revisited

https://doi.org/10.1016/S0003-4975(02)04161-9Get rights and content

Abstract

Poland’s syndrome is a rare congenital anomaly characterized by unilateral chest wall hypoplasia and ipsilateral hand abnormalities. Literary data suggest its sporadic nature. The prevailing theory of its cause is hypoplasia of the subclavian artery or its branches, which may lead to a range of developmental changes. The incidence of Poland’s syndrome varies between groups (male versus female patients, congenital versus familial cases, and so on) and ranges from 1 in 7,000 to 1 in 100,000 live births. Cases of Poland’s syndrome associated with leukemia, carcinoma of the hypoplastic breast, and other conditions, confirm the relationship between developmental defects and tumors, and require oncologic awareness. Various manifestations, age, and gender require different surgical approaches. Our experience, which includes 27 patients (15 male, 12 female), 20 of whom (12 male, 8 female) underwent operation, suggests that the repair should be done in two stages in children and in a single stage in adults. Reconstruction and/or stabilization of the aplastic ribs may be achieved using bone grafts or prosthetic mesh. Muscle flaps and breast implants may be used to correct muscle deficiency and breast hypoplasia and to help achieve a complete cosmetic repair.

Section snippets

History

Alfred Poland, a student–demonstrator in anatomy, described his case in 1841; however, there were earlier reports of this anomaly: in 1826 (Lallemand) and in 1839 (Froriep) 7, 8, 9. The full anatomical spectrum of the disease was first summarized by Thompson in 1895. In 1900, Furst suggested a common cause. In 1962, Clarkson coined the name Poland’s syndactyly after more than 300 patients had been reported 1, 10, 11, 12. The condition is also known as hand and ipsilateral thorax syndrome,

Incidence

The reported incidence of Poland’s syndrome ranges from 1 in 7,000 to 1 in 100,000. Males are affected more frequently by a 2:1 to 3:1 ratio 16, 17, 18, 19. Poland’s syndrome has also been diagnosed in 1 of 19,000 mammograms [19]. The right side of the body was found to be involved in 60% to 75% of patients.

There is a substantial difference between sporadic and familial cases of Poland’s syndrome, as well as in its occurrence in different groups (male versus female populations, mild versus

Etiology

Poland’s syndrome is a nongenetic, congenital disorder with low (<1%) risk of reoccurrence in the same family [22]. A report of Poland’s syndrome in one identical female twin suggests the sporadic nature of the syndrome [23]. Familial transmission has been reported only in about 20 patients 24, 25, 26, 27, 28, 29, 30. The vertical transmission from parent to child or affected siblings with normal parents is consistent with delayed mutation of an autosomal dominant gene. Such a dominant mutation

Clinical features

A common denominator of all patients with Poland’s syndrome is the absence of the sternocostal head of the pectoralis major muscle. In most cases, the pectoralis minor muscle is absent as well. The presence of Poland’s syndrome in athletes indicates that its features do not necessarily cause functional impairment 15, 28, 34, 35. The pectoral muscle anatomy may be demonstrated by mediolateral oblique mammograms [29].

In some cases of Poland’s syndrome, the latissimus dorsi, external oblique, and

Operative indications

Surgical intervention may be indicated for the following reasons: (1) unilateral depression of the chest wall and the possibility of its progression, (2) lack of adequate protection of the heart and lung, (3) paradoxical movement of the chest wall, (4) hypoplasia or aplasia of the female breast, and (5) cosmetic defect due to lack of the pectoralis major muscle and axillary fold in male patients.

Surgical treatment

The operative strategy for the treatment of Poland’s syndrome should be guided by the degree of the deformity, the age, and the sex of the patient. The repair may be done in one or two stages.

In children with severe aplasia of the ribs and pronounced depression of the chest wall, the correction should be done in two stages. Initially, the rib defect should be repaired, then a muscle flap transposition should be carried out after puberty. The latter may be combined with mammoplasty in female

Anesthesia

Unilateral ventilation of the opposite lung using a double lumen endotracheal tube is recommended to prevent intraoperative pulmonary injury in patients with aplasia of the ribs and lung herniation [3]. To avoid paradoxical respiration and consequential inadequate pulmonary ventilation, manual ventilation of the lungs with positive pressure is useful in patients with absent ribs [72]. Succinylcholine and halothane are contraindicated for anesthesia in patients with Poland’s syndrome because of

Hand procedure

Separation of the fingers should be performed early, preferably in the first year of life, before abnormal function patterns have developed and the deformity has progressed. Restoration of some hand function in patients with ectrodactylia is possible by toe-transfer or by prosthesis. Parents must be informed that they should expect the hand to remain hypoplastic [34].

Complications

Early attempts using free latissimus dorsi flaps were largely unsuccessful because of atrophy occurring due to omission of a neurovascular pedicle [62]. In patients in which the hypoplastic latissimus dorsi was replanted, partial skin necrosis was also reported [75]. Because the rib grafts may not be effectively stabilized and tend to rotate on their axis, embedding the medial ends of the grafts into a deepened opening in the side of the sternum and suturing grafts to the prosthetic sheet above

Conclusion

Poland’s syndrome is a rare congenital disorder characterized by unilateral chest wall hypoplasia, ipsilateral hand abnormalities, and a variety of associated anomalies. It is suggested that the deformity has a sporadic nature. Hypoplasia of the subclavian artery or one of its branches is considered to be the main pathogenetic mechanism. The incidence of the anomaly varies in different groups, but on average is around 1 in 32,000 live births. The well-known association between developmental

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