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Ann Thorac Surg 2002;74:2218-2225
© 2002 The Society of Thoracic Surgeons

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Review

Poland’s syndrome revisited

^a The Department of Thoracic and Cardiovascular Surgery, Carolinas Heart Institute, Carolinas Medical Center, Charlotte, North Carolina, USA

* Address reprint requests to Dr Robicsek, Dept. of Thoracic and Cardiovascular Surgery, Carolinas Medical Center, 1001 Blythe Blvd #300, Charlotte, NC 28203, USA
e-mail: tjohn{at}sanger-clinic.com

	Abstract

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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.

	Introduction

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 

"Clinical manifestations of Poland’s syndrome are extremely variable" [3].

Poland’s syndrome is a rare congenital anomaly characterized by hypoplasia of the breast and nipple, scarcity of subcutaneous tissue, absence of the costosternal portion of the pectoralis major muscle, lack of the pectoralis minor muscle, aplasia or deformity of the costal cartilages or ribs II to IV or III to V, alopecia of the axillary and mammary region, and unilateral brachysyndactyly (Fig 1) [1].

View larger version (59K): [in this window] [in a new window]   Fig 1. Anatomic representation of classic Poland’s syndrome manifestations.

View larger version (106K): [in this window] [in a new window]   Fig 2. Photograph of a patient with Poland’s syndrome.

	History

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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) [7–9]. 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, 10–12]. The condition is also known as hand and ipsilateral thorax syndrome, limb/body-wall defect, fissura thoracis lateralis, acro-pectoral-renal field defect, or subclavian artery supply disruption sequence, and many other names. Concisely, the disease may be defined as "pectoral-aplasia-dysdactylia syndrome" or "unilateral chest-hand deformity." Since the publication of Baudinne and colleagues [13] in 1967, the condition has been generally referred to as Poland’s syndrome.

The first report on the surgical correction of Poland’s syndrome was published in 1952 by Ravitch and Handelsman [14]. A summary of diagnostic and treatment modalities were given in Urschel’s review in 2000 [3]. Recently, Glicenstein also presented a review that included 20 patients and the results of surgical treatment [15].

	Incidence

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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 [16–19]. 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 severe forms). In sporadic cases of Poland’s syndrome, there is a male predominance; also the right side is affected more than twice as often in males, whereas in females, the number of left- and right-side lesions is almost equal. In familial cases, the incidence of Poland’s syndrome in males and females is approximately the same, and there is no right-side predominance [19–21].

	Etiology

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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 [24–30]. 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 may be associated with increased parental (especially paternal) age [24, 26–30].

In the etiology of Poland’s syndrome, currently the prevailing theory is that at the end of the sixth week of gestation, when the upper limb bud adjacent to the chest wall is still in a stage of development, the interruption of the embryonic blood supply causes hypoplasia of the ipsilateral subclavian artery or one of its branches. Data indicating decreased size of the subclavian artery (<50% in diameter) and low flow velocity support this theory. The site and degree of flow impairment may determine the extent and the severity of the anomaly. Hypoplasia of the internal thoracic artery could cause the absence of the sternocostal portion of the pectoralis major muscle, whereas hypoplasia of the brachial artery may lead to hand abnormalities [18, 21, 31].

Another hypothesis is that disruption of the lateral plate mesoderm (from which the pectoralis muscle develops) between 16 and 28 days after fertilization may account for all the defects [20].

A variety of other factors, such as autosomal dominant inheritance, single gene defects, trauma, viral infection, intrauterine insult from attempted abortion, or the teratogenic effects of environmental xenobiotics have also been suggested as causes of Poland’s syndrome [1, 16, 32]. It was also noted that smoking by the mother during pregnancy may double the risk for Poland’s syndrome [33].

	Clinical features

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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 serratus anterior muscles are also affected. Whenever a well-defined axillary fold, composed by the lower border of the pectoralis major muscle is absent, the posterior fold, formed by the latissimus dorsi and teres major muscles, can be seen from the front.

Breast involvement may vary from mild hypoplasia to complete absence (amastia) and is present in more than a third of female patients [36]. The nipple and areola are usually hypoplastic and elevated, lightly pigmented, or even absent (athelia). Supernumerary nipples may also be present. There is only a single report of symptomatic macromastia on the affected side [37].

The rib cage itself may be entirely normal; however, in most patients the chest wall is sunken on one side. The latter is caused by hypoplasia of the ribs and cartilages, which are deformed and thin. As stated before, ribs II to IV, or III to V are most commonly involved, but the second rib is less frequently affected (Fig 3A–D). Aplasia of the anterior portions of between one and three ribs, with severe chest wall depression occurs in about 11% to 25% of patients (Fig 4A–D) [5, 36, 38]. Rib defects are present in 15% of patients with right-sided pectoralis major defects [39]. The sternal ends of aplastic ribs may be fused together and the sternum may rotate toward the involved side, forming an "asymmetrical" contralateral pectus carinatum [5, 28]. The progression of the chest deformity occurs mostly during growth periods [1, 2].

View larger version (84K): [in this window] [in a new window]   Fig 3. Chest involvement in moderate form of Poland’s syndrome. (A) Three-dimensional, oblique view showing chest wall depression with hypoplasia of ribs III to V. Mild rotation of the sternum. (B) Cross-sectional view showing normal position of the heart between the sternum and vertebral column. (C) Lateral view showing unilateral depression of the ribs. (D) Frontal view showing hypoplasia of the involved ribs. Normal position of the heart.

View larger version (84K): [in this window] [in a new window]   Fig 4. Chest involvement in severe form of Poland’s syndrome. (A) Three-dimensional, oblique view showing chest wall defect with aplasia of ribs III to V. Rotation of the sternum. (B) Cross-sectional view showing that the unprotected heart is shifted toward the unaffected side. (C) Lateral view showing unilateral depression of the ribs. (D) Frontal view showing aplasia of the involved ribs. Dextrocardia.

Lung herniation is seen in 8% of the patients. In such patients, paradoxical motion of the chest wall may be of some clinical significance during stress or during severe pulmonary infections [1]. The vital capacity may be as low as 48% of normal and may increase up to 68% of normal after surgical correction [41]. Conventional anteroposterior radiography usually demonstrates a hyperlucent lung on the involved side [29]. A single case of giant bulla in the contralateral lung was described in a patient with left-sided Poland’s syndrome [42]. There is also a tendency for the heart to be shifted toward the unaffected side [28].

The frequency of isolated dextrocardia is about 1 in 30,000 live births. Dextrocardia, which has been reported in 17 patients with Poland’s syndrome, appears to be a dextroposition without inversion. In all these patients, Poland’s syndrome was left-sided and associated with rib defects [39, 43]. Dextrocardia was reported in 5.6% of a series of 144 patients with Poland’s syndrome, and in 9.6% of those, the defect was left-sided [21]. This relatively high number suggests that dextrocardia may be a part of Poland’s complex and that it may be caused by a disturbance of vascular development rather than being the initial phase in the Poland’s sequence. There is an increased occurrence of dextrocardia among the children of consanguineous parents [39, 44]. Although isolated dextrocardia is almost always associated with other cardiovascular anomalies, dextrocardia in Poland’s sequence is not.

Hand abnormalities in Poland’s syndrome are unilateral and vary from shortness of the middle phalanges with cutaneous webbing (syndactyly or mitten hand), to complete absence of the hand (ectrodactyly). Hand involvement contralateral to the chest deformity is rare [34, 45]. Although hand anomalies in Poland’s syndrome were reported to occur from 13.5% to 56%, in turn, about 10% of patients with syndactyly have Poland’s syndrome, as well [5, 10, 34, 40]. Hypoplasia or aplasia of the middle phalanges is not necessarily confined to the digits that are in the syndactyly. Aplasia of the middle phalanges on the ulnar side of the hand is more common than on the radial side. The first web space, as a rule, is moderately shallow. Even if all digits are hypoplastic, the thumb may appear disproportionately small and malrotated. This occurs in only 10% of patients [38, 46]. The lower extremity counterpart of Poland’s syndrome is very rare [47].

There is a well-known association between Poland’s syndrome and Möbius syndrome, that is, a bilateral congenital facial nerve palsy with paralysis of the abductors of the eye [48]. It has been proposed that the cause of Möbius syndrome is a premature regression of the primitive trigeminal arteries [10]. Simultaneous absence of pectoral muscles and ipsilateral hand deformities constitutes the Poland’s–Möbius syndrome, which occurs in 15% of patients with Möbius syndrome and was first reported in 1973 [49, 50]. The first description of a familial case of Poland’s–Möbius syndrome was given by Rojas-Martinez in 1991 [51]. An association of atrial septal defect with Poland’s–Möbius syndrome was also reported [52].

In some patients with Poland’s syndrome, absence of the upper portion of the serratus anterior, which has been attributed to the decrease of blood flow in the suprascapular arteries, leads to elevation and winging of the scapula (Sprengel’s deformity or scapula alata) [21, 53].

Poland’s syndrome may also coexist with Klippel-Feil syndrome, which is characterized by shortness of the neck. The latter is attributed to fusion of the cervical vertebrae and to abnormalities of the brainstem and cerebellum, due to a delay in the development of the vertebral arteries [21].

The association of aplasia of the pectoralis major muscle with renal anomalies, such as unilateral renal agenesis or duplication of the urinary collecting system, is defined as an acro-pectoral-renal field defect, and may cause renal hypertension [54, 55]. For this reason, renal studies are recommended for all patients with aplasia of the pectoralis major muscle [54].

A relationship has been shown between tumors and Poland’s syndrome as well as between tumors and other developmental defects. The explanation may lie in abnormal homeobox and tumor suppressor genes [56]. Cases of Poland’s syndrome have been known to be associated with leukemia, non-Hodgkin’s lymphoma, cervical cancer, leiosarcoma, and lung cancer [57, 58]. Hypoplasia does not exempt the breast from the pathologic processes that are encountered in normal mammary tissue. Because invasive ductal carcinoma was described in the hypoplastic breasts of patients with Poland’s syndrome, cases of Poland’s syndrome should be monitored for early detection of cancer [59]. The association of leukemia and breast cancer with Poland’s syndrome presents a theoretical risk for the use of radiation therapy.

Although there is no substantially increased risk of reoccurrence of the defect in families with only one affected child and no affected parent, genetic counseling should still be a part of the management of patients with congenital abnormalities such as Poland’s syndrome [24–27, 29, 48]. A thorough examination of the proband’s close relatives is recommended, and should be focused on stigmas such as anomalies of dermatoglyphics and minor shortening of the palm. If they are absent, the prognosis is favorable; if there are minimal manifestations, a risk of up to 50% in the offspring must be foreseen [48].

Poland’s syndrome, being unrecognized as a congenital abnormality, could be mistakenly considered the result of a nerve injury caused by trauma [60].

	Operative indications

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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 patients [28, 61].

In children with mild forms of Poland’s syndrome, which is limited to absence of the pectoralis muscles and breast hypoplasia, the operation should be postponed until after puberty. At that time, a muscle flap transposition combined with breast augmentation may be carried out in female patients.

In adults, a one-stage stabilization and reconstruction of the chest wall with a simultaneous muscle transposition is recommended. Again, in women, augmentation mammoplasty may be added to the procedure [2, 3, 35].

Whenever there is a large defect of the ribs and lung herniation, the chest wall should be stabilized by using subperiostially taken split rib grafts from the unaffected side, other bony allo- or autografts, mesh-patch, or a combination of several of the above (Fig 5). If bone grafts are used, the medial end of the grafts are to be sutured into hollows made into the side of the sternum [1]. Laterally, the grafts are attached to the freshened surface of the ends of the ribs. Bone grafts may also be secured in place with heavy-duty staples [28]. The reconstruction of the ribs and cartilages not only allows the cosmetic correction of the defect, but also prevents paradoxical respiration and provides firm support for a muscle flap or for a prosthetic implant. If mesh is used, it should be stretched taut and sutured to the edges of the defect. If both bony grafts and mesh are used, the latter should be sutured to the rib grafts as well. A scarcity of muscle and subcutaneous tissue on the affected side may still be obvious. Transfer of the latissimus dorsi muscle and the implantation of a silicon breast prosthesis may provide the necessary correction.

View larger version (25K): [in this window] [in a new window]   Fig 5. Surgical correction of a severe form of Poland’s syndrome with aplasia of ribs III to V and sternal rotation. (A) Split rib grafts are harvested from the contralateral side. (B) Grafts are sutured medially into previously created sternal notches, and laterally to the ends of the aplastic ribs. Sternal rotation is corrected by osteotomy and secured with a figure-eight suture. (C) Prosthetic mesh is sutured on top of the rib grafts and to the edges of the defect.

View larger version (26K): [in this window] [in a new window]   Fig 6. Surgical correction of Poland’s syndrome with aplasia of the anterior portion of two ribs. (A) Anatomic status and intended lines of incisions. (B) Unaffected ribs above and below the defect are split and attached to the stumps of the aplastic ribs.

Hypoplastic breasts may be augmented using prosthetic implants or musculocutaneous flaps [15]. This should be done only after puberty to adapt it to the size of the contralateral, fully developed breast. A musculocutaneous flap is favored over a simple muscular flap because the skin and subcutaneous tissues are usually too thin and weak to accommodate a considerable volume of transferred muscle and prosthesis beneath them (Fig 7) [2, 3, 65].

View larger version (114K): [in this window] [in a new window]   Fig 7. Single stage reconstruction of the chest in adult patients with Poland’s syndrome. (A) Dorsal view of myocutaneous flap construction with preservation of the thoracodorsal neurovascular pedicle, transfer of the latissimus flap through an axillary tunnel, and suture of the anterior chest wall skin to the island (paddle) of the transplant. (B) Frontal view of myocutaneous flap construction with preservation of the thoracodorsal neurovascular pedicle, transfer of the latissimus flap through an axillary tunnel, and suture of the anterior chest wall skin to the island (paddle) of the transplant. (ART = artery;RECONSTR= reconstruction.) (Reprinted with permission from The Society of Thoracic Surgeons [Ann Thorac Surg 1984, 37, 204–11].)

The condition of the latissimus dorsi muscle will influence both the surgical approach and the results. A hypoplastic latissimus dorsi can be present as an avascular fibrous sheet with a normal-looking posterior axillary fold [67]. Such a situation cannot be diagnosed with a simple clinical examination and the anatomy of the muscle can only be accurately demonstrated by computed tomographic scan or magnetic resonance imaging [68]. If the latissimus dorsi muscle is hypoplastic or missing, microsurgical transfer of the contralateral latissimus dorsi is a viable option. There are also reports of using upper gluteal flaps or microvascular-free transverse rectus abdominis muscle (TRAM) flaps nourished by the internal thoracic vessels as recipient vessels, in lieu of the hypoplastic latissimus dorsi [69, 70]. Preoperative angiography is helpful to provide assessment of the vascular supply before microvascular tissue transfer [18, 67].

A transferred muscle flap alone may be inadequate to provide chest wall projection, especially in patients with large rib defects and hypoplasia of the involved muscles [15, 71]. In such individuals, a prefabricated chest wall implant could be an option [71].

Our experience includes 27 patients (15 male and 12 female), 20 of whom (12 male and 8 female) we operated on. In 14 of our patients, one of the plastic techniques mentioned (separation of fused ribs, freshening of the aplastic ends of involved cartilages, and reattachment to the sternum) was implemented. Twice, bone allografts from the tibia were used. In 6 patients, a Marlex mesh patch was sutured to the edges of the defect without previous manipulation of the moderately deformed and aplastic ribs. Twice, a latissimus dorsi muscle flap was added to the mesh correction.

Plastic procedures using ribs or prosthetic mesh alone are able to correct concave deformities, create a stable anterior chest wall, and prevent lung herniation. The involved hemithorax, however, will remain flatter unless a muscle flap transposition is added. A well-planned physical therapy program designed to create a slight hypertrophy of transformed muscle flaps may further enhance the result [28].

	Anesthesia

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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 the risk of cardiac arrest and malignant hyperthermia [73]. Hemocoagulation disorders in Poland’s syndrome were also reported [74]. Epidural anesthesia with the tip of the catheter placed at the level of the second or third thoracic vertebrae should be applied during and after the operation [28].

	Hand procedure

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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 it is advisable. Also, after covering the rib defect with autogenous fascis lata, slackening may occur in about 6 months, causing the defect to reappear [1].

	Conclusion

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 
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 anomalies and tumors constitutes oncologic awareness in patients with Poland’s syndrome.

In planning the surgical correction, the degree of both the chest wall deformity and muscular deficiency must be considered. Repair may be done in one or two stages, depending on the age and sex of the patient.

Reconstruction or stabilization of the aplastic ribs may be achieved using bone grafts or a prosthetic mesh patch. Muscle flaps and breast implants may be used to correct muscle deficiency and breast hypoplasia and to help achieve a complete cosmetic repair.

	References

Top Abstract Introduction History Incidence Etiology Clinical features Operative indications Surgical treatment Anesthesia Hand procedure Complications Conclusion References

 

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