Unilateral hypoplasia or aplasia of the chest wall
muscles, mainly pectoralis major and cutaneous syndactyly of the ipsilateral hand.
Poland Sequence; Poland Syndactyly; Poland Anomaly.
Named after Sir Alfred Poland who, in 1841, described a chest wall
anomaly while still a medical student studying dissection on cadaver. His observations
were reported in the Guy's Hospital Gazette. He specifically noted the absence of the
sternocostal portion of the pectoralis muscle, absence of the pectoralis minor,
hypoplastic serratus, but the presence of an intact clavicular origin.
Estimated in one review at 1:30,000 to 40,000 live births.
Sporadic but some cases may show autosomal
Unknown, but it has been suggested that there may
be an interruption of early embryonic blood supply in the subclavian
arteries, the vertebral arteries, and/or their branches, resulting in
unilateral upper limb deformities and unilateral or, very rarely, bilateral
chest wall deformities. The term subclavian artery supply disruption
sequence (SASDS) has been suggested for a group of birth defects—Poland,
Moebius, and Klippel-Feil sequences—possibly caused by it.
There may be aplasia of the sternal head of
pectoralis major, of serratus anterior, and of latissimus dorsi. Unilateral
symbrachydactyly may occur and there may be patchy absence of axillary hair.
Unilateral breast aplasia occurs in females. Chest wall defects are variable and may
include rudimentary development or absence of the anterior portions of the
second to fifth ribs. Absence of bone or muscle from the chest wall may
cause paradoxical respiratory movements. The anomaly may be associated with
dextrocardia, atrial septal defect, contralateral syndactyly, club foot, toe
syndactyly, hemivertebrae, and scoliosis. Renal aplasia, hypospadias, and
inguinal hernia may also occur. Situs inversus has been reported with this
Ensure full cardiac investigations,
including echocardiography, if there is suspicion of any cardiac lesion.
Chest radiograph to elucidate extent of bony defect in chest wall. Blood
examination: urea, creatinine, and electrolytes if renal abnormalities are
Because paradoxical chest movement
during spontaneous ventilation may result in inadequate ventilation,
mechanical ventilation is recommended. Antibiotic prophylaxis may be
required for invasive procedures in the presence of cardiac defects. Renal
aplasia requires care with fluid balance and administration of renally
Atracurium, cis-atracurium, or
mivacurium are suitable muscle relaxants if renal aplasia exists.
Suxamethonium should be avoided, especially if associated with Moebius
syndrome. If there is compromised respiratory function, then care should be
taken with the administration of opioids and benzodiazepines.
Poland-Moebius Syndrome: Both the Poland
and the Moebius syndromes are well-described malformations. The Moebius
syndrome is characterized by multiple cranial nerve palsies, orofacial
malformations, and limb anomalies. The association probably represents a
formal genesis malformation syndrome ...