|IMAGES IN CLINICAL NEONATOLOGY
|Year : 2016 | Volume
| Issue : 1 | Page : 67-68
Herniating mass in the Chest-Poland syndrome
Iyer Harohalli Venkatesh1, Siddu Charki2, SR Kumar2
1 Consultant Neonatologist, Department of Neonatology, Manipal Hospital, Bangalore, Karnataka, India
2 Department of Neonatology, Fellow in Neonatology, Manipal Hospital, Bangalore, Karnataka, India
|Date of Web Publication||6-Jan-2016|
Iyer Harohalli Venkatesh
Consultant Neonatologist, Manipal Hospital, Bengaluru - 560 017, Karnataka
Source of Support: None, Conflict of Interest: None
A late preterm male infant was admitted to the neonatal intensive care unit with an asymmetric chest wall and a herniating mass through the left second intercostal space on crying. On auscultation, the heart sounds were audible on the right side of the chest. Systemic examination was otherwise unremarkable. A roentgenogram of the chest revealed dextro position of heart and hyperlucent left lung fields. Echocardiogram showed heart on the right with a structurally normal heart. A clinical diagnosis of Poland's syndrome was made based on the hypoplasia of the left pectoral muscles, ribs and nipple, dextroposition of heart and lung herniation. His clinical course was uneventful and doing remarkably well on follow up.
Keywords: Dextroposition of heart, lung herniation, neonate
|How to cite this article:|
Venkatesh IH, Charki S, Kumar S R. Herniating mass in the Chest-Poland syndrome. J Clin Neonatol 2016;5:67-8
Poland syndrome is a rare congenital anomaly, consisting of the combination of unilateral absence of sternocostal head of the pectoralis major muscle with ipsilateral brachysyndactyly. We present a case of Poland syndrome with ipsilateral brachysyndactyly and contralateral single palmar crease presenting with respiratory distress.
A late, preterm, male infant born to a primi mother out of in vitro fertilization was admitted to the neonatal intensive care unit with an asymmetric chest wall and a herniating mass through the left second intercostal space on crying [Figure 1]. On auscultation, the heart sounds were audible on the right side of the chest. Systemic examination was otherwise unremarkable. A roentgenogram of the chest revealed dextrocardia and hyperlucent left lung fields [Figure 2]. Computerized tomography of the chest demonstrated mediastinal shift causing “heart on the right” with absent first and second ribs on the left side anteriorly [Figure 3]. Echocardiogram showed heart on the right with a structurally normal heart. A clinical diagnosis of Poland's syndrome was made. His clinical course was uneventful and doing remarkably well on follow-up.
|Figure 2: Chest X-ray suggestive of dextroposition of heart and deficient anterior 2 ribs on the left side|
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|Figure 3: Contrast computed tomography and reconstructed image demonstrating dextroposition of heart with absent first 2 ribs anteriorly on left side|
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Poland syndrome was first reported by Alfred Poland, a British anatomist, in 1841, as the association of aplasia of the sternal head of the pectoralis major muscle with ipsilateral symbrachydactyly,,, as seen in our case. The term Poland's syndactyly was coined by Clarkson in 1962. The affected infants may have variable associated features, such as underdevelopment or absence of one nipple including the areola and or patchy absence of hair in the axilla. Since then, several cases of Poland syndrome have been reported with varying associations such as rib deformities, Sprengel's deformity, hemivertebrae, axillary webs, radio-ulnar synostosis, absent kidney, and ipsilateral Moebius syndrome (in our index case, single palmar crease is present on the right side). The incidence is 1:25,000 with higher frequency among males. In 75% of the unilateral cases, it is located in the right hemithorax. The majority of the cases are sporadic, and the recurrence risk is negligible; however, reports of the parent to child transmission as well as affected siblings born to unaffected parents were present.
Mechanical factors during embryonic life could explain strong association between left-sided Poland syndrome and dextrocardia. Accordingly, partial agenesis of two or more ribs is needed to displace the heart toward the right side as rightly demonstrated in our index case.
Several reconstructive procedures are available to correct the functional and structural deformities associated with this syndrome. As for the chest deformity for soft tissue reconstruction, transposition of latissimus dorsi muscle has been used by many authors with satisfactory esthetic and functional results.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Friedman T, Reed M, Elliott AM. The carpal bones in Poland syndrome. Skeletal Radiol 2009;38:585-91.
Ferraro GA, Perrotta A, Rossano F, D'Andrea F. Poland syndrome: Description of an atypical variant. Aesthetic Plast Surg 2005;29:32-3.
Cordero García C, Nieto Castilla A, López Jiménez E, Amores García I. Dextrocardia associated with left-sided Poland syndrome. Am J Phys Med Rehabil 2009;88:168.
[Figure 1], [Figure 2], [Figure 3]