Tabish Hazir ( The Children’s Hospital, Pakistan Institute of Medical Sciences, Islamabad. )
Munazza Saleem Malik ( The Children’s Hospital, Pakistan Institute of Medical Sciences, Islamabad. )
Poland anomaly was first reported in Guys Hospital report, 18411. Since then many cases have been described. Among the upper limb developmental defects, Poiand anomaly has a prevalence of 1 in 20,000. According to anotherreport 10% of all cases of upper limb syndactyly have Poland anomaly2 also. Poland anomaly is a sporadic developmental disorder involving the upper limb. Primary defect seems to be in the development of the proximal subclavian artery, with early deficit of blood flow to the distal limb and the pectoral region, yielding partial loss of tissue in those regions3
There is a male preponderance with 70% involving the right side. The clinical spectrum is variable depending upon the extent of expression. Upperlimb defect is distal agenesis of varying severity with syndactyly being the commonest presentation. Thoracic deformities include hypoplasia and absence of subcutaneous tissue of anterior chest on the affected side; absence orhypoplasia, with displacement of nipple and breast; absence of sternocostal part of pectoralis major muscle; absence of pectoralis minor and costal cartilage abnormalities4.
One hour old female baby, born full term with birth weight of 2.7Kg, was shifted to neonatal intensive care unit of Childrens’ Hospital on account of multiple congenital abnormalities. On examination, left pectomlis major muscle was absent which resulted in the obvious depression of the chest wall on the left side. There was displacement of right nipple. There was severe distal hypoplasia of the left upper limb with rudimentaiy hand consisting only of skin tags attached to the forearm. Onauscultation, heart sounds were heard on the right side of the chest. Rest of the physical examination was unremarkable.
X-ray chest showed dextmcardia alongwith malformed and mat-aligned left upper ribs which were normal in number. Echocardiography confirmed dextrocardia without associated cardiac abnormalities. Ultrasound of abdomen was normal with no renal abnormality.
Since the first case report in 1841, many cases have been described with vaiying features. Takayama and Ireland et al reviewed 43 cases of Poland anomaly5. Mace, Kaplan et al reported seven cases6. In these case reports, limb and thoracic cage defects are well recognized and virtually constant
features are anomalies of the nipple, subcutaneous tissue and pectoralis major and minor. Upper limb defects with vaiying degree of severity include sy ndactyly, brrachy dactyly, oligodactyly and occasionally more severe reduction deficiency of the upper limb. In addition to these consistent abnormalities, there are certain less common associated abnormalities which are present in some patients of Poland anomaly. Ocrasional associated abnormalities include renal abnormalities7, heniivertebrae, scoliosis and Moebius syndrome exhibiting paralysis of the sixth and seventh cranial nerves8 None of these case reports mention the association of Poland anomaly with dextrocardia which was present in our patient.
1. Poland, A. Deficiency of pectoral muscles. Guy’s Hospital Rep. 1841. In Kenneth Lyons Jones (Ed.) Smiths recognizable patterns of human malfonnations, 4th ed, Philadelphis, W.B. Saunders Co., 1988, pp. 260-61.
2. Claenkson. P. Poland syndactyly. Guy’s Hosp. Rep.. 1962. In Jones K.L. (Ed.) Smith’s recognizable pattern of Human malformations. 4th Ed. Philadelphia, W.B. Saunders Co.. 1988. pp.260-61.
3. Bouvet, J., Maroteaux, P. and Briard-Guillemot, M. Poland\\\'s syndrome: Clinical and genetic studies - physiopathology. Nouv. Presse. Med., 1976;5: 185-7.
4. Goodman, R.M. and Gorlin, R.J. Poland anomaly. The malformed infant and child. New York. Oxford University Press, 1983, pp. 74-75.
5. Ireland, D.C.R., Takayama, N., Flat, A.E. Poland syndrome. A rtwiew of 43 cases. J. Bone Joint Surg., 1976;58 A:52-58
6. Mace, J.W., Kaplan, J.M., Shanenbergen, I.E. et al. Poland syndrome. Report of sevencases and review of literature. Clin, Pcdiatr.. 1972;1 1:98-102.
7. Hoover, G.H., Flatt, A.E. and Weiss, MW. The hand and Apert syndrome. J. Bone Joint Surg., 1970;52 A:878-892.
8. Goodman, R.M. and Gorlin, R.J. Poland anomaly. The malformed infant and child. New York. Oxford University Press, 1983, pp. 74-75.