Presentation of Different Types of Hand Anomalies in Poland’s Syndrome

Research Article

Austin Anaplastology. 2016; 1(1): 1003.

Presentation of Different Types of Hand Anomalies in Poland’s Syndrome

Fijalkowska M* and Antoszewski B

Department of Plastic, Reconstructive and Aesthetic Surgery, Medical University of Lodz, Poland

*Corresponding author: Marta Fijalkowska, Department of Plastic, Reconstructive and Aesthetic Surgery, Medical University of Lodz, Poland

Received: September 16, 2016; Accepted: October 22, 2016; Published: November 04, 2016

Abstract

Introduction: Poland’s syndrome is characterized by variable clinical picture. Classic deformity includes unilateral hypoplasia or aplasia of the sternocostal head of the pectoralis major muscle and ipsilateral hand malformations – in such form pathology is known as complete Poland’s anomaly. Variable anomalies in Poland’s syndrome cause that there is no uniform treatment schedule.

The aim of this study is to present different types of hand anomalies in patients with Poland’s syndrome and own experience of its treatment.

Material and Method: An analysis of types of hand defects and their surgical treatment was carried out in the group of 66 patients with Poland’s syndrome who have been hospitalized in Plastic, Reconstructive and Aesthetic Surgery Department /Medical University of Lodz/ during years 1990-2010.

Results: In the analyzed group there were 19 patients with complete Poland’s syndrome, 8 of them were surgically treated. Syndactyly observed in patients with complete Poland’s anomaly was surgically removed in early childhood.

Conclusion: Poland’s syndrome is characterized by variable clinical picture and require individual plan of treatment, depending on patient’s age, sex and severity of clinical defects. The treatment of patients with complete Poland’s syndrome should be started at the kindergarten age and the surgeries mainly involve separation of syndactyly.

Keywords: Poland’s syndrome; Hand anomaly; Classification; Syndactyly; Treatment

Introduction

Poland’s syndrome was first described by Alfred Poland in 1841. It is characterized by a variable clinical picture, while all the potential symptoms are rarely present in a single patient. The typical elements of this anomaly include unilateral hypoplasia or absence of the sternocostal portion of pectoralis major muscle and hand defects affecting the same side of the body [1-4]. In women with Poland’s syndrome, disturbed development of the breast is observed on the affected body side. Breast growth inhibition has many forms, from mild micromastia to amastia [1,3-5].

Coexisting defects of the chest wall and the hand are known as complete Poland’s syndrome [1,6]. However, more commonly, patients suffer from isolated pectoralis major muscle hypoplasia and are diagnosed with partial Poland’s syndrome [1,4,6]. Many authors agree that the key, constant and sufficient symptom which allow the Poland’s syndrome diagnosis are pectoralis major muscle abnormalities [6,7].

The incidence of Poland’s syndrome reported by different authors varies, it ranges from 1:20 000 to 1:100 000 [2-4,6,8]. This condition is three times more common in men than in women and usually the right side is affected in 75% of cases [6,9]. The aetiology of Poland’s syndrome is still under discussion. Disruption of the normal development of the subclavian artery, the vertebral artery and/or their branches, defective migration of fetal mesodermal tissue and intrauterine insults, mainly hypoxia, have been suggested as possible causes [3,9].

In the available literature we can find attempts of classification of hand’s anomalies in Poland’s syndrome.

Gausewitz in 1984 has divided them into four groups: type 1- five digits present even if hypoplastic; type 2- functional border digits with absent central digits (cleft hand); type 3- more severe absence deformities with no functional digits; type 4- radial ray defects and absent thumb [10].

In 1998 Minguella and Cabrera on the basis of clinical and radiological symptoms classified patients with Poland’s syndrome into 5 types: type 1, without syndactyly; type 2 syndactyly and hypoplastic or aplastic middle phalanx; type 3 or “shovel hand” with complete syndactyly also affecting the thumb; type 4 longitudinal deficiency of some digits or radius and type 5 transversal absence [11].

Kuklik (2000) has quoted classification of hand anomalies, which has three subgroups: A- symbrachydactyly associated with brachymesophalangia without oligodactyly; B- the distal phalanges of the second to the fourth fingers are absent; C- the ectrodactyly (cleft) of the hand with no absence of other defect of the carpal bones [12].

The most precise classification was offered by Al-Qattan (2001). He has divided hand’s anomalies in Poland’s syndrome in seven grading types with increasing severity of anomaly:

The aim of this study is to present different types of hand anomalies in patients with Poland’s syndrome and its treatment.

Material and Method

In the years 1990-2010, 66 patients with Poland’s syndrome were treated in Plastic, Reconstructive and Aesthetic Surgery Department/ Medical University of Lodz/. Each patient underwent clinical examination and photographic documentation was performed. On the basis of ambulatory cards, hospital histories and photos the data base of patients with Poland’s anomaly was created. The collected data included: age, sex, and side of anomaly, distinction between complete and partial defect, type of hand anomaly classified according to Al- Qattan (2001) and methods of introduced treatment.

In the analyzed group there were 8 men and 58 women. The patients’ age, at the time of first consultation, was from 6 to 65 years (on average 28.22 years; SD=10.9).

On the basis of patients’ documentation and created data base the analysis of performed treatment was conducted.

Due to the descriptive character of the study, the statistical tests were not accomplished.

Results

In analyzed group only 19 patients had complete Poland’s syndrome (28.8%) (13 females and 6 males).According to Al-Qattan (2001) classification type 2 of anomaly was present in 8 patients (42.1%), subtype 3A in 5 (26.3%), subtype 3B in 3(15.8%) (Figure 1), subtype 4C in 1 (5.3%) and subtype 4D in 2(10.5%) (Figure 2). Remaining 47 patients (71.2%) with partial defect were classified to type 1 of hand malformation.