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Surgical correction of the hand in epidermolysis bullosa dystrophica
Surgical Correction o f the Hand in Epidermolysis Bullosa Dystrophica M. 3". Gough and R. E. Page
SURGICAL C O R R E C T I O N OF T H E H A N D IN EPIDERMOLYSIS BULLOSA DYSTROPHICA M. J. G O U G H and R. E. PAGE, Leeds SUMMARY Epidermolysis bullosa dystrophica (polydysplastic type) is a rare congenital skin anomaly which, in the hands, because they are exposed to repeated trauma, results in a severe "mitten"-like deformity. Functional benefit was obtained in three patients by separation of the digits and application of split-thickness grafts, Wolfe grafts or "split-off" (epidermis) grafts. Arthrodesis of the interphalangeal joints and filleting of the little finger to provide a flap which could be turned in to the palm, resulted in an improvement in hand function. INTRODUCTION Since the original description of epidermolysis bullosa by Von Hebra in 1870, many different types have been described and classified (Pearson, 1962). Epidermolysis bullosa dystrophica of the polydysplastic type is inherited as an autosomal recessive and of the scarring varieties, is the type most frequently encountered by surgeons. The salient features include formation of skin bullae or blisters throughout life commencing at or soon after birth on those areas exposed to pressure e.g. neck, shoulders, elbows, knees, buttocks, hands and feet. The mucous membrane of the oral cavity, oesophagus and the anal canal may be involved. Patients are frequently anaemic, o f small stature and particularly prone to infection. Many complications may ensue e.g. oesophageal stenosis, squamous cell carcinoma, dental caries, death from infection, but the abnormality to be discussed in this article is the "mitten"-like deformity o f the hands. (Fig. 1). PATIENTS AND METHODS Three patients with epidermolysis bullosa dystrophica (polydysplastic) have been treated in this unit in recent years. Brief case histories are given below:1. SM, a thirteen year old Pakistani girl, was first seen four years ago with mitten deformities of both hands and feet. Surgical release of the digits of the right hand was performed and split-off (epidermis) grafts applied to areas of exposed subcutaneous fat. Due to difficulty in obtaining good extension of the metacarpal joints a transverse palmar releasing incision was made and the resulting defect closed with a flap of skin from the amputated little finger. Temporary immobilisation was obtained with Kirschner wires. No further surgical treatment was undertaken until November 1977. At this time separation of a completely closed right hand was performed together with multiple interphalangeal joint arthrodesis on the thumb, index and ring fingers in an attempt to maintain extension of the digits. At the time of writing this girl has moderate function in the right hand. 2. IB, caucasian female, first presented to us at the age of forty-two years in 1969 with a squamous cell carcinoma on her right forearm which was excised and a splitthickness skin graft applied to the defect. In October 1970 attempts were made to improve function in the right hand by separating the digits and amputating the little R. E. Page, Department of Plastic Surgery, St. James's Hospital, BeckettStreet, Leeds9. TheHand--Vol. 11
No. 1
1979
55
Surgical Correction o f the Hand in Epiderrnolysis Bullosa Dystrophica M. J. Gough and R. E. Page
Fig. 1. Mitten-like deformity of hand.
Fig. 2. Separation of Digits.
finger. Wolfe grafts were applied to defects in the hand. In an attempt to maintain extension, arthrodesis of the interphalangeal joints of the middle and ring fingers was performed. Movement of the right thumb remained poor due to necrosis of the head of the proximal phalanx which was therefore removed. Early in the postoperative period functional return was good but after eighteen months, further surgical separation was necessary and on this occasion disability returned within six months. In July 1977 further separation of the digits of the right hand was performed to produce a three pronged mitten. Split-off (epidermis) grafts were applied to the defects. The mitten deformity returned within six months. 3. EK was a twenty-two year old female when first seen in 1965 with mitten deformities of both hands and feet. Initial treatment of the right hand involved separation of the digits with split-thickness grafts applied to the web spaces and palm. Good function was obtained and the patient requested separation of the left hand which was undertaken in April 1966. On this occasion Wolfe grafts were applied to the pulp o f three fingers where fat was exposed. Thirteen months after the first operation on the right hand a more limited separation of the web spaces was requested. Sixteen months after this (February 1968) the right hand was again completely closed, though surgical intervention produced improvement lasting seven months. During 1969 and 1970 a number of procedures were performed on both hands to try and obtain a more lasting improvement. The digits of both hands were 56
The Hand--VoL 11
No. 1
1979
Surgical Correction o f the Hand in Epidermolysis Bullosa Dystrophica M. J. Gough and R. E. Page
Fig. 3. Split off or epidermis graft.
Fig. 4. Arthrodesis of interphalangeal joints.
separated, Wolfe grafts applied to the thumb web spaces and split-thickness grafts to other defects. Arthrodesis of the proximal interphalangeal~ joints of the middle and ring fingers was performed on both hands, together with arthrodesis of the distal interphalangeal joint of the right middle finger. In addition the little finger of the right hand was amputated and the filleted skin used to cover a defect in the palm of the right hand. In 1973 further separation of a completely closed right hand was performed. In September 1973 the patient died at home. The cause of death was given as acute cardiac failure. DISCUSSION In spite of the severe constitutional nature of this condition and the reduced life span of the patients, we have found that corrective surgery for the hand deformities to be of benefit. The simplest procedure of peeling o f f layers of tissue to allow separation of the fingers was first described by Kitlowski and Banfield (1948). (Fig. 2). We have performed this procedure on three hands but scattered small areas of skin loss were produced and the mitten deformity tended to return after a period of seven months. Separation of the digits followed by split-thickness skin or Wolfe grafting has been described by various authors - - Pers (1965) and Rees and Swingard (1967). It is difficult to gauge the success of such procedures because precise follow-up data was not published. We have performed fourteen split-thickness skin grafts and seven Wolfe grafts. Approximately 60% of both types of graft appeared to take TheHand--Vol. 11
No. 1
1979
57
Surgical Correction o f the Hand in Epidermolysis Bullosa Dystrophica M. J. Gough and R. E. Page
satisfactorily after seven days of immobilisation. In the remaining 40% poor graft take was experienced because of infection, which had often spread from nearby infected bullae. On those occasions when the grafts or bullae have become severely infected, we have used oral Ampicillin, Cloxacillin, Erythromycin and Tetracycline with one case of Penicillin sensitivity. Following such grafts functional benefit was obtained for between six and thirteen months before significant flexion deformity returned. Studies with the electron microscope (Briggaman and Wheeler, 1975) have revealed defective anchoring fibrils in the dermis which results in a natural layer of separation immediately below the basement membrane of the epidermis. We have made use of this plane of separation to obtain epidermis or "split-off" grafts. The size of the "split-off" graft required was lightly incised on the skin and the epidermal layer lifted with the Howarths elevator. (Fig. 3). Such grafts were thin and donor areas tended to heal more rapidly than split-thickness skin donor areas. We have performed eleven "split-off" grafts on hands and the majority of these have taken satisfactorily after seven days. Again recurrent flexion deformity appears after approximately six months. In three cases, because of severe skeletal deformity, we have resorted to amputation of the little finger. This procedure opened the palm and in two cases the skin obtained by filleting the digit was used to correct the shortage of palmar skin as the metacarpal joints were extended. We have performed thirteen arthrodesis procedures with the view to maintaining skeletal position in the face of progressive skin contraction. Arthrodesis of the interphalangeal joints was fairly satisfactory because some finger movement remained possible at the metacarpal phalangeal joints. (Fig. 4). Functional return was usually good in the early post operative phase but it was difficult to assess whether or not a prolonged period of functional benefit has been obtained. ACKNOWLEDGEMENTS
We would like to thank Mr M. H. Shaw and Mr D. S. Eastwood for permission to study their patients and for their encouragement in the preparation of the manuscript. REFERENCES BRIGGAMAN, R. A. and WHEELER, C. E. (1975) The Epidermal-Dermal Junction. The Journal of Investigative Dermatology, 65: 71-84. KITLOWSKI, E. A. and BANFIELD, E. E. (1948) Epidermolysis bullosa hereditaria; report of case with unusual associated deformities. Plastic and Reconstructive Surgery 3:481-488. PEARSON, R. W. (1962) Studies On The Pathogenesis Of Epidermolysis Bullosa. The Journal of Investigative Dermatology 39: 551-575. PERS, M. (1965) Skin Grafting m a Case of Epidermolysis Bullosa. Acta Chirurgica Scandinavica 129: 333-339.
58
The Hand-- Vol. l I
No. l
1979
SURGICAL C O R R E C T I O N OF T H E H A N D IN EPIDERMOLYSIS BULLOSA DYSTROPHICA M. J. G O U G H and R. E. PAGE, Leeds SUMMARY Epidermolysis bullosa dystrophica (polydysplastic type) is a rare congenital skin anomaly which, in the hands, because they are exposed to repeated trauma, results in a severe "mitten"-like deformity. Functional benefit was obtained in three patients by separation of the digits and application of split-thickness grafts, Wolfe grafts or "split-off" (epidermis) grafts. Arthrodesis of the interphalangeal joints and filleting of the little finger to provide a flap which could be turned in to the palm, resulted in an improvement in hand function. INTRODUCTION Since the original description of epidermolysis bullosa by Von Hebra in 1870, many different types have been described and classified (Pearson, 1962). Epidermolysis bullosa dystrophica of the polydysplastic type is inherited as an autosomal recessive and of the scarring varieties, is the type most frequently encountered by surgeons. The salient features include formation of skin bullae or blisters throughout life commencing at or soon after birth on those areas exposed to pressure e.g. neck, shoulders, elbows, knees, buttocks, hands and feet. The mucous membrane of the oral cavity, oesophagus and the anal canal may be involved. Patients are frequently anaemic, o f small stature and particularly prone to infection. Many complications may ensue e.g. oesophageal stenosis, squamous cell carcinoma, dental caries, death from infection, but the abnormality to be discussed in this article is the "mitten"-like deformity o f the hands. (Fig. 1). PATIENTS AND METHODS Three patients with epidermolysis bullosa dystrophica (polydysplastic) have been treated in this unit in recent years. Brief case histories are given below:1. SM, a thirteen year old Pakistani girl, was first seen four years ago with mitten deformities of both hands and feet. Surgical release of the digits of the right hand was performed and split-off (epidermis) grafts applied to areas of exposed subcutaneous fat. Due to difficulty in obtaining good extension of the metacarpal joints a transverse palmar releasing incision was made and the resulting defect closed with a flap of skin from the amputated little finger. Temporary immobilisation was obtained with Kirschner wires. No further surgical treatment was undertaken until November 1977. At this time separation of a completely closed right hand was performed together with multiple interphalangeal joint arthrodesis on the thumb, index and ring fingers in an attempt to maintain extension of the digits. At the time of writing this girl has moderate function in the right hand. 2. IB, caucasian female, first presented to us at the age of forty-two years in 1969 with a squamous cell carcinoma on her right forearm which was excised and a splitthickness skin graft applied to the defect. In October 1970 attempts were made to improve function in the right hand by separating the digits and amputating the little R. E. Page, Department of Plastic Surgery, St. James's Hospital, BeckettStreet, Leeds9. TheHand--Vol. 11
No. 1
1979
55
Surgical Correction o f the Hand in Epiderrnolysis Bullosa Dystrophica M. J. Gough and R. E. Page
Fig. 1. Mitten-like deformity of hand.
Fig. 2. Separation of Digits.
finger. Wolfe grafts were applied to defects in the hand. In an attempt to maintain extension, arthrodesis of the interphalangeal joints of the middle and ring fingers was performed. Movement of the right thumb remained poor due to necrosis of the head of the proximal phalanx which was therefore removed. Early in the postoperative period functional return was good but after eighteen months, further surgical separation was necessary and on this occasion disability returned within six months. In July 1977 further separation of the digits of the right hand was performed to produce a three pronged mitten. Split-off (epidermis) grafts were applied to the defects. The mitten deformity returned within six months. 3. EK was a twenty-two year old female when first seen in 1965 with mitten deformities of both hands and feet. Initial treatment of the right hand involved separation of the digits with split-thickness grafts applied to the web spaces and palm. Good function was obtained and the patient requested separation of the left hand which was undertaken in April 1966. On this occasion Wolfe grafts were applied to the pulp o f three fingers where fat was exposed. Thirteen months after the first operation on the right hand a more limited separation of the web spaces was requested. Sixteen months after this (February 1968) the right hand was again completely closed, though surgical intervention produced improvement lasting seven months. During 1969 and 1970 a number of procedures were performed on both hands to try and obtain a more lasting improvement. The digits of both hands were 56
The Hand--VoL 11
No. 1
1979
Surgical Correction o f the Hand in Epidermolysis Bullosa Dystrophica M. J. Gough and R. E. Page
Fig. 3. Split off or epidermis graft.
Fig. 4. Arthrodesis of interphalangeal joints.
separated, Wolfe grafts applied to the thumb web spaces and split-thickness grafts to other defects. Arthrodesis of the proximal interphalangeal~ joints of the middle and ring fingers was performed on both hands, together with arthrodesis of the distal interphalangeal joint of the right middle finger. In addition the little finger of the right hand was amputated and the filleted skin used to cover a defect in the palm of the right hand. In 1973 further separation of a completely closed right hand was performed. In September 1973 the patient died at home. The cause of death was given as acute cardiac failure. DISCUSSION In spite of the severe constitutional nature of this condition and the reduced life span of the patients, we have found that corrective surgery for the hand deformities to be of benefit. The simplest procedure of peeling o f f layers of tissue to allow separation of the fingers was first described by Kitlowski and Banfield (1948). (Fig. 2). We have performed this procedure on three hands but scattered small areas of skin loss were produced and the mitten deformity tended to return after a period of seven months. Separation of the digits followed by split-thickness skin or Wolfe grafting has been described by various authors - - Pers (1965) and Rees and Swingard (1967). It is difficult to gauge the success of such procedures because precise follow-up data was not published. We have performed fourteen split-thickness skin grafts and seven Wolfe grafts. Approximately 60% of both types of graft appeared to take TheHand--Vol. 11
No. 1
1979
57
Surgical Correction o f the Hand in Epidermolysis Bullosa Dystrophica M. J. Gough and R. E. Page
satisfactorily after seven days of immobilisation. In the remaining 40% poor graft take was experienced because of infection, which had often spread from nearby infected bullae. On those occasions when the grafts or bullae have become severely infected, we have used oral Ampicillin, Cloxacillin, Erythromycin and Tetracycline with one case of Penicillin sensitivity. Following such grafts functional benefit was obtained for between six and thirteen months before significant flexion deformity returned. Studies with the electron microscope (Briggaman and Wheeler, 1975) have revealed defective anchoring fibrils in the dermis which results in a natural layer of separation immediately below the basement membrane of the epidermis. We have made use of this plane of separation to obtain epidermis or "split-off" grafts. The size of the "split-off" graft required was lightly incised on the skin and the epidermal layer lifted with the Howarths elevator. (Fig. 3). Such grafts were thin and donor areas tended to heal more rapidly than split-thickness skin donor areas. We have performed eleven "split-off" grafts on hands and the majority of these have taken satisfactorily after seven days. Again recurrent flexion deformity appears after approximately six months. In three cases, because of severe skeletal deformity, we have resorted to amputation of the little finger. This procedure opened the palm and in two cases the skin obtained by filleting the digit was used to correct the shortage of palmar skin as the metacarpal joints were extended. We have performed thirteen arthrodesis procedures with the view to maintaining skeletal position in the face of progressive skin contraction. Arthrodesis of the interphalangeal joints was fairly satisfactory because some finger movement remained possible at the metacarpal phalangeal joints. (Fig. 4). Functional return was usually good in the early post operative phase but it was difficult to assess whether or not a prolonged period of functional benefit has been obtained. ACKNOWLEDGEMENTS
We would like to thank Mr M. H. Shaw and Mr D. S. Eastwood for permission to study their patients and for their encouragement in the preparation of the manuscript. REFERENCES BRIGGAMAN, R. A. and WHEELER, C. E. (1975) The Epidermal-Dermal Junction. The Journal of Investigative Dermatology, 65: 71-84. KITLOWSKI, E. A. and BANFIELD, E. E. (1948) Epidermolysis bullosa hereditaria; report of case with unusual associated deformities. Plastic and Reconstructive Surgery 3:481-488. PEARSON, R. W. (1962) Studies On The Pathogenesis Of Epidermolysis Bullosa. The Journal of Investigative Dermatology 39: 551-575. PERS, M. (1965) Skin Grafting m a Case of Epidermolysis Bullosa. Acta Chirurgica Scandinavica 129: 333-339.
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The Hand-- Vol. l I
No. l
1979