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Congenital palmar nail syndrome
CONGENITAL
PALMAR
NAIL
SYNDROME
M. A. RIDER From the Queen’s Medical Centre, Nottingham
Two patients are described who have a congenital ectopic nail on the little finger associated with absent flexion in this finger. One patient was treated by surgical excision of the nail. Journal of Hand Surgery (British Volume, 1992) 17B: 371-372
Congenital ectopic nail (the presence of an extra nail in an abnormal site) is a very rare deformity. Two children are described with nails on the palmar surface of the little fingers, who also have a tapering finger, absent flexion and radiographic abnormalities. It is suggested that these features constitute a discrete syndrome, different from simple ectopic nail.
joint was missing. At six-month follow-up the finger was well healed. Discussion There have been fewer than 20 cases of congenital ectopic nail described in the literature. Most of these were small and very abnormal nails occurring in any position on the distal phalanx, wit,h normal function and X-ray appearance. Such nails are typified by the cases reported by Kikuchi (1978) and Aoki (1983). However, there have been cases which more closely resemble the patients described here (Kiryu, 1978 ; Miura, 1978; Kalisman, 1982, 1983; Keret, 1987). These had in common a tapering appearance of the finger, often bilateral and with a predeliction for the ulnar fingers, a
Case reports Cme 1 (Patient of Mr N. J. Barton of Nottingham) An 18-month-old boy was referred with abnormalities of both little fingers which had been present since birth. The fingers were markedly short and tapered. The dorsal nails were normal but on the left there was also an ectopic nail on the palmar surface (Fig. 1). This was well developed, being only slightly smaller and more irregular than the dorsal nail, and the two were just touching at their tips. The skin on the palmar surface was thin and flexion creases were absent. There was no active flexion at either interphalangeal joint and only 30” passively. There was full flexion at the M.P. joint, although the fingers were usually held in hyperextension at this joint. X-rays showed a very small pointed distal phalanx. Joint spaces were present (Fig. 2). The child had had dysplastic hips which had been successfully treated with abduction splints. Both fifth toes were over-riding. There were no other abnormalities and no family history of congenital abnormalities. Case 2 (Patient of Mr P. J. Sykes of Chepstow)
A nine-month-old boy was seen with a similar abnormality (Fig. 3). Both little fingers were small with absent flexion creases. There was no active or passive flexion at either interphalangeal joint. A palmar nail was present on the right little finger. There were no other congenital abnormalities. Interestingly his mother had an absent P.I.P. joint in the right little finger, and absent ulnar rays on the left. Apparently his grandmother and an uncle also have congenital abnormalities of the ulnar rays, although these have not been examined by the author. As the palmar nail was causing problems with infection and catching when the child was aied two years, it was avulsed, the nail bed excised, the skin closed with 6/O catgut and the small remaining defect allowed to granulate. It was noted at operation that there was no profundus tendon and that the volar plate of the D.I.P.
Fig. 1 371
Case 1: palmar
view of the left hand.
312
THE
JOURNAL
OF HAND
SURGERY
VOL.
17B No. 3 JUNE
1992
Fig. 3 Case 2: palmar view of the right hand.
Fig. 2
Case 1: lateral X-ray of the little finger showing the hypoplastic distal phalanx.
The operation performed on the second case appears to be the only record of treatment of this abnormality. It was successful in preventing the problems of infection and catching of the palmar nail, but made no attempt to restore movement to the finger. No operation has yet been performed on the first child. M.R.I. may be useful to investigate the tendons and joints when he is older. If joints are present, it may be possible to construct a flexor mechanism. If not, he may decide to have the finger amputated. Acknowledgements
palmar ectopic nail, absence of active flexion at the interphalangeal joints and a small pointed distal phalanx on X-ray. In these, absence of active flexion was a constant finding, but the range of passive flexion was more varied. Most cases had decreased or absent passive flexion, which suggests either stiffness secondary to disuse or actual absence of the joint. If symphalangism is the cause, it is unusual that this should occur at both distal and proximal interphalangeal joints. One child described by Kalisman (1983) had full passive flexion in the finger which may suggest either a neurological cause, or a mechanical one such as absence of the flexor apparatus. The abnormality is difficult to classify. Symphalangism is regarded as a failure of bony differentiation. The palmar nail should probably be regarded as a duplication, though it is very unusual that duplication should occur in the dorsal-palmar plane as opposed to the more common radial-ulnar plane seen, for example, in polydactyly and mirror hand.
Thanks are due to Mr N. J. Barton and Mr P. J. Sykes for allowing me to report their patients.
References AOKI, K. (1983). Two cases of congenital onychoheterotopia. Japanese Journal of Dermatology, 93: 1167-l 172. KALISMAN, M., GOLBERG, R. and SHIP, A. G. (1982). Dorsal skin and finger nails on the volar aspect of the hand: an unusual anatomic deformity. Plastic and Reconstructive Surgery, 69: 694-696. KALISMAN, M. and KLEINERT, H. E. (1983). A circumferential finger nail finger nail on the palmar aspect of the finger. Journal of Hand Surgery, 8 : 1: 58-60. KERET, D. and GER, E. (1987). Double finger nails on the small fingers. Journal of Hand Surgery, 12A: 608-610. KIKUCHI, I., ONO, T. and OGATA, K. (1978). Ectopic nail case reports. Plastic and Reconstructive Surgery, 61: 5: 781-783. KIRYU, M. (1978). Congenital hypoplasia of the little finger associated with nail anomaly. Japanese Journal of Plastic and Reconstructive Surgery, 21: 3: 216-221. MIURA, T. (1978). Two families with congenital nail anomalies: nail formation in ectopic areas. Journal of Hand Surgery, 3 : 4 : 348-351.
Accepted: 14August 1991 M. A. Rider, 80A Pierrepont 0
1992 The Bntish
Society
Road,
West Bridgford,
for Surgeq
of the Hand
Nottingham
NG2
5DW.
PALMAR
NAIL
SYNDROME
M. A. RIDER From the Queen’s Medical Centre, Nottingham
Two patients are described who have a congenital ectopic nail on the little finger associated with absent flexion in this finger. One patient was treated by surgical excision of the nail. Journal of Hand Surgery (British Volume, 1992) 17B: 371-372
Congenital ectopic nail (the presence of an extra nail in an abnormal site) is a very rare deformity. Two children are described with nails on the palmar surface of the little fingers, who also have a tapering finger, absent flexion and radiographic abnormalities. It is suggested that these features constitute a discrete syndrome, different from simple ectopic nail.
joint was missing. At six-month follow-up the finger was well healed. Discussion There have been fewer than 20 cases of congenital ectopic nail described in the literature. Most of these were small and very abnormal nails occurring in any position on the distal phalanx, wit,h normal function and X-ray appearance. Such nails are typified by the cases reported by Kikuchi (1978) and Aoki (1983). However, there have been cases which more closely resemble the patients described here (Kiryu, 1978 ; Miura, 1978; Kalisman, 1982, 1983; Keret, 1987). These had in common a tapering appearance of the finger, often bilateral and with a predeliction for the ulnar fingers, a
Case reports Cme 1 (Patient of Mr N. J. Barton of Nottingham) An 18-month-old boy was referred with abnormalities of both little fingers which had been present since birth. The fingers were markedly short and tapered. The dorsal nails were normal but on the left there was also an ectopic nail on the palmar surface (Fig. 1). This was well developed, being only slightly smaller and more irregular than the dorsal nail, and the two were just touching at their tips. The skin on the palmar surface was thin and flexion creases were absent. There was no active flexion at either interphalangeal joint and only 30” passively. There was full flexion at the M.P. joint, although the fingers were usually held in hyperextension at this joint. X-rays showed a very small pointed distal phalanx. Joint spaces were present (Fig. 2). The child had had dysplastic hips which had been successfully treated with abduction splints. Both fifth toes were over-riding. There were no other abnormalities and no family history of congenital abnormalities. Case 2 (Patient of Mr P. J. Sykes of Chepstow)
A nine-month-old boy was seen with a similar abnormality (Fig. 3). Both little fingers were small with absent flexion creases. There was no active or passive flexion at either interphalangeal joint. A palmar nail was present on the right little finger. There were no other congenital abnormalities. Interestingly his mother had an absent P.I.P. joint in the right little finger, and absent ulnar rays on the left. Apparently his grandmother and an uncle also have congenital abnormalities of the ulnar rays, although these have not been examined by the author. As the palmar nail was causing problems with infection and catching when the child was aied two years, it was avulsed, the nail bed excised, the skin closed with 6/O catgut and the small remaining defect allowed to granulate. It was noted at operation that there was no profundus tendon and that the volar plate of the D.I.P.
Fig. 1 371
Case 1: palmar
view of the left hand.
312
THE
JOURNAL
OF HAND
SURGERY
VOL.
17B No. 3 JUNE
1992
Fig. 3 Case 2: palmar view of the right hand.
Fig. 2
Case 1: lateral X-ray of the little finger showing the hypoplastic distal phalanx.
The operation performed on the second case appears to be the only record of treatment of this abnormality. It was successful in preventing the problems of infection and catching of the palmar nail, but made no attempt to restore movement to the finger. No operation has yet been performed on the first child. M.R.I. may be useful to investigate the tendons and joints when he is older. If joints are present, it may be possible to construct a flexor mechanism. If not, he may decide to have the finger amputated. Acknowledgements
palmar ectopic nail, absence of active flexion at the interphalangeal joints and a small pointed distal phalanx on X-ray. In these, absence of active flexion was a constant finding, but the range of passive flexion was more varied. Most cases had decreased or absent passive flexion, which suggests either stiffness secondary to disuse or actual absence of the joint. If symphalangism is the cause, it is unusual that this should occur at both distal and proximal interphalangeal joints. One child described by Kalisman (1983) had full passive flexion in the finger which may suggest either a neurological cause, or a mechanical one such as absence of the flexor apparatus. The abnormality is difficult to classify. Symphalangism is regarded as a failure of bony differentiation. The palmar nail should probably be regarded as a duplication, though it is very unusual that duplication should occur in the dorsal-palmar plane as opposed to the more common radial-ulnar plane seen, for example, in polydactyly and mirror hand.
Thanks are due to Mr N. J. Barton and Mr P. J. Sykes for allowing me to report their patients.
References AOKI, K. (1983). Two cases of congenital onychoheterotopia. Japanese Journal of Dermatology, 93: 1167-l 172. KALISMAN, M., GOLBERG, R. and SHIP, A. G. (1982). Dorsal skin and finger nails on the volar aspect of the hand: an unusual anatomic deformity. Plastic and Reconstructive Surgery, 69: 694-696. KALISMAN, M. and KLEINERT, H. E. (1983). A circumferential finger nail finger nail on the palmar aspect of the finger. Journal of Hand Surgery, 8 : 1: 58-60. KERET, D. and GER, E. (1987). Double finger nails on the small fingers. Journal of Hand Surgery, 12A: 608-610. KIKUCHI, I., ONO, T. and OGATA, K. (1978). Ectopic nail case reports. Plastic and Reconstructive Surgery, 61: 5: 781-783. KIRYU, M. (1978). Congenital hypoplasia of the little finger associated with nail anomaly. Japanese Journal of Plastic and Reconstructive Surgery, 21: 3: 216-221. MIURA, T. (1978). Two families with congenital nail anomalies: nail formation in ectopic areas. Journal of Hand Surgery, 3 : 4 : 348-351.
Accepted: 14August 1991 M. A. Rider, 80A Pierrepont 0
1992 The Bntish
Society
Road,
West Bridgford,
for Surgeq
of the Hand
Nottingham
NG2
5DW.