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Macrodactyly of the great toe for thirty-five years
Journal of Plastic, Reconstructive & Aesthetic Surgery (2009) 62, e520ee522
CORRESPONDENCE AND COMMUNICATION
Macrodactyly of the great toe for thirty-five years Macrodactyly is a rare congenital abnormality in which there is an increase in the size of all elements of the digit.1
Incidence in the foot seems to be much lower than that in the hand.2 Generally, most patients are diagnosed and treated from childhood; however, macrodactyly might become more severe if diagnosis and treatment are delayed for a long time. Herein we report a rare case of long term (35 years) macrodactyly of a toe resulting from delayed treatment.
Figure 1 (A) Dorsal view and (B) medial-thenar view of the left foot showing extreme enlargement and incredible abnormality of the left foot and great toe which curved dorsally laterally. (C) Anteroposterior and (D) oblique radiographs of the left foot showed remarkable overgrowth of the first metatarsal bone and first phalange bone with significant deformity. (E) The deformed left foot and bone with bulky adipose tissue accumulated, compared with the normal right foot. 1748-6815/$ - see front matter ª 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2008.04.070
Correspondence and communication A 35-year-old woman was referred to our hospital with a giant deformed great toe of the left foot present since birth. A big hallux with masses on the left foot were detected at birth, which increased in size as she grew and made the foot deformed, especially the great toe. Furthermore, with the overgrowth of the first ray, her left foot became much larger, wider and thicker. This made walking and shoe wear more and more difficult and even caused the size of the shoes of both feet to differ from each other to such a large extent that the left one is seven sizes larger than the right one. At the age of 35 the patient, who could stand the unsightly appearance, difficulty of shoe wear and ambulation restriction no more, went to our hospital for a radical treatment. She had neither family history of congenital anomalies nor notable pathologic background. On physical examination, the left great toe and forefoot showed extreme enlargement and incredible abnormality (Figure 1A, B). The very deformed hallux, 6 cm in sagittal diameter and 4 cm in horizontal diameter at the proximal phalange, formed a mushroom-like tenacious mass 8 8 7 cm at the distal phalange without a nail on it and pushed the lesser toes to displace laterally. The skin of the sole became remarkably thick and the sole was full of fat, making the foot resemble a flatfoot, or very like elephantiasis. Furthermore, another mass 8 8 cm in size with a broad base was found on the medial dorsum of the left foot. None of the stigmata of neurofibromatosis were found on the patient. A plain radiograph of the left foot showed remarkable overgrowth of the first metatarsal bone and first phalange bone with significant deformity (Figure 1C, D). A CT scan of the left foot (Figure 1E) showed that the adipose layer was apparently thickened. Bulgy sclerosis can be seen in the first metatarsophalangeal articulation and interphalangeal joint, with thickened adipose tissue of lamellar low density detected in the mushroom-like distal phalange of the hallux. The laboratory studies were unremarkable. Summarising the data above, a diagnosis of macrodactyly of the left foot was made. The major aim in the treatment of foot macrodactyly is the reconstruction of pain-free, functional feet with a good cosmetic appearance if possible.3 So, a debulking procedure and toe reconstruction were recommended. However, the patient, who felt strongly about her ugly giant great toe, rejected it and strongly requested an amputation as a final and economical surgery. So, great toe amputation, first metatarsal bone plasty and defatting of the left foot were performed. Final histological test showed a neural fibrolipoma. Fortunately, the wound healed very well after surgery. At the latest follow up 6 months postoperatively, the patient was very satisfied with her foot and shoe wear became easier than preoperation. We graded her left foot as fair, according to Chang’s4 grading system, as it was painless, but she had to wear a shoe that was at least two sizes larger than the opposite foot (Figure 2AeC).
e521 However, in our case, the pathology report of the lesion is neural fibrolipoma with macrodactyly. Therefore, we supposed that both the adipose tissue and the nerve mutation be involved in the lesion, and either might be dominant. For treatment, operative therapy might be the first choice. We agree with Sabapathy et al.3 that the major aim in the treatment of foot macrodactyly is the reconstruction
Discussion The pathogenesis of macrodactyly still remains to be defined. The term macrodystrophia lipomatosa progressive was derived as the basic lesion. In recent studies, Syed5 inclined that in pedal macrodactyly, excessive proliferation and accumulation of adipose tissue was the basic lesion.
Figure 2 (A) The postoperative dorsal view of both feet, and (B) anteroposterior and (C) oblique radiographs of the left foot showed an improved bony structure of the foot.
e522 of pain-free, functional feet. Amputation is a simple way to shorten the involved digit, while not indicated for patients who desire preservation of the nail for aesthetic reasons. The 35-year-old patient presented here accepted amputation at the metatarsophalangeal level combined with the removal of the fibro-fatty tissue because she refused other procedures. Although the patient seemed satisfied with the result of treatment, the final appearance was not as good as expected. For severe macrodactyly of great toe that has remained untreated for a long time, amputation of the first phalanges and plastic operation of the first ray could be an alternative treatment.
References 1. Kotwal PP, Farooque M. Macrodactyly. J Bone Joint Surg 1998; 80-B:651e3.
Correspondence and communication 2. Macrodactyly. In: Kelikian H, editor. Congenital deformities of the hand and forearm. Philadelphia: W.B. Saunders Co; 1974. p. 610e60. 3. Sabapathy SR, Roberts JO, Regan PJ, et al. Pedal macrodactyly treated by digital shortening and free nail graft; a report of two cases. Br J Plast Surg 1990;43:116e9. 4. Hsieh Chang Chia, Jay Kumar S, Riddle Eric C, et al. Macrodactyly of the foot. J Bone Joint Surg 2002;84-A:1189e94. 5. Syed A, Sherwani R, Azam Q, et al. Congenital macrodactyly: a clinical study. Acta Orthop Belg 2005;71:399e404.
Zhao Fengdong Zhou Rongrong Shi Peihua Fan Shunwu Department of Orthopaedics, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, 310016, P. R. China E-mail address: [email protected]
CORRESPONDENCE AND COMMUNICATION
Macrodactyly of the great toe for thirty-five years Macrodactyly is a rare congenital abnormality in which there is an increase in the size of all elements of the digit.1
Incidence in the foot seems to be much lower than that in the hand.2 Generally, most patients are diagnosed and treated from childhood; however, macrodactyly might become more severe if diagnosis and treatment are delayed for a long time. Herein we report a rare case of long term (35 years) macrodactyly of a toe resulting from delayed treatment.
Figure 1 (A) Dorsal view and (B) medial-thenar view of the left foot showing extreme enlargement and incredible abnormality of the left foot and great toe which curved dorsally laterally. (C) Anteroposterior and (D) oblique radiographs of the left foot showed remarkable overgrowth of the first metatarsal bone and first phalange bone with significant deformity. (E) The deformed left foot and bone with bulky adipose tissue accumulated, compared with the normal right foot. 1748-6815/$ - see front matter ª 2008 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2008.04.070
Correspondence and communication A 35-year-old woman was referred to our hospital with a giant deformed great toe of the left foot present since birth. A big hallux with masses on the left foot were detected at birth, which increased in size as she grew and made the foot deformed, especially the great toe. Furthermore, with the overgrowth of the first ray, her left foot became much larger, wider and thicker. This made walking and shoe wear more and more difficult and even caused the size of the shoes of both feet to differ from each other to such a large extent that the left one is seven sizes larger than the right one. At the age of 35 the patient, who could stand the unsightly appearance, difficulty of shoe wear and ambulation restriction no more, went to our hospital for a radical treatment. She had neither family history of congenital anomalies nor notable pathologic background. On physical examination, the left great toe and forefoot showed extreme enlargement and incredible abnormality (Figure 1A, B). The very deformed hallux, 6 cm in sagittal diameter and 4 cm in horizontal diameter at the proximal phalange, formed a mushroom-like tenacious mass 8 8 7 cm at the distal phalange without a nail on it and pushed the lesser toes to displace laterally. The skin of the sole became remarkably thick and the sole was full of fat, making the foot resemble a flatfoot, or very like elephantiasis. Furthermore, another mass 8 8 cm in size with a broad base was found on the medial dorsum of the left foot. None of the stigmata of neurofibromatosis were found on the patient. A plain radiograph of the left foot showed remarkable overgrowth of the first metatarsal bone and first phalange bone with significant deformity (Figure 1C, D). A CT scan of the left foot (Figure 1E) showed that the adipose layer was apparently thickened. Bulgy sclerosis can be seen in the first metatarsophalangeal articulation and interphalangeal joint, with thickened adipose tissue of lamellar low density detected in the mushroom-like distal phalange of the hallux. The laboratory studies were unremarkable. Summarising the data above, a diagnosis of macrodactyly of the left foot was made. The major aim in the treatment of foot macrodactyly is the reconstruction of pain-free, functional feet with a good cosmetic appearance if possible.3 So, a debulking procedure and toe reconstruction were recommended. However, the patient, who felt strongly about her ugly giant great toe, rejected it and strongly requested an amputation as a final and economical surgery. So, great toe amputation, first metatarsal bone plasty and defatting of the left foot were performed. Final histological test showed a neural fibrolipoma. Fortunately, the wound healed very well after surgery. At the latest follow up 6 months postoperatively, the patient was very satisfied with her foot and shoe wear became easier than preoperation. We graded her left foot as fair, according to Chang’s4 grading system, as it was painless, but she had to wear a shoe that was at least two sizes larger than the opposite foot (Figure 2AeC).
e521 However, in our case, the pathology report of the lesion is neural fibrolipoma with macrodactyly. Therefore, we supposed that both the adipose tissue and the nerve mutation be involved in the lesion, and either might be dominant. For treatment, operative therapy might be the first choice. We agree with Sabapathy et al.3 that the major aim in the treatment of foot macrodactyly is the reconstruction
Discussion The pathogenesis of macrodactyly still remains to be defined. The term macrodystrophia lipomatosa progressive was derived as the basic lesion. In recent studies, Syed5 inclined that in pedal macrodactyly, excessive proliferation and accumulation of adipose tissue was the basic lesion.
Figure 2 (A) The postoperative dorsal view of both feet, and (B) anteroposterior and (C) oblique radiographs of the left foot showed an improved bony structure of the foot.
e522 of pain-free, functional feet. Amputation is a simple way to shorten the involved digit, while not indicated for patients who desire preservation of the nail for aesthetic reasons. The 35-year-old patient presented here accepted amputation at the metatarsophalangeal level combined with the removal of the fibro-fatty tissue because she refused other procedures. Although the patient seemed satisfied with the result of treatment, the final appearance was not as good as expected. For severe macrodactyly of great toe that has remained untreated for a long time, amputation of the first phalanges and plastic operation of the first ray could be an alternative treatment.
References 1. Kotwal PP, Farooque M. Macrodactyly. J Bone Joint Surg 1998; 80-B:651e3.
Correspondence and communication 2. Macrodactyly. In: Kelikian H, editor. Congenital deformities of the hand and forearm. Philadelphia: W.B. Saunders Co; 1974. p. 610e60. 3. Sabapathy SR, Roberts JO, Regan PJ, et al. Pedal macrodactyly treated by digital shortening and free nail graft; a report of two cases. Br J Plast Surg 1990;43:116e9. 4. Hsieh Chang Chia, Jay Kumar S, Riddle Eric C, et al. Macrodactyly of the foot. J Bone Joint Surg 2002;84-A:1189e94. 5. Syed A, Sherwani R, Azam Q, et al. Congenital macrodactyly: a clinical study. Acta Orthop Belg 2005;71:399e404.
Zhao Fengdong Zhou Rongrong Shi Peihua Fan Shunwu Department of Orthopaedics, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, 310016, P. R. China E-mail address: [email protected]