- Email: [email protected]
Reflex sympathetic dystrophy associated with deep peroneal nerve entrapment
Brain & Development 20 (1998) 80–82
Original article
Reflex sympathetic dystrophy associated with deep peroneal nerve entrapment Enrico Parano a , b , d ,*, Vito Pavone c, Filippo Greco d, Marcello Majorana e, Rosario R. Trifiletti a a
Department of Neurology and Neuroscience, Cornell University, New York, NY, USA b IBFSNC, CNR, Catania, Italy c Orthopedic Clinic, University of Catania, Catania, Italy d Division of Pediatric Neurology, University of Catania, Catania, Italy e Institute of Radiology, University of Catania, Catania, Italy
Received 21 March 1997; revised version received 6 October 1997; accepted 29 October 1997
Abstract Reflex sympathetic dystrophy (RSD or causalgia) has been rarely reported in children. We now report a 10-year-old boy with RSD in whom we performed extensive ancillary investigations including thermography, bone scintigraphy and dermo-echography. The clinical, laboratory and ancillary studies reported here highlight the profound focal acquired autonomic disturbance in this disorder. The patient presented a compression of the deep peroneal nerve at the inferior extensor retinaculum in the affected limb. Surgical decompression lead to rapid improvement of the patient’s condition. Peripheral nerve decompression should be considered as a possible treatment of RSD in children, particularly those that are refractory to conservative maneuvers. 1998 Elsevier Science B.V. Keywords: Reflex sympathetic dystrophy; Causalgia; Entrapment neuropathy; Peroneal nerve; Thermography; Scintigraphy
1. Introduction Reflex sympathetic dystrophy (RSD) is well-recognized in adults, but has been infrequently reported in children [1,2]. As in adults, RSD in children frequently follows trauma, casting, surgery, congenital anomaly and tumor [3]. In adults, an association between RSD and nerve compression has been noted [4,5], but a similar association has not been reported in children. We now report a child with clinical and ancillary evidence of RSD who also has evidence of compression of the deep peroneal nerve. Signs and symptoms of RSD, which had previously been refractory to therapy, rapidly improved with nerve decompression.
2. Case report A 10-year-old boy was referred to the University of Cat-
* Corresponding author. Clinica Pediatrica, Universita’ di Catania, Viale Andrea Doria 6, 95125 Catania, Italy. Fax: +39 95 222532.
0387-7604/98/$19.00 1998 Elsevier Science B.V. All rights reserved PII S0387-7604 (97 )0 0106-X
ania, Italy, for a several month history of pain in his left foot, which had rendered him unable to walk. Since the age of about 6, the child experienced recurrent headaches, lasting for minutes to 6–8 h. For several years prior, he had recurrent episodes of abdominal pain, aerophagia, nausea and vomiting. Several medical specialists were consulted and the child was considered to be ‘pain prone’, without clear organic basis, and was referred for psychotherapy. At the age of 7, he presented again with acute abdominal pain and was hospitalized. Tests at this time included also a barium enema, upper GI series, urography, cystoscopy, esophagogastroduodenoscopy, abdominal echography, MRI of the head and spine, and electroencephalogram, all of which resulted normal. At the age of 9, he began to experience multiple episodes of excruciating pain in the dorsum of his left foot. This was initially successfully treated with local anti-inflammatory agents. Six months later, following a mild injury while playing soccer, he sustained a minor left foot trauma. After about a month, he again experienced pain and weakness in his left foot. This progressed to a greater extent than previously, and anti-inflammatory agents failed to provide
E. Parano et al. / Brain & Developlment 20 (1998) 80–82
81
benefit. Plain X-rays of the left foot were normal. Immobilization of the affected extremity by casting failed to provide benefit, and in fact lead to exacerbation of foot pain. The cast was removed after about 1 month. The patient was then hospitalized at the Pediatric Clinic of the University of Catania, Italy, for further evaluation and management. On physical examination he appeared to be in distress from continuous left foot pain. Skin appeared mildly mottled throughout with hyperidrosis except in the left limb. The left extremity was hypotrofic, dry, cold to touch and cyanotic. The patient complained of dull pain in the left foot, most marked dorsally; this pain was noted spontaneously but was greatly exacerbated by the slightest motion
Fig. 3. 99mTc-MDP scintigraphy-early vascular phase. Reduced perfusion in the left foot (SN) as compared to the right foot (DX).
Fig. 1. Thermography. Marked hypothermia of the left limb in a ‘stocking glove distribution’. Thermal differences between left and right limbs are at least 4°C at the foot and ankle, and 1.5°C at the knee. From top to the bottom, each colour represent 1°C of difference: white on top scale, warmer; blue on the bottom scale, colder. Numbers in the left scale, refer a thermal difference of one grade °C, correlated to the colour’s scale. Fig. 2. Dermo-echography. Upper panel, increased echodensity of the left foot (L) due to mild fibrosis and anhidrosis as compared to the right foot (R). Lower panel, same findings of above at 2× magnification.
or light touch. The patient had minimal ankle motion and no motion of the toes, as these movements elicited pain. The remainder of the neurologic examination was normal. Plain X-rays of the foot on admission suggested mild osteopenia, more marked in the left than right foot. Computed tomography of the left foot suggested mild unspecific musculotendinous inflammation, probably secondary to RSD. Thermography of the inferior limbs revealed marked hypothermia of the left leg at the foot and, to a lesser extent, the calf and distal third of the thigh. The thermal differences between the left and right feet were at least 4°C at the foot and ankle, and 1.5°C at the knee (Fig. 1). Dermo-echography showed increased echodensity of the left foot, with a mild increase in fibrous tissue and signs of anhidrosis (Fig. 2). Triphasic bone scintigraphy with technetium 99m-methylene diphosphonate (99mTc-MDP) suggested reduced perfusion of the left foot and calf during the early phase (at 5 min) (Fig. 3). A late phase study (at 2 h) showed decreased radionuclide uptake in the left foot. Nerve conduction studies (NCS) and needle electromyography (EMG) could not be performed due to the marked pain elicited. Other diagnostic tests included CT scan of the abdomen, pelvis and lumbosacral spinal cord and Doppler studies of the lower extremities, all of which returned normal. A battery of laboratory tests, including glucose, albumin, creatine phosphokinase, hemogram, anti-nuclear antibodies, C3 and C4, C-reactive protein and rheumatoid factor were all normal. The patient was referred to an orthopedic hospital where surgical exploration of the left foot demonstrated a normal color, volume and course of the deep peroneal nerve. Proximally, at the inferior extensor retinaculum, a small band of
82
E. Parano et al. / Brain & Development 20 (1998) 80–82
fibro-adipose tissue was present. Decompression of the nerve lead to marked reduction in pain and improved mobility at the metarso-phalangeal joints within a few days.
3. Discussion Reflex sympathetic dystrophy (RSD) in both adults and children features focal limb pain with paroxysmal exacerbation, associated with other features including edema, abnormal sweating, cyanosis or erythema [1]. In severe cases, and more commonly in adults than children, RSD includes dystrophic skin changes and muscle atrophy. Although well-described in children, RSD is uncommon, especially in males: 52 of the 829 patients with RSD reported by Veldman et al. [6] were less than 20 years of age and of those, only eight were male. Our patient is typical of reported cases of childhood RSD in that he developed persistent focal pain, apparently without any specific cause. In a review of their experience with 70 children with RSD, Wilder et al. [2] found a mean age of 12.5 years, with 61 of their cases involving the lower extremities and 35 occurring after sports injury. Using the grading system proposed by Bechter and Casten [7], our patient is classified as grade 1 (severe RSD) as were eight of the 18 childhood RSD patients reported by Silber and Majd [1]. Three ancillary studies confirm the clinical diagnosis of RSD in our patient. Using thermography (Fig. 1), we documented marked relative hypothermia of the left lower extremity to the level of the distal third of the thigh. The thermographic abnormality observed has a typical ‘stocking glove’ distribution also seen in all of the six children with RSD reported by Lightman et al. [8]. Unlike the two patients reported by Lightman et al. [8] with extensive thermographic abnormalities of a lower limb, our patient did not improve after several months of conservative measures. Bone scintigraphy showed decreased radionuclide uptake in the early (Fig. 3) and late phases. Decreased late phase uptake was also seen in 11 of 15 children studied by 99mTcMDP scintigraphy by Goldsmith et al. [9]; this result differs from adult RSD, in which radionuclide uptake is usually increased. We also performed dermo-echography (Fig. 2), which apparently has not been previously reported in RSD, showing abnormalities which may correlate with anhidrosis and dystrophic changes, but the significance and specificity of these findings are unknown. Our patient had deep peroneal nerve entrapment observed at surgical exploration. In our patient it seems to be more
likely that the entrapment of the deep peroneal nerve was the result of RSD and the compression made RSD itself worse. A strong association between peripheral nerve compression and RSD has been reported in adults; Grundberg and Reagan [4] found evidence of compression neuropathy in 28 of 93 adults with upper extremity RSD, all of whom failed to respond to conservative measures and responded dramatically to nerve decompression. In another series [5], 30 of 35 adults had RSD associated with peripheral nerve compression. This is, to our knowledge, the first report of RSD associated with surgically-observed nerve compression in a child, and it illustrates that peripheral nerve decompression should be considered when evaluating children with RSD, particularly those that are refractory to conservative measures.
Acknowledgements Dr Parano was supported by IBFSNC, CNR, Catania, Italy. Dr Trifiletti was supported by the John Merck Fund.
References [1] Silber TJ, Majd M. Reflex sympathetic dystrophy syndrome in children and adolescents: report of 18 cases and review of the literature. Am J Dis Child 1988;142:1325–1330. [2] Wilder RT, Berde CB, Wolohan M, Vieyra MA, Masek BJ, Micheli LJ. Reflex sympathetic dystrophy in children. J Bone Jt Surg (Am) 1992;74:910–919. [3] Jones HR Jr, Felice KJ, Gross PT. Pediatric peroneal mononeuropathy: a clinical and electromyographic study. Muscle Nerve 1993;16:1167–1173. [4] Grundberg AB, Reagan DS.Compression syndromes in reflex sympathetic dystrophy. J Hand Surg (Am) 1991;16:731–736. [5] Monsivais JJ, Baker J, Monsivais D. The association of peripheral nerve compression and reflex sympathetic dystrophy. J Hand Surg (Br) 1993;18:337–338. [6] Veldman PH, Reynen HM, Arntz IE, Goris RJ. Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet 1993;342:1012–1016. [7] Bechter AM, Casten DR. Reflex sympathetic dystrophy: criteria for diagnosis and treatment. Anesthesiology 1955;16:994–1003. [8] Lightman HI, Pochaczevsky R, Aprin H, Ilowite NY. Thermography in childhood reflex sympathetic dystrophy. J Pediatr 1987;111:551– 555. [9] Goldsmith DP, Vivino FB, Eichenfeld AH, Athreya BH, Heyman S. Nuclear imaging and clinical features of childhood reflex neurovascular dystrophy: comparison with adults. Arthritis Rheum 1989;32:480–485.
Original article
Reflex sympathetic dystrophy associated with deep peroneal nerve entrapment Enrico Parano a , b , d ,*, Vito Pavone c, Filippo Greco d, Marcello Majorana e, Rosario R. Trifiletti a a
Department of Neurology and Neuroscience, Cornell University, New York, NY, USA b IBFSNC, CNR, Catania, Italy c Orthopedic Clinic, University of Catania, Catania, Italy d Division of Pediatric Neurology, University of Catania, Catania, Italy e Institute of Radiology, University of Catania, Catania, Italy
Received 21 March 1997; revised version received 6 October 1997; accepted 29 October 1997
Abstract Reflex sympathetic dystrophy (RSD or causalgia) has been rarely reported in children. We now report a 10-year-old boy with RSD in whom we performed extensive ancillary investigations including thermography, bone scintigraphy and dermo-echography. The clinical, laboratory and ancillary studies reported here highlight the profound focal acquired autonomic disturbance in this disorder. The patient presented a compression of the deep peroneal nerve at the inferior extensor retinaculum in the affected limb. Surgical decompression lead to rapid improvement of the patient’s condition. Peripheral nerve decompression should be considered as a possible treatment of RSD in children, particularly those that are refractory to conservative maneuvers. 1998 Elsevier Science B.V. Keywords: Reflex sympathetic dystrophy; Causalgia; Entrapment neuropathy; Peroneal nerve; Thermography; Scintigraphy
1. Introduction Reflex sympathetic dystrophy (RSD) is well-recognized in adults, but has been infrequently reported in children [1,2]. As in adults, RSD in children frequently follows trauma, casting, surgery, congenital anomaly and tumor [3]. In adults, an association between RSD and nerve compression has been noted [4,5], but a similar association has not been reported in children. We now report a child with clinical and ancillary evidence of RSD who also has evidence of compression of the deep peroneal nerve. Signs and symptoms of RSD, which had previously been refractory to therapy, rapidly improved with nerve decompression.
2. Case report A 10-year-old boy was referred to the University of Cat-
* Corresponding author. Clinica Pediatrica, Universita’ di Catania, Viale Andrea Doria 6, 95125 Catania, Italy. Fax: +39 95 222532.
0387-7604/98/$19.00 1998 Elsevier Science B.V. All rights reserved PII S0387-7604 (97 )0 0106-X
ania, Italy, for a several month history of pain in his left foot, which had rendered him unable to walk. Since the age of about 6, the child experienced recurrent headaches, lasting for minutes to 6–8 h. For several years prior, he had recurrent episodes of abdominal pain, aerophagia, nausea and vomiting. Several medical specialists were consulted and the child was considered to be ‘pain prone’, without clear organic basis, and was referred for psychotherapy. At the age of 7, he presented again with acute abdominal pain and was hospitalized. Tests at this time included also a barium enema, upper GI series, urography, cystoscopy, esophagogastroduodenoscopy, abdominal echography, MRI of the head and spine, and electroencephalogram, all of which resulted normal. At the age of 9, he began to experience multiple episodes of excruciating pain in the dorsum of his left foot. This was initially successfully treated with local anti-inflammatory agents. Six months later, following a mild injury while playing soccer, he sustained a minor left foot trauma. After about a month, he again experienced pain and weakness in his left foot. This progressed to a greater extent than previously, and anti-inflammatory agents failed to provide
E. Parano et al. / Brain & Developlment 20 (1998) 80–82
81
benefit. Plain X-rays of the left foot were normal. Immobilization of the affected extremity by casting failed to provide benefit, and in fact lead to exacerbation of foot pain. The cast was removed after about 1 month. The patient was then hospitalized at the Pediatric Clinic of the University of Catania, Italy, for further evaluation and management. On physical examination he appeared to be in distress from continuous left foot pain. Skin appeared mildly mottled throughout with hyperidrosis except in the left limb. The left extremity was hypotrofic, dry, cold to touch and cyanotic. The patient complained of dull pain in the left foot, most marked dorsally; this pain was noted spontaneously but was greatly exacerbated by the slightest motion
Fig. 3. 99mTc-MDP scintigraphy-early vascular phase. Reduced perfusion in the left foot (SN) as compared to the right foot (DX).
Fig. 1. Thermography. Marked hypothermia of the left limb in a ‘stocking glove distribution’. Thermal differences between left and right limbs are at least 4°C at the foot and ankle, and 1.5°C at the knee. From top to the bottom, each colour represent 1°C of difference: white on top scale, warmer; blue on the bottom scale, colder. Numbers in the left scale, refer a thermal difference of one grade °C, correlated to the colour’s scale. Fig. 2. Dermo-echography. Upper panel, increased echodensity of the left foot (L) due to mild fibrosis and anhidrosis as compared to the right foot (R). Lower panel, same findings of above at 2× magnification.
or light touch. The patient had minimal ankle motion and no motion of the toes, as these movements elicited pain. The remainder of the neurologic examination was normal. Plain X-rays of the foot on admission suggested mild osteopenia, more marked in the left than right foot. Computed tomography of the left foot suggested mild unspecific musculotendinous inflammation, probably secondary to RSD. Thermography of the inferior limbs revealed marked hypothermia of the left leg at the foot and, to a lesser extent, the calf and distal third of the thigh. The thermal differences between the left and right feet were at least 4°C at the foot and ankle, and 1.5°C at the knee (Fig. 1). Dermo-echography showed increased echodensity of the left foot, with a mild increase in fibrous tissue and signs of anhidrosis (Fig. 2). Triphasic bone scintigraphy with technetium 99m-methylene diphosphonate (99mTc-MDP) suggested reduced perfusion of the left foot and calf during the early phase (at 5 min) (Fig. 3). A late phase study (at 2 h) showed decreased radionuclide uptake in the left foot. Nerve conduction studies (NCS) and needle electromyography (EMG) could not be performed due to the marked pain elicited. Other diagnostic tests included CT scan of the abdomen, pelvis and lumbosacral spinal cord and Doppler studies of the lower extremities, all of which returned normal. A battery of laboratory tests, including glucose, albumin, creatine phosphokinase, hemogram, anti-nuclear antibodies, C3 and C4, C-reactive protein and rheumatoid factor were all normal. The patient was referred to an orthopedic hospital where surgical exploration of the left foot demonstrated a normal color, volume and course of the deep peroneal nerve. Proximally, at the inferior extensor retinaculum, a small band of
82
E. Parano et al. / Brain & Development 20 (1998) 80–82
fibro-adipose tissue was present. Decompression of the nerve lead to marked reduction in pain and improved mobility at the metarso-phalangeal joints within a few days.
3. Discussion Reflex sympathetic dystrophy (RSD) in both adults and children features focal limb pain with paroxysmal exacerbation, associated with other features including edema, abnormal sweating, cyanosis or erythema [1]. In severe cases, and more commonly in adults than children, RSD includes dystrophic skin changes and muscle atrophy. Although well-described in children, RSD is uncommon, especially in males: 52 of the 829 patients with RSD reported by Veldman et al. [6] were less than 20 years of age and of those, only eight were male. Our patient is typical of reported cases of childhood RSD in that he developed persistent focal pain, apparently without any specific cause. In a review of their experience with 70 children with RSD, Wilder et al. [2] found a mean age of 12.5 years, with 61 of their cases involving the lower extremities and 35 occurring after sports injury. Using the grading system proposed by Bechter and Casten [7], our patient is classified as grade 1 (severe RSD) as were eight of the 18 childhood RSD patients reported by Silber and Majd [1]. Three ancillary studies confirm the clinical diagnosis of RSD in our patient. Using thermography (Fig. 1), we documented marked relative hypothermia of the left lower extremity to the level of the distal third of the thigh. The thermographic abnormality observed has a typical ‘stocking glove’ distribution also seen in all of the six children with RSD reported by Lightman et al. [8]. Unlike the two patients reported by Lightman et al. [8] with extensive thermographic abnormalities of a lower limb, our patient did not improve after several months of conservative measures. Bone scintigraphy showed decreased radionuclide uptake in the early (Fig. 3) and late phases. Decreased late phase uptake was also seen in 11 of 15 children studied by 99mTcMDP scintigraphy by Goldsmith et al. [9]; this result differs from adult RSD, in which radionuclide uptake is usually increased. We also performed dermo-echography (Fig. 2), which apparently has not been previously reported in RSD, showing abnormalities which may correlate with anhidrosis and dystrophic changes, but the significance and specificity of these findings are unknown. Our patient had deep peroneal nerve entrapment observed at surgical exploration. In our patient it seems to be more
likely that the entrapment of the deep peroneal nerve was the result of RSD and the compression made RSD itself worse. A strong association between peripheral nerve compression and RSD has been reported in adults; Grundberg and Reagan [4] found evidence of compression neuropathy in 28 of 93 adults with upper extremity RSD, all of whom failed to respond to conservative measures and responded dramatically to nerve decompression. In another series [5], 30 of 35 adults had RSD associated with peripheral nerve compression. This is, to our knowledge, the first report of RSD associated with surgically-observed nerve compression in a child, and it illustrates that peripheral nerve decompression should be considered when evaluating children with RSD, particularly those that are refractory to conservative measures.
Acknowledgements Dr Parano was supported by IBFSNC, CNR, Catania, Italy. Dr Trifiletti was supported by the John Merck Fund.
References [1] Silber TJ, Majd M. Reflex sympathetic dystrophy syndrome in children and adolescents: report of 18 cases and review of the literature. Am J Dis Child 1988;142:1325–1330. [2] Wilder RT, Berde CB, Wolohan M, Vieyra MA, Masek BJ, Micheli LJ. Reflex sympathetic dystrophy in children. J Bone Jt Surg (Am) 1992;74:910–919. [3] Jones HR Jr, Felice KJ, Gross PT. Pediatric peroneal mononeuropathy: a clinical and electromyographic study. Muscle Nerve 1993;16:1167–1173. [4] Grundberg AB, Reagan DS.Compression syndromes in reflex sympathetic dystrophy. J Hand Surg (Am) 1991;16:731–736. [5] Monsivais JJ, Baker J, Monsivais D. The association of peripheral nerve compression and reflex sympathetic dystrophy. J Hand Surg (Br) 1993;18:337–338. [6] Veldman PH, Reynen HM, Arntz IE, Goris RJ. Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet 1993;342:1012–1016. [7] Bechter AM, Casten DR. Reflex sympathetic dystrophy: criteria for diagnosis and treatment. Anesthesiology 1955;16:994–1003. [8] Lightman HI, Pochaczevsky R, Aprin H, Ilowite NY. Thermography in childhood reflex sympathetic dystrophy. J Pediatr 1987;111:551– 555. [9] Goldsmith DP, Vivino FB, Eichenfeld AH, Athreya BH, Heyman S. Nuclear imaging and clinical features of childhood reflex neurovascular dystrophy: comparison with adults. Arthritis Rheum 1989;32:480–485.