- Email: [email protected]
Forearm Compartment Syndrome in the Newborn: Report of 24 Cases
Forearm Compartment Syndrome in the Newborn: Report of 24 Cases Raymond Ragland III, MD, Didier Moukoko, MD, Marybeth Ezaki, MD, Peter R. Carter, MD, Janith Mills, PA-C, Dallas, TX
Purpose: Isolated cases of ischemia, compartment syndrome, or Volkmann’s ischemic contracture in the forearm of the newborn infant have been reported in the past. The purpose of this study is to review a large series of patients with neonatal forearm compartment syndrome and to report the important clinical features. Methods: A search of medical records from 1980 to 2000 identified 24 children with evidence of ischemia of the forearm at the time of birth. Records and images were reviewed for prenatal and birth history, maternal factors, medical conditions, pattern of involvement, treatment, and outcomes. Patients were grouped according to the extent of initial soft-tissue involvement. Results: All patients presented with a sentinel forearm skin lesion. Patterns of involvement ranged from mild skin and subcutaneous lesions to dorsal and volar compartment syndrome with or without distal tissue loss. Early treatment intervention was limited to a single case in which the diagnosis of compartment syndrome was made and an emergency fasciotomy was performed with a good outcome. In other cases tissue loss, compressive neuropathy, muscle loss, and late skeletal changes were responsible for impaired function. Distal bone growth abnormality was common. Conclusions: Forearm compartment syndrome in the newborn is not as uncommon as previously thought. The skin lesion was the common, salient, initial diagnostic finding. Early diagnosis and appropriate referral led to the salvage of a functional limb in 1 of the patients in this series. The severity of the initial insult correlated with the degree of impairment in growth and function. The delayed diagnosis and treatment of an evolving compartment syndrome may compromise further final function. (J Hand Surg 2005;30A:997–1003. Copyright © 2005 by the American Society for Surgery of the Hand.) Key words: Skin lesion, newborn, forearm, compartment syndrome, Volkmann’s ischemic contracture.
Case reports of neonatal forearm compartment syndrome have appeared in the literature under a variety of names, usually depending on the bias of the au-
From the Texas Scottish Rite Hospital for Children, Dallas, TX. Received for publication May 14, 2003; accepted in revised form May 11, 2005. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Corresponding author: Marybeth Ezaki, MD, Department of Orthopaedic Surgery, Hand Surgery Service, Texas Scottish Rite Hospital for Children, 2222 Welborn St, Dallas, TX 75219; e-mail: marybeth.ezaki @tsrh.org. Copyright © 2005 by the American Society for Surgery of the Hand 0363-5023/05/30A05-0017$30.00/0 doi:10.1016/j.jhsa.2005.06.003
thor. Cantaboni and Taveggia1 believed this represented a form of cutis aplasia and Lightwood2 called it neonatal sclerema; Hensinger,3 Tsur et al,4 Caouette-Laberge et al,5 Leauté-Labrèze et al,6 Henssge and Linka,7 Perricone and Granata,8 Silfen et al,9 Kline and Russell Moore,10 Tsujino and Hooper,11 and Cham et al12 recognized vascular compromise and compartment syndrome as the basis for the condition of the soft tissue. Rombouts et al13 reported on the neurologic findings and related them to compression palsy. The combined number of cases of these 13 investigators totals 17. Skin lesions or open wounds are unusual findings in the newborn,14 but when found on the forearm a The Journal of Hand Surgery
997
998
The Journal of Hand Surgery / Vol. 30A No. 5 September 2005
skin lesion may be an indicator of concomitant neonatal compartment syndrome and tissue ischemia. The spectrum of presentation varies and may range from a local skin lesion to a swollen forearm to distal gangrene. The skin lesion was present in most reported cases1,3– 8,10,12,14 and in all of our cases, but the association of a skin lesion and underlying compartment syndrome often is not appreciated and emergency fasciotomy rarely has been performed.15 The skin lesion is obvious and often is the focus of treatment in the nursery. This lesion, when small, usually heals with minimal treatment and has trivial long-term consequences. If an underlying compartment syndrome is left untreated, however, it will evolve into a Volkmann’s contracture with a combination of extrinsic muscle infarction, intrinsic muscle paralysis, and loss of hand sensibility. Treatment of an end-stage ischemic contracture will involve salvage reconstructive procedures. Because the insult occurs at such an early age, physeal involvement is likely to result in altered growth of the extremity.5 This study reviewed a large series of patients with neonatal forearm compartment syndrome and reports the important clinical features.
Materials and Methods With permission of the institutional review board we identified 24 patients with the diagnosis of forearm ischemia who presented with acute or evolving ischemic contractures or with contractures, neuropathy, or growth insult related to a neonatal ischemic event. These patients were studied retrospectively by chart and x-ray review. Twenty patients were from our institution and were treated by 1 or both of the senior authors (M.E., P.R.C.). Four additional patients were added to this series by 4 other surgeons. Available records were reviewed for gestational and perinatal history, degree of soft-tissue involvement, skeletal involvement and growth alteration of the limb, nerve injury, early surgical intervention, late treatment, and reconstructive surgery. Nerve involvement and residual deficit were assessed with sensory and motor testing when possible. Each patient’s range of motion and ability to use the limb were documented. We saw 10 infants in the first 3 months and the size of the skin lesion could be assessed directly. Photographs of each infant’s limb on the day of birth were available in an additional 3 patients to judge the size and location of the skin lesion. The parents were asked about the initial diagnosis and treatment that was offered. Muscle involvement was assessed by palpation
Table 1. Associated Neonatal Conditions Condition Maternal varicella infection Maternal diabetes Gestational Pre-existing, insulin-dependent Fetal distress during delivery (5 cases) Placental disruption Placental insufficiency Premature deliveries 31 weeks (twins) 33 weeks (twins) 35 weeks Difficult vaginal delivery Transverse lie requiring cesarean section Respiratory insufficiency Cardiac murmur Polycystic kidney Ischemic encephalopathy Gastroesophageal reflux disorder Radial head dislocation Cutis aplasia in sibling Coagulation abnormality Abnormal coagulation profile Increased maternal lipoprotein A levels
Number of Patients 2 2 1 1 2 1 1 3
2 2 2 1 1 1 1 1 1 3 2 1
and by contracture identified by a tenodesis effect on digital motion by wrist position, loss of active contraction, and in some cases by direct visualization at surgery. The diagnosis was made in the late cases by the residual findings of compartment ischemia and/or skeletal growth changes combined with evidence of a coexisting skin lesion at birth. Bone growth abnormalities were assessed by clinical comparison with the normal opposite limb both with physical examination and radiographs.
Results There were 17 boys and 7 girls. The right upper extremity was involved in 13 patients and the left upper extremity was involved in 11 patients. None of the patients had bilateral involvement or a similar lesion in a lower limb or opposite upper limb. All identified conditions are listed in Table 1. There was no obvious cause, trend, or commonality of the associated conditions. Only 1 patient was evaluated and treated by a hand surgeon during the first 24 hours. An additional 13 patients first were seen during the first year (5 patients seen in the first month, 5 patients seen in the second and third months, 3 patients seen between the third and twelfth months), and the remaining 10
Ragland III et al / Forearm Compartment Syndrome in the Newborn
999
Figure 1. (A) Appearance of the involved limb in the delivery room. (B) Appearance of the limb and dorsal eschar at 2 weeks of age.
patients first were seen between the ages of 1 and 13 years (average age, 6.5 y).
Gestational and Perinatal History There were no cases of oligohydramnios, no reported uterine abnormalities, and no cases with an amnion disruption sequence. Other reported gestational and perinatal conditions are shown in Table 1. Although there are no patterns to the abnormalities found in this small group of patients, a number of the reported conditions suggest the potential for difficulty during the immediate prepartum time period.
Degree of Soft-Tissue Involvement All patients had a skin lesion. In 20 of the 24 patients the lesion was described on the day of birth as bullous or ulcerative with distal edema (Fig. 1). One child’s skin lesion was noted and first documented on day 3. Three mothers could not remember the exact appearance of the lesion. The location of the lesion typically involved the dorsal forearm, wrist, and/or hand. No mirror skin lesion indicating pressure on another body part was seen in any patient. The size of the skin lesion varied from 1 cm2 to those that involved the entire forearm and hand (Fig. 2). The depth of muscle involvement was used to classify the patients into 4 clinical groups. Group 1 comprised 7 patients who had evidence of skin involvement without compartment ischemia or muscle dysfunction, but all had late evidence of dysvascular skeletal development (Fig. 3). Group 2 comprised 7 patients who had a skin lesion with late extrinsic contractures involving only the superficial muscles of the dorsal compartment. Group 3 comprised 3 patients who had skin lesions associated with either a complete flexor or extensor compartment involvement. Group 4 comprised 7 patients who had both
flexor and extensor compartment involvement and often had associated distal tissue loss of the fingers or hand (Fig. 4). Gangrenous fingertips with tissue loss were seen in 3 patients. One patient had a gangrenous hand. These patients required formal amputation during the neonatal period. Three patients required later resurfacing procedures as part of contracture release, neurolysis, or in preparation for lengthening. Three of the group 4 patients had Doppler/sonographic studies that indicated proximal arterial thrombosis: 2 at the brachial level and 1 at the axillary level. One patient had dysvascular tips of 2 fingers that resolved completely after fasciotomy at 3 hours of age.
Skeletal Involvement Bone changes were present in 18 of 24 patients. Bone changes were widespread throughout all 4 groups and were not confined to only those with severe muscle involvement. Skeletal changes became more evident as the children grew and limb length discrepancies became more obvious. The characteristic findings were distortion of multiple physes with widen-
Figure 2. Appearance at 1 week of age with skin lesion and early demarcation of ischemic tissue.
1000
The Journal of Hand Surgery / Vol. 30A No. 5 September 2005
Figure 3. (A) Child with superficial scarring but no extrinsic flexion contractures. (B) Same child demonstrating no extrinsic extension contractures.
ing, flaring, premature closure, angular deformity, and shortening. The effects on skeletal growth varied from mildly shortened digits to a premature closure of the distal radial growth plate (Fig. 5). The cause of the physeal insult remains unclear.
Nerve Lesions Nerve involvement was present in 16 of 24 patients (Table 2) at the time of the initial evaluation. Only 4 patients spontaneously recovered nerve function. One infant had decompression of the posterior interosseous nerve at 3 weeks of age and recovered active finger extension. Nerve dysfunction persisted in the other 11 patients.
Early surgical intervention. One patient was treated with emergency fasciotomy 3 hours after delivery. Before fasciotomy marked cyanosis of 2 fingertips was present in association with a tight swollen forearm (Fig. 6). At the 1-year follow-up evaluation this patient had full use of what appeared to be a normal hand on the affected side. (Case of Charles Hamlin, M. D., Denver, Colorado.)
One additional patient had late fasciotomy at 3 weeks of age to decompress nerves but this failed to avert the need for transmetacarpal amputation later.
Late treatment: reconstructive surgery. Late reconstructive treatment for established Volkmann’s contracture is well documented in the literature.16 Fifteen of our patients had 19 late reconstructive procedures including neurolysis, debridement of infarcted muscle, release of contractures, soft-tissue resurfacing, correction of angular deformity, lengthening, and staged reconstruction of flexor and extensor function using either tendon transfer or free neurotized gracilis muscle transplantation. The timing of these procedures depended on the age at presentation, size of the limb, and surgical goals.
Discussion Forearm compartment syndrome has been reported in the literature under multiple names and has been ascribed to multiple causes. The presence of a skin lesion was noted in the reported patients, but the importance of this as a sentinel lesion of compart-
Figure 4. (A) Severe involvement of both flexor and extensor compartments with no distal tissue loss. (B) Both compartments involved with loss of distal tissue.
Ragland III et al / Forearm Compartment Syndrome in the Newborn
1001
Table 2. Nerve Palsy Nerve Involved Radial nerve (posterior interosseous) Ulnar nerve Median nerve Combined (radial and ulnar) Combined (median and ulnar) Combined (median, radial, and ulnar)
Figure 5. (A) Skeletal changes in a young child showing physeal damage and ischemic loss of distal tissue. (B) Adolescent with shortening and premature closure of multiple metacarpals and phalanges and alteration in distal radial physis.
ment syndrome usually was not recognized. The presence of a skin lesion in all of our patients makes a strong argument that this finding should not be treated lightly. The term lesser ischemic contracture of the upper limb has been used in cases that did not involve the entire limb.9 There did appear to be a correlation between the size of the skin lesion and the severity of the ischemia and functional outcome. Larger or circumferential lesions were associated with greater tissue loss and poorer function.
Possible Cause of the Condition The exact pathologic mechanism is unknown. Both extrinsic and intrinsic factors have been implicated. Whether the initial cause is intrinsic or extrinsic the end result is an increased compartment pressure. Proposed extrinsic causes include mechanical com-
Number of Patients 3 2 2 2 3 4
pression from combined effects of fetal posture and oligohydramnios,17,18 umbilical cord loops,8 amniotic band constriction,19 or direct birth trauma.17,18 None of our patients had clear evidence of any of these inciting conditions. There were no patients in whom a mirror lesion (implying 2-point compression of the extremity against another body part) was found, nor were any of our patients born to mothers with known uterine anomalies. There were no patients with fracture or injury to the brachial plexus during delivery and no other types of associated birth trauma among our patients. The appearance of the skin wound does place the event in the peripartum period. Intrinsic theories include a possible hypercoagulable state of the neonate20 leading to intra-arterial/ intravenous thrombosis.21–23 The relationship between the skin lesion and the underlying muscle ischemia is explained more readily by an extrinsic compression theory than by an intrinsic cause; however, the late effects of angular deformity and physeal closure distal to the lesion in the proximal forearm are explained less easily by a localized
Figure 6. (A) Appearance of forearm and hand at birth. (B) Palmar view of forearm and hand. Note fingertip ischemia just prior to emergency fasciotomy. (C) Appearance of hand at age 1 year.
1002
The Journal of Hand Surgery / Vol. 30A No. 5 September 2005
compressive event. The concept of an angiosome (an end-artery vascular supply of muscle and the overlying areas of skin) has been studied extensively by Taylor and Palmer.24 A large or small vascular event as the primary insult has been proposed as responsible for the related clinical findings of early skin lesion and muscle ischemia and also may explain the late skeletal changes caused by nutrient, periosteal, or epiphyseal arterial disruption. An alteration in procoagulant factors or diminished levels of anticoagulant has been reported.25 The few reports of neonates with hypercoagulability and compartment syndrome of the upper limb usually proceed rapidly to gangrene but reportedly have been treated successfully with heparin, thrombolytics, or thrombectomy.26,27 Thrombosis of larger named vessels also has been considered as in factor C or S deficiency, and 3 of our patients had sonographic studies that suggested evidence of thrombosis of a major artery (2 in the brachial artery, 1 in the axillary artery). Two of these 3 patients had laboratory findings suggestive of a coagulopathy. One had a positive family history of thrombophilia and increased levels of maternal lipoprotein A. Another had an initial protein C deficiency as a newborn that resolved by age 4. It is not clear if this was the primary mechanism or secondary retrograde thrombosis. These 3 patients had more extensive purpura and more extensive involvement of the extremity but all had the sentinel skin lesion. Whether these findings are indicative of a cause or are secondary alterations in laboratory studies remains a subject for further study. It has been proposed that a subclinical, in utero infection could initiate an inappropriate coagulation cascade.21,28 None of our patients exhibited signs of systemic infection. The diagnosis of cutis aplasia was made mistakenly in some of our patients, which again focused on the skin lesion while ignoring the important deeper pathology. True cutis aplasia is a specific diagnosis of localized disruption in skin development that presents at birth with eroded, absent, or scarred areas of skin.27,29 The condition is found most frequently on the scalp. When it has been reported in the literature the so-called cutis aplasia in the upper extremity has been noted to be the sentinel of underlying soft-tissue pathology.1,3,5,6,8,19 This is consistent with the 24 patients reported here and in 16 similar patients described in various case reports and smaller series in the literature.1– 6,8,10,20,30 We believe the diagnosis of cutis aplasia is confusing when it is applied to forearm skin lesions and that it
is best reserved for the classic scalp involvement. One patient in this series did have a sibling with the diagnosis of cutis aplasia of the scalp. Whether this finding is related by cause, genetic susceptibility, or merely coincidence is not known.
Recommendations for Treatment Recently reported cases refer to this condition as neonatal Volkmann’s syndrome because at the time of presentation many of these children show contractures and nerve changes.4 – 8 The term compartment syndrome in the newborn emphasizes the opportunity for early diagnosis and possibly treatment. Early recognition in the delivery room and urgent evaluation by a surgeon who can perform decompressive fasciotomy may help prevent the extent of tissue loss and the degree of functional impairment, especially in the milder cases, in which the possibility of reperfusion still may exist. The decision to perform surgery on an infant this small must not be taken lightly but should be based on appropriate risk assessment. In an evolving ischemic state caused by either intrinsic or extrinsic causes, intracompartmental pressures will increase and further compromise perfusion. Compartment pressure measurement in the neonate is not performed routinely and no standards for acceptable pressure gradients are available. Because the average diastolic pressure in a term newborn is 40 mm Hg or less,31 a very small increase in compartment pressure may impair muscle perfusion. As in the older patient with compartment syndrome, fasciotomy is the indicated procedure during the evolving phase of ischemia. Once the diagnosis is recognized a delay in decompression should be avoided. Although Doppler ultrasonography32 and angiography20 may provide diagnostic information, these modalities will delay treatment. Thrombolysis and thrombectomy also have been reported26,27 but these interventions do not decompress the forearm compartments and are unlikely to be effective in restoring arterial inflow and perfusion in the presence of increased compartment pressures. In only 1 patient in this series had a fasciotomy performed in the first hours after delivery. This was the only child who showed salvage of impending tissue loss. Wound care for children may include debridement or amputation of dysvascular tissue and local care for the skin lesion. Children who present later may require multiple staged procedures to debride infarcted muscle, decompress nerves, and reconstruct extrinsic muscle
Ragland III et al / Forearm Compartment Syndrome in the Newborn
function. Other procedures may be needed to address late bony deformity. The authors wish to thank the surgeons who contributed cases to this series: Charles Hamlin, MD, Denver, CO; Paul Manske, MD, St. Louis, MO; Paul Smith, FRCS, London, England; Guiseppe Ingrosso, MD, Milan, Italy.
References 1. Cantaboni A, Taveggia F. Aplasia cutis congenital of the upper extremity: presentation of two cases. Folia Hered Pathol 1966;15:101. 2. Lightwood R. Radial nerve palsy associated with localized subcutaneous fat necrosis in the newborn. Arch Dis Child 1951;26:436 – 437. 3. Hensinger RN. Gangrene of the newborn. A case report. J Bone Joint Surg 1975;57A:121–123. 4. Tsur H, Yaffe B, Engel Y. Impending Volkmann’s contracture in a newborn. Ann Plast Surg 1980;5:317–320. 5. Caouette-Laberge L, Bartoluzzi P, Egerszegi EP, Marton D. Neonatal Volkmann’s ischemic contracture of the forearm: a report of five cases. Plast Reconstr Surg 1992;90:621– 628. 6. Leauté-Labrèze C, Depaire-Duclos F, Sarlangue J, Fontan D, Sandler B, Maleville J, et al. Congenital cutaneous defects as complications in surviving co-twins. Aplasia cutis congenita and neonatal Volkmann’s ischemic contracture of the forearm. Arch Dermatol 1998;134:1121–1124. 7. Henssge J, Linka F. Volkmann’s contracture and constricting bandage. Beitr Orthop Traumatol 1968;15:27–28. 8. Perricone G, Granata C. Volkmann’s syndrome of the forearm in a newborn infant. Chir Organi Mov 1982;68:121– 125. 9. Silfen R, Amir A, Sirota L, Hauben DJ. Congenital Volkmann–Lesser ischemic contracture of the upper limb. Ann Plast Surg 2000;45:313–317. 10. Kline SC, Russell Moore J. Neonatal compartment syndrome. J Hand Surg 1992;17A:256 –259. 11. Tsujino A, Hooper G. Neonatal compression ischaemia of the forearm. J Hand Surg 1997;22B:612– 614. 12. Cham P, Drolet B, Segura A, Esterly N. Congenital Volkmann ischaemic contracture: a case report and review. Br J Dermatol 2004;150:357–363. 13. Rombouts JJ, Debauche C, Verellen G, Lyon G. Congenital paralysis due to compression. Apropos of four cases. Ann Chir Main Memb Super 1993;12:39 – 44. 14. Solomon LM, Esterly NB. Developmental Abnormalities— congenital absence of skin. In: Schaeffer A, ed. Neonatal Dermatology. Philadelphia: W.B. Saunders, 1973:49 –59. 15. Engel J, Heim M, Tsur H. Late complications of neonatal Volkmann’s ischaemia. Hand 1982;14:162–163.
1003
16. Littler JW. Principles of reconstructive surgery of the hand. In: Converse JM, McCarthy J, eds. Reconstructive plastic surgery. Principles and procedures in correction, reconstruction and transplantation. 2nd ed. Philadelphia: W.B. Saunders, 1977:3130 –3137. 17. Stavorovsky M, Iellen A, Spirer Z. Acute ischemia of the limb of a newborn treated successfully by thrombectomy. Am J Surg 1975;129:337–340. 18. Dunn PM. Congenital postural deformities. Br Med Bull 1976;32:71–76. 19. Pers M. Congenital absence of skin: pathogenesis and relation to ring-constriction. Acta Chir Scand 1963;126:388 – 396. 20. Armstrong AP, Page RE. Intrauterine vascular deficiency of the upper limb. J Hand Surg 1997;22B:607– 611. 21. Gross RE. Arterial embolism and thrombosis in infancy. Am J Dis Child 1945;70:61–73. 22. Fee HJ, McAvoy JM, Dainko EA. Neonatal arterial occlusion. J Pediatr Surg 1977;12:711–713. 23. Wisemann NE, Briggs JN, Bolton VS. Neonatal arterial occlusion with ischemic limb gangrene. J Pediatr Surg 1977; 12:707–711. 24. Taylor GI, Palmer JH. The vascular territories (angiosomes) of the body: experimental study and clinical applications. Br J Plast Surg 1987;40:113–141. 25. Giacoia GP. High dose urokinase therapy in newborn infants with major vessel thrombosis. Clin Pediatr 1993;32:231– 237. 26. Ricciardelli E, Morgan RF, Lin KY. In utero brachial artery thrombosis: limb salvage with postnatal urokinase infusion. Ann Plast Surg 1995;34:81– 83. 27. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol 1986;14: 646 – 660. 28. Christiansen SD, Desai NS, Pulito AR, Slack MR. Ischemic extremities due to compartment syndromes in a septic neonate. J Pediatr Surg 1983;18:641– 643. 29. Paltzik RL, Aiello AM. Aplasia cutis congenita associated with valvular heart disease. Cutis 1985;36:57–58. 30. Carr MM, Al-Qattan M, Clarke HM. Extremity gangrene in utero. J Hand Surg 1996;21B:652– 655. 31. Versmold H, Kitterman J, Phibbs R, Gregory G, Tooley W. Aortic blood pressure in the first twelve hours of life in infants with birth weight 610 to 4,220 grams. Pediatrics 1981;67:607–713. 32. Duncan BW, Adzick NS, Longaker MT, Edwards JR, Nelson RM, Koerper MA. In utero arterial embolism from renal vein thrombosis with successful postnatal thrombolytic therapy. J Pediatr Surg 1991;26:741–743.
Purpose: Isolated cases of ischemia, compartment syndrome, or Volkmann’s ischemic contracture in the forearm of the newborn infant have been reported in the past. The purpose of this study is to review a large series of patients with neonatal forearm compartment syndrome and to report the important clinical features. Methods: A search of medical records from 1980 to 2000 identified 24 children with evidence of ischemia of the forearm at the time of birth. Records and images were reviewed for prenatal and birth history, maternal factors, medical conditions, pattern of involvement, treatment, and outcomes. Patients were grouped according to the extent of initial soft-tissue involvement. Results: All patients presented with a sentinel forearm skin lesion. Patterns of involvement ranged from mild skin and subcutaneous lesions to dorsal and volar compartment syndrome with or without distal tissue loss. Early treatment intervention was limited to a single case in which the diagnosis of compartment syndrome was made and an emergency fasciotomy was performed with a good outcome. In other cases tissue loss, compressive neuropathy, muscle loss, and late skeletal changes were responsible for impaired function. Distal bone growth abnormality was common. Conclusions: Forearm compartment syndrome in the newborn is not as uncommon as previously thought. The skin lesion was the common, salient, initial diagnostic finding. Early diagnosis and appropriate referral led to the salvage of a functional limb in 1 of the patients in this series. The severity of the initial insult correlated with the degree of impairment in growth and function. The delayed diagnosis and treatment of an evolving compartment syndrome may compromise further final function. (J Hand Surg 2005;30A:997–1003. Copyright © 2005 by the American Society for Surgery of the Hand.) Key words: Skin lesion, newborn, forearm, compartment syndrome, Volkmann’s ischemic contracture.
Case reports of neonatal forearm compartment syndrome have appeared in the literature under a variety of names, usually depending on the bias of the au-
From the Texas Scottish Rite Hospital for Children, Dallas, TX. Received for publication May 14, 2003; accepted in revised form May 11, 2005. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Corresponding author: Marybeth Ezaki, MD, Department of Orthopaedic Surgery, Hand Surgery Service, Texas Scottish Rite Hospital for Children, 2222 Welborn St, Dallas, TX 75219; e-mail: marybeth.ezaki @tsrh.org. Copyright © 2005 by the American Society for Surgery of the Hand 0363-5023/05/30A05-0017$30.00/0 doi:10.1016/j.jhsa.2005.06.003
thor. Cantaboni and Taveggia1 believed this represented a form of cutis aplasia and Lightwood2 called it neonatal sclerema; Hensinger,3 Tsur et al,4 Caouette-Laberge et al,5 Leauté-Labrèze et al,6 Henssge and Linka,7 Perricone and Granata,8 Silfen et al,9 Kline and Russell Moore,10 Tsujino and Hooper,11 and Cham et al12 recognized vascular compromise and compartment syndrome as the basis for the condition of the soft tissue. Rombouts et al13 reported on the neurologic findings and related them to compression palsy. The combined number of cases of these 13 investigators totals 17. Skin lesions or open wounds are unusual findings in the newborn,14 but when found on the forearm a The Journal of Hand Surgery
997
998
The Journal of Hand Surgery / Vol. 30A No. 5 September 2005
skin lesion may be an indicator of concomitant neonatal compartment syndrome and tissue ischemia. The spectrum of presentation varies and may range from a local skin lesion to a swollen forearm to distal gangrene. The skin lesion was present in most reported cases1,3– 8,10,12,14 and in all of our cases, but the association of a skin lesion and underlying compartment syndrome often is not appreciated and emergency fasciotomy rarely has been performed.15 The skin lesion is obvious and often is the focus of treatment in the nursery. This lesion, when small, usually heals with minimal treatment and has trivial long-term consequences. If an underlying compartment syndrome is left untreated, however, it will evolve into a Volkmann’s contracture with a combination of extrinsic muscle infarction, intrinsic muscle paralysis, and loss of hand sensibility. Treatment of an end-stage ischemic contracture will involve salvage reconstructive procedures. Because the insult occurs at such an early age, physeal involvement is likely to result in altered growth of the extremity.5 This study reviewed a large series of patients with neonatal forearm compartment syndrome and reports the important clinical features.
Materials and Methods With permission of the institutional review board we identified 24 patients with the diagnosis of forearm ischemia who presented with acute or evolving ischemic contractures or with contractures, neuropathy, or growth insult related to a neonatal ischemic event. These patients were studied retrospectively by chart and x-ray review. Twenty patients were from our institution and were treated by 1 or both of the senior authors (M.E., P.R.C.). Four additional patients were added to this series by 4 other surgeons. Available records were reviewed for gestational and perinatal history, degree of soft-tissue involvement, skeletal involvement and growth alteration of the limb, nerve injury, early surgical intervention, late treatment, and reconstructive surgery. Nerve involvement and residual deficit were assessed with sensory and motor testing when possible. Each patient’s range of motion and ability to use the limb were documented. We saw 10 infants in the first 3 months and the size of the skin lesion could be assessed directly. Photographs of each infant’s limb on the day of birth were available in an additional 3 patients to judge the size and location of the skin lesion. The parents were asked about the initial diagnosis and treatment that was offered. Muscle involvement was assessed by palpation
Table 1. Associated Neonatal Conditions Condition Maternal varicella infection Maternal diabetes Gestational Pre-existing, insulin-dependent Fetal distress during delivery (5 cases) Placental disruption Placental insufficiency Premature deliveries 31 weeks (twins) 33 weeks (twins) 35 weeks Difficult vaginal delivery Transverse lie requiring cesarean section Respiratory insufficiency Cardiac murmur Polycystic kidney Ischemic encephalopathy Gastroesophageal reflux disorder Radial head dislocation Cutis aplasia in sibling Coagulation abnormality Abnormal coagulation profile Increased maternal lipoprotein A levels
Number of Patients 2 2 1 1 2 1 1 3
2 2 2 1 1 1 1 1 1 3 2 1
and by contracture identified by a tenodesis effect on digital motion by wrist position, loss of active contraction, and in some cases by direct visualization at surgery. The diagnosis was made in the late cases by the residual findings of compartment ischemia and/or skeletal growth changes combined with evidence of a coexisting skin lesion at birth. Bone growth abnormalities were assessed by clinical comparison with the normal opposite limb both with physical examination and radiographs.
Results There were 17 boys and 7 girls. The right upper extremity was involved in 13 patients and the left upper extremity was involved in 11 patients. None of the patients had bilateral involvement or a similar lesion in a lower limb or opposite upper limb. All identified conditions are listed in Table 1. There was no obvious cause, trend, or commonality of the associated conditions. Only 1 patient was evaluated and treated by a hand surgeon during the first 24 hours. An additional 13 patients first were seen during the first year (5 patients seen in the first month, 5 patients seen in the second and third months, 3 patients seen between the third and twelfth months), and the remaining 10
Ragland III et al / Forearm Compartment Syndrome in the Newborn
999
Figure 1. (A) Appearance of the involved limb in the delivery room. (B) Appearance of the limb and dorsal eschar at 2 weeks of age.
patients first were seen between the ages of 1 and 13 years (average age, 6.5 y).
Gestational and Perinatal History There were no cases of oligohydramnios, no reported uterine abnormalities, and no cases with an amnion disruption sequence. Other reported gestational and perinatal conditions are shown in Table 1. Although there are no patterns to the abnormalities found in this small group of patients, a number of the reported conditions suggest the potential for difficulty during the immediate prepartum time period.
Degree of Soft-Tissue Involvement All patients had a skin lesion. In 20 of the 24 patients the lesion was described on the day of birth as bullous or ulcerative with distal edema (Fig. 1). One child’s skin lesion was noted and first documented on day 3. Three mothers could not remember the exact appearance of the lesion. The location of the lesion typically involved the dorsal forearm, wrist, and/or hand. No mirror skin lesion indicating pressure on another body part was seen in any patient. The size of the skin lesion varied from 1 cm2 to those that involved the entire forearm and hand (Fig. 2). The depth of muscle involvement was used to classify the patients into 4 clinical groups. Group 1 comprised 7 patients who had evidence of skin involvement without compartment ischemia or muscle dysfunction, but all had late evidence of dysvascular skeletal development (Fig. 3). Group 2 comprised 7 patients who had a skin lesion with late extrinsic contractures involving only the superficial muscles of the dorsal compartment. Group 3 comprised 3 patients who had skin lesions associated with either a complete flexor or extensor compartment involvement. Group 4 comprised 7 patients who had both
flexor and extensor compartment involvement and often had associated distal tissue loss of the fingers or hand (Fig. 4). Gangrenous fingertips with tissue loss were seen in 3 patients. One patient had a gangrenous hand. These patients required formal amputation during the neonatal period. Three patients required later resurfacing procedures as part of contracture release, neurolysis, or in preparation for lengthening. Three of the group 4 patients had Doppler/sonographic studies that indicated proximal arterial thrombosis: 2 at the brachial level and 1 at the axillary level. One patient had dysvascular tips of 2 fingers that resolved completely after fasciotomy at 3 hours of age.
Skeletal Involvement Bone changes were present in 18 of 24 patients. Bone changes were widespread throughout all 4 groups and were not confined to only those with severe muscle involvement. Skeletal changes became more evident as the children grew and limb length discrepancies became more obvious. The characteristic findings were distortion of multiple physes with widen-
Figure 2. Appearance at 1 week of age with skin lesion and early demarcation of ischemic tissue.
1000
The Journal of Hand Surgery / Vol. 30A No. 5 September 2005
Figure 3. (A) Child with superficial scarring but no extrinsic flexion contractures. (B) Same child demonstrating no extrinsic extension contractures.
ing, flaring, premature closure, angular deformity, and shortening. The effects on skeletal growth varied from mildly shortened digits to a premature closure of the distal radial growth plate (Fig. 5). The cause of the physeal insult remains unclear.
Nerve Lesions Nerve involvement was present in 16 of 24 patients (Table 2) at the time of the initial evaluation. Only 4 patients spontaneously recovered nerve function. One infant had decompression of the posterior interosseous nerve at 3 weeks of age and recovered active finger extension. Nerve dysfunction persisted in the other 11 patients.
Early surgical intervention. One patient was treated with emergency fasciotomy 3 hours after delivery. Before fasciotomy marked cyanosis of 2 fingertips was present in association with a tight swollen forearm (Fig. 6). At the 1-year follow-up evaluation this patient had full use of what appeared to be a normal hand on the affected side. (Case of Charles Hamlin, M. D., Denver, Colorado.)
One additional patient had late fasciotomy at 3 weeks of age to decompress nerves but this failed to avert the need for transmetacarpal amputation later.
Late treatment: reconstructive surgery. Late reconstructive treatment for established Volkmann’s contracture is well documented in the literature.16 Fifteen of our patients had 19 late reconstructive procedures including neurolysis, debridement of infarcted muscle, release of contractures, soft-tissue resurfacing, correction of angular deformity, lengthening, and staged reconstruction of flexor and extensor function using either tendon transfer or free neurotized gracilis muscle transplantation. The timing of these procedures depended on the age at presentation, size of the limb, and surgical goals.
Discussion Forearm compartment syndrome has been reported in the literature under multiple names and has been ascribed to multiple causes. The presence of a skin lesion was noted in the reported patients, but the importance of this as a sentinel lesion of compart-
Figure 4. (A) Severe involvement of both flexor and extensor compartments with no distal tissue loss. (B) Both compartments involved with loss of distal tissue.
Ragland III et al / Forearm Compartment Syndrome in the Newborn
1001
Table 2. Nerve Palsy Nerve Involved Radial nerve (posterior interosseous) Ulnar nerve Median nerve Combined (radial and ulnar) Combined (median and ulnar) Combined (median, radial, and ulnar)
Figure 5. (A) Skeletal changes in a young child showing physeal damage and ischemic loss of distal tissue. (B) Adolescent with shortening and premature closure of multiple metacarpals and phalanges and alteration in distal radial physis.
ment syndrome usually was not recognized. The presence of a skin lesion in all of our patients makes a strong argument that this finding should not be treated lightly. The term lesser ischemic contracture of the upper limb has been used in cases that did not involve the entire limb.9 There did appear to be a correlation between the size of the skin lesion and the severity of the ischemia and functional outcome. Larger or circumferential lesions were associated with greater tissue loss and poorer function.
Possible Cause of the Condition The exact pathologic mechanism is unknown. Both extrinsic and intrinsic factors have been implicated. Whether the initial cause is intrinsic or extrinsic the end result is an increased compartment pressure. Proposed extrinsic causes include mechanical com-
Number of Patients 3 2 2 2 3 4
pression from combined effects of fetal posture and oligohydramnios,17,18 umbilical cord loops,8 amniotic band constriction,19 or direct birth trauma.17,18 None of our patients had clear evidence of any of these inciting conditions. There were no patients in whom a mirror lesion (implying 2-point compression of the extremity against another body part) was found, nor were any of our patients born to mothers with known uterine anomalies. There were no patients with fracture or injury to the brachial plexus during delivery and no other types of associated birth trauma among our patients. The appearance of the skin wound does place the event in the peripartum period. Intrinsic theories include a possible hypercoagulable state of the neonate20 leading to intra-arterial/ intravenous thrombosis.21–23 The relationship between the skin lesion and the underlying muscle ischemia is explained more readily by an extrinsic compression theory than by an intrinsic cause; however, the late effects of angular deformity and physeal closure distal to the lesion in the proximal forearm are explained less easily by a localized
Figure 6. (A) Appearance of forearm and hand at birth. (B) Palmar view of forearm and hand. Note fingertip ischemia just prior to emergency fasciotomy. (C) Appearance of hand at age 1 year.
1002
The Journal of Hand Surgery / Vol. 30A No. 5 September 2005
compressive event. The concept of an angiosome (an end-artery vascular supply of muscle and the overlying areas of skin) has been studied extensively by Taylor and Palmer.24 A large or small vascular event as the primary insult has been proposed as responsible for the related clinical findings of early skin lesion and muscle ischemia and also may explain the late skeletal changes caused by nutrient, periosteal, or epiphyseal arterial disruption. An alteration in procoagulant factors or diminished levels of anticoagulant has been reported.25 The few reports of neonates with hypercoagulability and compartment syndrome of the upper limb usually proceed rapidly to gangrene but reportedly have been treated successfully with heparin, thrombolytics, or thrombectomy.26,27 Thrombosis of larger named vessels also has been considered as in factor C or S deficiency, and 3 of our patients had sonographic studies that suggested evidence of thrombosis of a major artery (2 in the brachial artery, 1 in the axillary artery). Two of these 3 patients had laboratory findings suggestive of a coagulopathy. One had a positive family history of thrombophilia and increased levels of maternal lipoprotein A. Another had an initial protein C deficiency as a newborn that resolved by age 4. It is not clear if this was the primary mechanism or secondary retrograde thrombosis. These 3 patients had more extensive purpura and more extensive involvement of the extremity but all had the sentinel skin lesion. Whether these findings are indicative of a cause or are secondary alterations in laboratory studies remains a subject for further study. It has been proposed that a subclinical, in utero infection could initiate an inappropriate coagulation cascade.21,28 None of our patients exhibited signs of systemic infection. The diagnosis of cutis aplasia was made mistakenly in some of our patients, which again focused on the skin lesion while ignoring the important deeper pathology. True cutis aplasia is a specific diagnosis of localized disruption in skin development that presents at birth with eroded, absent, or scarred areas of skin.27,29 The condition is found most frequently on the scalp. When it has been reported in the literature the so-called cutis aplasia in the upper extremity has been noted to be the sentinel of underlying soft-tissue pathology.1,3,5,6,8,19 This is consistent with the 24 patients reported here and in 16 similar patients described in various case reports and smaller series in the literature.1– 6,8,10,20,30 We believe the diagnosis of cutis aplasia is confusing when it is applied to forearm skin lesions and that it
is best reserved for the classic scalp involvement. One patient in this series did have a sibling with the diagnosis of cutis aplasia of the scalp. Whether this finding is related by cause, genetic susceptibility, or merely coincidence is not known.
Recommendations for Treatment Recently reported cases refer to this condition as neonatal Volkmann’s syndrome because at the time of presentation many of these children show contractures and nerve changes.4 – 8 The term compartment syndrome in the newborn emphasizes the opportunity for early diagnosis and possibly treatment. Early recognition in the delivery room and urgent evaluation by a surgeon who can perform decompressive fasciotomy may help prevent the extent of tissue loss and the degree of functional impairment, especially in the milder cases, in which the possibility of reperfusion still may exist. The decision to perform surgery on an infant this small must not be taken lightly but should be based on appropriate risk assessment. In an evolving ischemic state caused by either intrinsic or extrinsic causes, intracompartmental pressures will increase and further compromise perfusion. Compartment pressure measurement in the neonate is not performed routinely and no standards for acceptable pressure gradients are available. Because the average diastolic pressure in a term newborn is 40 mm Hg or less,31 a very small increase in compartment pressure may impair muscle perfusion. As in the older patient with compartment syndrome, fasciotomy is the indicated procedure during the evolving phase of ischemia. Once the diagnosis is recognized a delay in decompression should be avoided. Although Doppler ultrasonography32 and angiography20 may provide diagnostic information, these modalities will delay treatment. Thrombolysis and thrombectomy also have been reported26,27 but these interventions do not decompress the forearm compartments and are unlikely to be effective in restoring arterial inflow and perfusion in the presence of increased compartment pressures. In only 1 patient in this series had a fasciotomy performed in the first hours after delivery. This was the only child who showed salvage of impending tissue loss. Wound care for children may include debridement or amputation of dysvascular tissue and local care for the skin lesion. Children who present later may require multiple staged procedures to debride infarcted muscle, decompress nerves, and reconstruct extrinsic muscle
Ragland III et al / Forearm Compartment Syndrome in the Newborn
function. Other procedures may be needed to address late bony deformity. The authors wish to thank the surgeons who contributed cases to this series: Charles Hamlin, MD, Denver, CO; Paul Manske, MD, St. Louis, MO; Paul Smith, FRCS, London, England; Guiseppe Ingrosso, MD, Milan, Italy.
References 1. Cantaboni A, Taveggia F. Aplasia cutis congenital of the upper extremity: presentation of two cases. Folia Hered Pathol 1966;15:101. 2. Lightwood R. Radial nerve palsy associated with localized subcutaneous fat necrosis in the newborn. Arch Dis Child 1951;26:436 – 437. 3. Hensinger RN. Gangrene of the newborn. A case report. J Bone Joint Surg 1975;57A:121–123. 4. Tsur H, Yaffe B, Engel Y. Impending Volkmann’s contracture in a newborn. Ann Plast Surg 1980;5:317–320. 5. Caouette-Laberge L, Bartoluzzi P, Egerszegi EP, Marton D. Neonatal Volkmann’s ischemic contracture of the forearm: a report of five cases. Plast Reconstr Surg 1992;90:621– 628. 6. Leauté-Labrèze C, Depaire-Duclos F, Sarlangue J, Fontan D, Sandler B, Maleville J, et al. Congenital cutaneous defects as complications in surviving co-twins. Aplasia cutis congenita and neonatal Volkmann’s ischemic contracture of the forearm. Arch Dermatol 1998;134:1121–1124. 7. Henssge J, Linka F. Volkmann’s contracture and constricting bandage. Beitr Orthop Traumatol 1968;15:27–28. 8. Perricone G, Granata C. Volkmann’s syndrome of the forearm in a newborn infant. Chir Organi Mov 1982;68:121– 125. 9. Silfen R, Amir A, Sirota L, Hauben DJ. Congenital Volkmann–Lesser ischemic contracture of the upper limb. Ann Plast Surg 2000;45:313–317. 10. Kline SC, Russell Moore J. Neonatal compartment syndrome. J Hand Surg 1992;17A:256 –259. 11. Tsujino A, Hooper G. Neonatal compression ischaemia of the forearm. J Hand Surg 1997;22B:612– 614. 12. Cham P, Drolet B, Segura A, Esterly N. Congenital Volkmann ischaemic contracture: a case report and review. Br J Dermatol 2004;150:357–363. 13. Rombouts JJ, Debauche C, Verellen G, Lyon G. Congenital paralysis due to compression. Apropos of four cases. Ann Chir Main Memb Super 1993;12:39 – 44. 14. Solomon LM, Esterly NB. Developmental Abnormalities— congenital absence of skin. In: Schaeffer A, ed. Neonatal Dermatology. Philadelphia: W.B. Saunders, 1973:49 –59. 15. Engel J, Heim M, Tsur H. Late complications of neonatal Volkmann’s ischaemia. Hand 1982;14:162–163.
1003
16. Littler JW. Principles of reconstructive surgery of the hand. In: Converse JM, McCarthy J, eds. Reconstructive plastic surgery. Principles and procedures in correction, reconstruction and transplantation. 2nd ed. Philadelphia: W.B. Saunders, 1977:3130 –3137. 17. Stavorovsky M, Iellen A, Spirer Z. Acute ischemia of the limb of a newborn treated successfully by thrombectomy. Am J Surg 1975;129:337–340. 18. Dunn PM. Congenital postural deformities. Br Med Bull 1976;32:71–76. 19. Pers M. Congenital absence of skin: pathogenesis and relation to ring-constriction. Acta Chir Scand 1963;126:388 – 396. 20. Armstrong AP, Page RE. Intrauterine vascular deficiency of the upper limb. J Hand Surg 1997;22B:607– 611. 21. Gross RE. Arterial embolism and thrombosis in infancy. Am J Dis Child 1945;70:61–73. 22. Fee HJ, McAvoy JM, Dainko EA. Neonatal arterial occlusion. J Pediatr Surg 1977;12:711–713. 23. Wisemann NE, Briggs JN, Bolton VS. Neonatal arterial occlusion with ischemic limb gangrene. J Pediatr Surg 1977; 12:707–711. 24. Taylor GI, Palmer JH. The vascular territories (angiosomes) of the body: experimental study and clinical applications. Br J Plast Surg 1987;40:113–141. 25. Giacoia GP. High dose urokinase therapy in newborn infants with major vessel thrombosis. Clin Pediatr 1993;32:231– 237. 26. Ricciardelli E, Morgan RF, Lin KY. In utero brachial artery thrombosis: limb salvage with postnatal urokinase infusion. Ann Plast Surg 1995;34:81– 83. 27. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol 1986;14: 646 – 660. 28. Christiansen SD, Desai NS, Pulito AR, Slack MR. Ischemic extremities due to compartment syndromes in a septic neonate. J Pediatr Surg 1983;18:641– 643. 29. Paltzik RL, Aiello AM. Aplasia cutis congenita associated with valvular heart disease. Cutis 1985;36:57–58. 30. Carr MM, Al-Qattan M, Clarke HM. Extremity gangrene in utero. J Hand Surg 1996;21B:652– 655. 31. Versmold H, Kitterman J, Phibbs R, Gregory G, Tooley W. Aortic blood pressure in the first twelve hours of life in infants with birth weight 610 to 4,220 grams. Pediatrics 1981;67:607–713. 32. Duncan BW, Adzick NS, Longaker MT, Edwards JR, Nelson RM, Koerper MA. In utero arterial embolism from renal vein thrombosis with successful postnatal thrombolytic therapy. J Pediatr Surg 1991;26:741–743.