- Compartment syndrome is one of the few orthopaedic emergencies
- Children with supracondylar humerus fractures, forearm fractures, and tibia fractures are at increased risk and should be monitored closely
- Agitation, anxiety, and an increase in analgesic needs may be the first signs of compartment syndrome in children
- Compartment syndrome is a clinical diagnosis and compartment measurements should only be performed in an obtunded patient or when the diagnosis is not clear
- With prompt diagnosis and treatment, good long term clinical results can be expected
Compartment syndrome is an orthopedic emergency. Elevated compartment pressure decreases perfusion, causing muscle necrosis and nerve ischemia. Prolonged ischemia can result in irreversible damage to muscles, nerves, and the skin. Compartment syndrome is a leading cause of medical malpractice lawsuits, with an unusually high percentage settled for the plaintiff (Bhattacharyya, 2004; Prasarn, 2009). Identifying high-risk patients and prompt diagnosis and treatment are critical. Diagnosis can be particularly difficult in young children, who may have associated anxiety, inability, or unwillingness to cooperate with an exam. This inability to communicate effectively can make timely diagnosis challenging. Additionally, patients may be admitted to general pediatric floors, whose staff may not be trained to identify patients with increased intra-compartmental pressures. Physicians taking care of children should be aware of the unique features of compartment syndrome in children and be able to identify patients at risk who will benefit from close monitoring.
Compartment syndrome may occur in any area where the skeletal muscle is surrounded by a layer of fascia. It occurs most commonly in the leg, forearm, hand, and foot.
Most orthopedic surgeons and residents understand the "5 Ps" of compartment syndrome (pain, paresthesia, paralysis, pallor, and pulselessness). Pain out of proportion and pain with passive stretch are usually the first signs of increased intra-compartmental pressure in adults. However, examining an anxious frightened young child for pain with passive stretch can be very difficult and quantifying the amount of pain is not reliable in young children who may not be able to communicate effectively. In a report of 33 children with compartment syndrome the 5 Ps were found to be relatively unreliable for diagnosis of compartment syndrome in children (Bae, 2001). They found that increasing analgesic requirement was documented on average 7.3 hours before a change in the vascular status and was a more sensitive indicator of compartment syndrome in children than the traditional 5 Ps. Children at risk for compartment syndrome should be closely monitored for the "3 As": increasing anxiety, agitation, and analgesic requirement (Noonan, 2010). Nursing documentation of the patient’s analgesic use can be helpful to make an early diagnosis. Injudicious use of regional anesthesia may mask the primary symptom (pain) of compartment syndrome (Mubarak, 1997; Price, 1996). Therefore, use of regional anesthetic techniques for patients at high risk for compartment syndrome should be discouraged. Although compartment syndrome is a clinical diagnosis, measurement of compartment pressure can be helpful in certain clinical scenarios. Measuring compartment pressure in an obtunded child or a child with a communication disability can help confirm or rule out this diagnosis.
Normal compartment pressures in children are higher than normal pressures in adults. Staudt et al measured pressure in four lower leg compartments in 20 healthy children and 20 healthy adults (Staudt, 2008). On average pressures in 4 compartments varied between 13.3 mm Hg and 16.6 mm Hg in children and between 5.2 mm Hg and 9.7 mm Hg in adults. Compartment pressures decrease with increasing distance from fracture site, hence measurements should be taken within 5 cm of the fracture site to ensure accuracy (Heckman, 1994).
The pressure threshold that mandates fasciotomy is debatable. Absolute pressures of 30-45 mmHg have been suggested as indications. Calculating the diastolic blood pressure minus the compartment pressure as greater than 30 mmHg (?P) has also been recommended by several authors (Hargens, 1981; Heppenstall, 1988; McQueen, 1996). Because normal compartment pressures are higher in children, these values cannot be used as reliable standards in children. Direct measurement of intracompartmental pressure using a needle and catheter is invasive and can be difficult in children. Near infrared spectroscopy is a noninvasive method that has potential utility to diagnose increased compartment pressures (Shuler, 2010; Tobias, 2007). This method uses differential light absorption properties of oxygenated hemoglobin to measure tissue ischemia, similar to the method used in pulse oximeter. In contrast to pulse oximeter, near infrared spectroscopy is able to sample deeper tissue (3 cm below the skin level). Shuler et al reported on near infrared spectroscopy findings of fourteen adult patients with acute compartment syndrome. They found that lower tissue oxygenation levels correlated with increased intra-compartmental pressures but were not able to define a cutoff value for which near infrared spectroscopy measurements would indicate significant tissue ischemia. Use of this method to diagnose compartment syndrome in children has been reported (Tobias, 2007).
Compartment syndrome remains a clinical diagnosis. Clinicians should monitor high-risk patients and closely monitor their analgesic use. Compartment measurements should be interpreted with caution, as the cut off values may be different in children than adults. The literature fails to support treatment of compartment syndrome based solely on compartment pressure measurements.
Radiographs of the effected extremity are routinely obtained when evaluating fractures, but do not play a specific role in the diagnosis of elevated compartment pressures. They may be useful to plan the site of compartment measurement.
Traumatic injuries are responsible for the majority of cases of compartment syndrome. Although all children with fractures should be monitored for signs and symptoms of compartment syndrome, certain injuries are at higher risk for developing compartment syndrome.
Supracondylar humerus fractures
Compartment syndrome may develop in 0.1% to 0.3% of children presenting with supracondylar humerus fractures (Battaglia, 2002) (Ramachandran, 2008). Elbow flexion in casts beyond 90 degrees and vascular injuries increase the risk. Mubarak et al reported 9 cases of volar forearm compartment syndrome. The majority were associated with elbow flexion past 90 degrees after closed reduction (Mubarak, 1979). Battaglia et al measured compartment pressures in 29 children with supracondylar humerus fractures and reported the highest pressures in the deep volar compartment close to the fracture site. They also reported increased pressures when elbow was flexed more than 90 degrees (Battaglia, 2002). Although compartment syndrome associated with supracondylar humerus fractures is mostly seen in volar compartment of the forearm, compartment syndrome of the mobile wad, anterior arm compartment, and posterior arm compartment are also reported in children (Diesselhort, 2014; Mai, 2011). Choi et al reported two cases of compartment syndrome in patients who presented with pulseless, poorly perfused hands. There were no cases of compartment syndrome in children with pulseless but well perfused hands ("pink, pulseless") (Choi, 2010). Recent reports indicate that the time from injury to surgery does not change the rate of compartment syndrome in Type III supracondylar fractures (Gupta, 2004; Iyengar, 1999; Leet, 2002; Mehlman, 2001). However, most of the patients in these studies were neurovascularly intact, and it should be emphasized that the authors did not recommend purposely delaying treatment in patients with severe injuries, including excessive bruising and swelling, skin dimpling, neurological deficits, or absent radial pulses. Compartment syndrome has been reported in patients with low energy injuries and intact radial pulses, but with severe swelling and a mean delay of 22 hours (range 6-64) before surgery (Ramachandran, 2008). Based on available data and expert consensus, we recommend urgent or emergent treatment of children with supracondylar humerus fractures with excessive bruising and swelling, skin dimpling, neurological deficits or absent radial pulses. Patients should be closely monitored for signs of compartment syndrome before and after surgery.
Compartment syndrome has been reported in children with ipsilateral humerus and forearm fractures. Blakemore et al reported a 33% rate of compartment syndrome in children with displaced distal humerus and forearm fractures (Blakemore, 2000). Another retrospective review of 16 children with floating elbow injuries reported 2 acute compartment syndromes and 4 delayed compartment syndromes among the 10 patients who were treated by closed reduction and plaster casting of the forearm fracture. No signs of compartment syndrome were seen in patients who underwent stabilization of both the distal humerus and forearm fractures with K-wires (Ring, 2001). We recommend against circumferential casting to treat forearm fractures in children presenting with floating elbow injuries.
Compartment syndrome is extremely rare in children with forearm fractures who are treated with closed manipulation and casting or splinting. However, it is more common in children with open forearm fractures and fractures treated by closed reduction and intramedullary nailing (Haasbeek, 1995; Flynn, 2010; Blackman, 2014). Prolonged closed manipulation of fractures during surgery is associated with an increased risk of compartment syndrome (Yuan, 2004). A low threshold for open reduction during forearm fracture nailing is advisable to avoid excessive swelling from prolonged manipulation. We recommend close monitoring of all children treated operatively for forearm fractures.
Children with tibia fractures, especially those sustained in motor vehicle accidents, are at risk for compartment syndrome. Hope at al reported compartment syndrome in 4 (4%) of ninety-two children presenting with open tibia fractures (Hope, 1992). A recent report on compartment syndrome of the leg in children showed an average delay of 20.5 hours from injury to diagnosis, which may indicate slower development of compartment syndrome in children or difficulty in making the diagnosis (Flynn, 2011). Tibial tubercle fractures are at increased risk due to potential associated injury of the recurrent anterior tibial artery (Pandya, 2012). We recommend close monitoring of children who present with high energy tibial shaft fractures or tibial tubercle fractures. Mubarak reported 6 patients with distal tibial physeal fractures who presented with severe pain and swelling of the ankle, hypesthesia of the first web space, weakness of extensor hallucis longus and extensor digitorum communis, and pain on passive flexion of the toes (Mubarak, 2002). The intramuscular pressure measured more than 40 mmHg beneath the extensor retinaculum and less than 20 mmHg in anterior compartment in each of these patients. All had prompt relief of pain and improved sensation and strength within 24 hours after release of the superior extensor retinaculum and stabilization of fractures.
Compartment syndrome after femur fractures is uncommon. Compartment syndrome and Volkmann's ischemic contracture has been reported after 90/90 spica casting for treatment of femur fractures in children (Mubarak, 2006). The authors recommended against 90/90 spica casting of femur fractures, in favor of a technique that includes 45° of flexion at the hip and 45° of flexion at the knee, wrapping the body and leg portion of the cast to the below-knee level at the same time, and avoiding traction that places pressure in the popliteal fossa.
Miscellaneous and non-traumatic causes of compartment syndrome: Neonatal compartment syndrome is very rare and difficult to diagnose. It may be caused by a combination of low neonatal blood pressure and birth trauma (Macer, 2006). Ragland et al reported 24 cases of neonatal compartment syndrome and the diagnosis was made within 24 hours in only 1 patient (Ragland, 2005). They described a “sentinel skin” lesion on the forearm of these patients as the only initial sign of the compartment syndrome. Late diagnosis may result in contractures and growth arrest of the involved extremity. In their series, only 1 patient had a fasciotomy within 24 hours and resulted in a good functional outcome. Function was impaired in the other 23 cases. High clinical suspicion is the key to early diagnosis and treatment for these patients.
Medical problems causing intra compartmental bleeding (liver failure, renal failure, hemophilia, or leukemia) may cause compartment syndrome (Alioglu, 2006; Lee, 2011). Limb ischemia and subsequent reperfusion may also cause compartment syndrome. Correction of coagulation defects may take priority over surgical treatment in these cases, although this decision should be made on a case-by-case basis (Alioglu, 2006).
Snakebites can cause compartment syndrome in children, especially if the fangs penetrate through the fascia. Shaw et al reported successful use of antivenin in prevention of surgical treatment in 16 of 19 patients with rattlesnake bites (Shaw, 2002). In their report, two patients had limited surgical debridement and only one patient underwent fasciotomy for compartment syndrome. The authors recommended use of antivenin in snakebites in children to prevent compartment syndrome (Shaw, 2002).
Iatrogenic compartment syndrome has been reported due to IV infiltration and retained phlebotomy tourniquets. This is especially common in obtunded patients and those treated in intensive care units. Delayed diagnosis is common in this situation, leading to a higher than normal rate of extremity amputation (Prasarn, 2009).
When compartment syndrome is suspected, circumferential dressings should be split and casts should be bi-valved. If the clinical diagnosis of compartment syndrome is made, emergent fasciotomy and decompression is indicated. Treatment of the etiology of compartment syndrome should also be kept in mind while planning the fasciotomy. Treatment of clotting deficiencies in cases caused by excessive bleeding, fracture fixation, and vascular repair may be indicated while performing the fasciotomy and decompression. In the forearm, a volar incision is used to decompress the superficial and deep compartments as well as the carpal tunnel. Dorsal decompression may be needed if the mobile wad and dorsal compartments are involved. In the leg, all four compartments (anterior, lateral, superficial, and deep posterior) should be fully decompressed. This can be achieved with either a two incision (medial and anterolateral) or one incision (lateral) technique. Delayed primary skin closure and split thickness skin grafting are commonly used for closure. Vacuum assisted closure can be used prior to final closure, and may minimize the need for skin grafting (Yang, 2006). ?
Flynn et al reported on the outcome of forty-three cases of acute compartment syndrome of the leg in children from two pediatric trauma centers (Flynn, 2011). The average time from injury to fasciotomy was 20.5 hours (3.9 to 118 hours). Functional outcome was excellent at the time of follow up in forty-one patients. The two patients that lost function had their fasciotomies performed more than 80 hours after their injuries. Despite a long period from injury to surgery in many cases, excellent results were achieved with fasciotomy in most patients. Bae et al reported the outcomes of 33 children with compartment syndrome of upper and lower extremity treated with fasciotomies. They reported that full functional recovery was achieved in 30 of 33 cases (91%) by an average of 2.5 months following surgery (Bae, 2001).
A missed compartment syndrome can have devastating complications such as chronic pain, irreversible muscle and nerve damage, fixed contractures, and the possible need for amputation. The best method for avoiding complications is to maintain a high level of suspicion for compartment syndrome and to intervene early.
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Pooya Hosseinzadeh, MD
Vishwas Talwalkar, MD
Jenifer Powers, MD