Tag Archives: compartment syndrome

Compartment syndrome in a patient on warfarin with a ruptured Baker’s cyst

by Megan Wilder, DPM1; Kenneth Hegewald, DPM2; Thomas Landino, DPM3pdflrg

The Foot and Ankle Online Journal 7 (1): 4

Compartment syndrome of the lower leg is typically viewed as a surgical emergency. Elevated pressure within a closed myofascial space impairs perfusion pressure below a level necessary for muscle viability. It is caused by bleeding or edema in a closed, non-elastic muscle compartment surrounded by fascia and bone. We report the case of a 66-year-old patient on warfarin with acute compartment syndrome caused by hemorrhaging from a ruptured Baker’s cyst. The patient responded well to an emergent fasciotomy. The present case highlights the need for an awareness of acute compartment syndrome in patients on warfarin therapy and clinical symptoms consistent with compartment syndrome.

Key words: Baker’s cyst, compartment syndrome, lower extremity, warfarin

ISSN 1941-6806
doi: 10.3827/faoj.2014.0701.0004

Address correspondence to:Megan Wilder, DPM; Franciscan Foot and Ankle Institute, Federal Way, WA, Email: MeganWilder@fhshealth.org

1 Podiatric Medicine and Surgery Resident (Postgraduate Year I), Franciscan Foot and Ankle Institute, Federal Way, WA
2 Podiatric Medicine and Surgery Resident (Postgraduate Year 2), Franciscan Foot and Ankle Institute, Federal Way, WA
3 Attending Staff, Department of Orthopedics and Sports Medicine, Virginia Mason Medical Center, Federal Way, WA


Compartment syndrome of the lower leg is a surgical emergency in which elevated pressure within a closed myofascial space reduces circulation below a level necessary for muscle viability. It is caused by bleeding or edema in a closed, non-elastic muscle compartment surrounded by fascia and bone [1]. The long-term consequences were described by Volkmann in the 19th century following the application of casts [2]. Acute compartment syndrome is typically associated with fractures, closed soft tissue injuries, revascularization procedures, and crush injuries [3]. Anticoagulation has been suggested as risk factor in the development of acute compartment syndromes [4-6].

Extravasation of a ruptured Baker’s cyst and its damage on surrounding tissue has been linked to development of compartment syndrome [7,8]. However, we did not discover any cases where a Baker’s cyst with concomitant anticoagulation leading to compartment syndrome have been discussed.

Patient and Methods

A 66 year-old female presented to the Emergency department with two days of left lower extremity edema and pain that began suddenly and had continued to progress. Past medical history included: psoriatic arthritis, Baker’s cyst left lower extremity, left venous stripping, hypertension, GERD, and pulmonary embolism. The patient had a hardware removal from the right foot two weeks prior, but denied any other trauma or injury.

Examination revealed non-erythematous left calf measuring 40 cm in diameter (right calf measuring 33cm) with pain rated severe. Overlying skin was tense and unyielding compared to the contralateral limb. Sensation and pulses were normal. The patient had pain out of proportion with passive dorsiflexion and plantar flexion of the left ankle. Radiographs of the left lower extremity were negative for fracture revealing only arthritic changes.

1

Figure 1 MRI T2 Sagittal. The mass is centered in the proximal half of the left calf an extends 20.1 cm in length x7.7 cm transverse x4.4 cm AP and compresses the medial head of the gastrocnemius muscle with myositis evident in the proximal fibers of the muscle. Edematous changes are present over the posterior fascial plane at the interface of the fascial plane and subcutaneous fat.

Venous and arterial ultrasounds were performed and returned negative for deep venous thrombosis. Further ultrasound exam findings revealed an area of approximately 10 cm at the posterior left knee which was read as possible hematoma versus Baker cyst (Figures 1 and 2). With high clinical suspicion of compartment syndrome a wick catheter was used to measure intracompartmental pressures. The patient’s blood pressure was 139/70 mmHg Initial compartment pressures were read at: Anterior-12mmHg, Lateral-10mmHg, Deep Posterior-45 mmHg, Superficial Posterior-12mmHg. The deep posterior and superficial posterior compartment pressures were repeated and with consistent readings at 30mmHg and 12mmHg respectively.

2

Figure 2 MRI T2 Axial. The heterogeneity signal and characteristics of the mass as well fluid level are suspicious for an active hematoma adjacent to semimembranosus/popliteal cysts within the gastrocnemius bursa.

Given the unique presentation, a STAT MRI of the left lower extremity was completed. MRI findings revealed a complex large mass to the posteromedial proximal half of the calf with accompanying gastrocnemius muscle edema, suggestive of a complex hematoma with active bleeding (Figure 2). The characteristic enhancement suggested the possibility of a chronic popliteal cyst with intracystic hemorrhage. The mass appeared to be superficial to the muscle and compressed the muscle component. Also of note, the patient was on warfarin for a pulmonary embolism that had occurred 6 months previously. Patient’s laboratory values were: Prothrombin time 26.3, INR 2.4, partial thromboplastin time 41. The patient was taken to the operating room for emergent open fasciotomy of the compartments of the left lower extremity.

3

Figure 3 Intraoperative hemorrhaging and herniation of muscle belly upon release of the fascial compartments.

Through a standard anterolateral extensile approach all four-muscle compartments of the lower extremity were decompressed and hematoma was evacuated (Figure 3). The incision site was approximated using staples (Figure 4). Patient was placed in a modified Jones compression posterior splint. 1500 mL of blood loss occurred during the operation, requiring type and cross match blood transfusion of two units of packed red blood cells.

4

Figure 4 Incision re-approximated utilizing staples.

Results

Post-operatively the patient was transferred to the ICU where pain was improved immediately post-operatively. After a 6-day hospital stay the patient was deemed medically stable and released for outpatient treatment. The patient was taken off of warfarin and an IVC filter was placed. The patient had wound dehiscence of the distal aspect of the incision requiring local wound care of a 4-month duration.

Discussion

Few cases relating an acute onset of compartment syndrome to a Baker’s cyst or as a spontaneous occurrence with anticoagulation have been described [4-8]. Petros et al, reported the incidence of a ruptured Baker’s cyst misdiagnosed as a deep venous thrombosis, which was then treated with anticoagulation creating hemorrhaging and hematoma into the lower extremity compartment [7].

The risk of developing a compartment syndrome after a ruptured Baker’s cyst especially when associated with coagulopathy should be considered. An acute compartment syndrome is a medical emergency. Irreversible changes are known to occur after 8-12 hours of increased compartment pressure. Immediate evaluation should include compartment pressure measurements, and if elevated, surgical decompression. A fasciotomy should be performed when the difference between compartment pressure and diastolic blood pressure is less than 30 mmHg or when clinical symptoms are obvious [9].

In summary, we present a patient with a ruptured Baker’s cyst on long-term anticoagulation therapy with an INR in the therapeutic range complicated by the development of a posterior compartment syndrome.

Acknowledgements: Craig Clifford, DPM; Research Chair, Franciscan Foot and Ankle Institute.

References

1. Tiwari A, Haq AI, Myint F et-al. Acute compartment syndromes. Br J Surg. 2002;89 (4): 397-412. [Pubmed]
2. Volkmann R. Die ischämischen Muskellähmungen und Kontracturen. Zentralbl Chir. 1881;8:801–803.
3. Masquelet AC. Acute compartment syndrome of the leg: pressure measurement and fasciotomy. Orthop Traumatol Surg Res. 2010;96 (8): 913-7. [Pubmed]
4. Griffiths D, Jones DH. Spontaneous compartment syndrome in a patient on long-term anticoagulation. J Hand Surg Br. 1993;18 (1): 41-2. [Pubmed]
5. Gaines RJ, Randall CJ, Browne KL et-al. Delayed presentation of compartment syndrome of the proximal lower extremity after low-energy trauma in patients taking warfarin. Am J Orthop. 2008;37 (12): E201-4. [Pubmed]
6. Byrne AM, Kearns SR, Kelly EP. Posterior compartment syndrome associated with clopidogrel therapy following trivial trauma. Emerg Med J. 2006;23 (9): 697-8. [Pubmed]
7. Petros DP, Hanley JF, Gilbreath P et-al. Posterior compartment syndrome following ruptured Baker’s cyst. Ann Rheum Dis. 1990;49 (11): 944-5. [Pubmed]
8. Hamlet M, Galanopoulos I, Mahale A et-al. Ruptured Baker’s cyst with compartment syndrome: an extremely unusual complication. BMJ Case Rep. 2012. [Pubmed]
9. Frink M, Hildebrand F, Krettek C et-al. Compartment syndrome of the lower leg and foot. Clin Orthop Relat Res. 2010;468 (4): 940-50. [Pubmed]
10. Sinikumpu JJ, Lepojärvi S, Serlo W et-al. Atraumatic compartment syndrome of the foot in a 15-year-old female. J Foot Ankle Surg. 52 (1): 72-5. [Pubmed]

Medial Subtalar Dislocation of the Foot Associated with an Acute Compartment Syndrome: A Case Report

by Muzamil Ahmad Baba1, M. A Halwai2, B.A Mir3, Adil Bashir4, Mubashir Wani5pdflrg

The Foot and Ankle Online Journal 6 (6): 1

Subtalar dislocation an uncommon injury, accounts for approximately 1% to 2% of all joint dislocations. Subtalar dislocation, also known as peritalar dislocation, refers to the simultaneous dislocation of the distal articulations of the talus at the talocalcaneal and talonavicular joints. This case report presents a rare situation of a medial subtalar dislocation complicated by an acute compartment syndrome that required an urgent fasciotomy which has never been reported before.

Key words: Subtalar dislocation, Compartment syndrome, Closed reduction, Fasciotomy.

Accepted: May, 2013
Published: June, 2013

ISSN 1941-6806
doi: 10.3827/faoj.2013.0606.001


Address correspondence to: Dr. Muzamil Ahmad Baba, Govt. Hospital for Bone and Joint Surgery Barzullah, Srinagar India, 190005. Mobile 9086181281. muzamilbaba79@yahoo.com.

1Orthopaedic Resident, Govt. Hospital for Bone and joint surgery Srinagar.
2Proffessor and Head Orthopaedics, Govt. Hospital for Bone and joint surgery Srinagar. drmahalwai@rediffmail.com


The subtalar dislocation occurs through the disruption of 2 separate bony articulations, the talonavicular and talocalcaneal joints.[1,2] These joints act as a hinge that transmits load and movement from the foot to the ankle. Subtalar dislocation is an uncommon injury that disturbs the normal anatomy and function of these joints. The medial dislocation, also known as an acquired clubfoot, is the most common of all subtalar dislocations, comprising approximately 80% to 85% of the cases.[3] The medial dislocation occurs through forceful inversion of the forefoot with the talar neck pivoting on the sustentaculum tali, which acts as a fulcrum to lever the calcaneus from the talus.

Initially, it is believed that the talonavicular joint is the first to dislocate, followed by rotary subluxation through the subtalar joint, with the talar head finally coming to rest between the extensor hallucis longus and the extensor digitorum longus on either the cuboid or navicular.[4] We present a rare case report of a 36-year-old male with a medial subtalar dislocation that was complicated by an acute compartment syndrome of the foot.

Case Report

A 36-year-old male presented to our hospital 12 hours after sustaining trauma to his right foot due to a twisting injury in his farm when he was carrying a heavy load over his right shoulder. He also gave a history of some manipulation by a quack, which did not give him any relief, but the intensity of pain increased following manipulation.

???????????????????????????????
Figure 1 The anteroposterior and Lateral radiograph of right foot showing a medial subtalar dislocation.

On examination, the patient was fully conscious and local examination revealed a deformity of the right foot with a prominence medially with tense swelling of the foot. Immediate radiographs in the emergency revealed a medial subtalar dislocation. (Fig.1) The dislocation was reduced in the emergency without anesthesia with gentle longitudinal and lateral traction of the foot, resulting in anatomic reduction of the talocalcaneal and talonavicular joints. (Fig.2) A posterior splint was applied and the limb was kept elevated and patient was observed every 15 minutes for two hours. The patient continued with pain, marked swelling encompassed the entire foot, with relative sparing of the digits. Sensation to pin prick was present to all the toes and web spaces, but two point discrimination was diminished. Pain on passive motion of the toes was present. The medial-plantar aspect of the foot (medial compartment) was particularly tense and swollen.

Severe bruising of the foot was evident. Further, there was pitting edema on the dorsum of the foot. (Fig.3 and 4) The toes were slightly pale and capillary refill was approximately 3 seconds on right and < less than 2 seconds on left foot. Sensory examination revealed continued paresthesia in the same distribution. After clinical examination, a decision for a fasciotomy was made. Intra-operatively tense compartment was confirmed and bulging muscle was noted in the medial incision.

???????????????????????????????
Figure 2 Immediate Post reduction anteroposterior and lateral radiograph of same patient.

Immediately postoperatively, the foot was placed in a well-padded splint, elevated, and intravenous antibiotics administered (cefazolin). Five days later, the fasciotomy wounds were closed. The patient was discharged on the day following fasciotomy closure. Follow-up at 2 years revealed well-healed incisions, absence of neuromuscular deficits with excellent functional recovery with no signs of AVN of the talus.

Discussion

Subtalar dislocations are rare, accounting for only 1% to 2% of all dislocations.[1] Smith noted only seven dislocations in a review of 535 dislocations of all types.[5] Leitner noted only 42 among 4215 dislocations.[6] Although first described in 1811 by Judcy and Dufaurets, it involves the disruption of the talocalcaneal and talonavicular joints, while the calcaneocuboid joint remains intact.[6]

STDisFig3 STDisFig4
Figure 3 and 4 Clinical photographs of patient showing tense foot compartment prior to fasciotomy.

Subtalar dislocation can occur in any direction. Significant deformity is always present. Up to 85% of dislocations are medial.[3] The calcaneus, with the rest of the foot is displaced medially while the talar head is prominent in the dorsolateral aspect of the foot. The navicular is medial and sometimes dorsal to the talar head and neck. Lateral dislocation occurs less often. In a lateral dislocation, the calcaneus is displaced lateral to the talus and the talar head is prominent medially. The navicular lies lateral to the talar neck. Rarely, a subtalar dislocation is reported to occur in a direct anterior or posterior direction, but these are usually associated with medial or lateral displacement as well. The direction of subtalar dislocation has important effects with respect to management and outcome. The method of reduction is different for each type of injury.

Radiographs of a subtalar dislocation may be difficult to interpret. The severity of the deformity makes it difficult to obtain true anteroposterior and lateral images of the foot, and standard ankle radiographs do not reveal the foot pathology.[7]

It is important to note that the relationship between the talus and tibia and fibula is normal in a peritalar dislocation because the point of injury is distal to the ankle joint. The anteroposterior view of the foot demonstrates the talonavicular dislocation. Usually interpretation of the plain radiographs provides enough information to determine the direction of the dislocation, such that the physician can proceed with an attempt at reduction.

All subtalar dislocations require a gentle and timely reduction. In most cases, reduction can be accomplished closed. Often the injury presents with skin tenting such that a prompt reduction will reduce the possibility of skin necrosis. Once the reduction is accomplished, it should be confirmed by clinical examination and radiographs. The outcome following simple dislocations treated with closed reduction seems to be favorable.[8] In some series, as few as 10% of patients with medial dislocations and 15% to 20% of lateral subtalar dislocations required open reduction.[6] Recent series, particularly from trauma centers, have noted the need for open reduction to be more common, with 32% of patients requiring open reduction in one series.[9] A variety of bone and soft tissue structures may become entrapped, resulting in a block to closed reduction. These impediments require open manipulation or release to facilitate reduction.

Subtalar dislocations have a wide variance in terms of their prognosis. Uncomplicated subtalar dislocations, stable following a closed reduction, have an excellent prognosis with minimal symptoms at long-term follow-up.[8] Certain subtalar dislocations are clearly associated with a worse prognosis. Lancaster, et al., in a review of the literature, noted that associated injuries and complications were associated with a worse result. In particular, soft tissue injury, extra-articular fracture, intra-articular fracture, and osteonecrosis were associated with a worse outcome.[10] Open fractures are undoubtedly associated with the poorest results. Goldner et al., reviewed 15 patients at a mean of 18 years following open subtalar dislocations. Associated injuries were noted to the tibial nerve in 10 patients; to the posterior tibial tendon in 5; and to the posterior tibial artery in 5. Seven patients ultimately required arthrodesis due to osteonecrosis or post traumatic arthritis.[11]

Osteonecrosis of the talus may develop following peritalar dislocations. Overall, osteonecrosis is uncommon and generally only noted with high-energy and open injuries. Theoretically, the talus is not displaced from the ankle mortise and therefore at least some of the blood supply should be preserved. However, Goldner, et al., noted osteonecrosis in 5 of 15 patients with grade 3 open subtalar dislocations.[11] In addition, Bibbo, et al., also observed osteonecrosis in three patients.[9] Although our case was complicated by development of a compartment syndrome but timely intervention resulted in an excellent result in our case.

Conclusion

The majority of subtalar dislocations can be treated in a closed manner with a period of nonweight bearing and immobilization with satisfactory results. Occasionally, these patients may develop a compartment syndrome which, if not treated in time, may lead to catastrophic results. A high clinical suspicion and observation is warranted especially in cases with delayed presentation to diagnose such a condition early and manage it in a timely manner.

References

1. Perugia D, Basile A, Massoni C, Gumina S, Rossi F, Ferretti A. Conservative treatment of subtalar dislocations. Int Orthop 2002 26: 56-60. [PubMed]
2. Barber JR, Bricker JD, Haliburton RA. Peritalar dislocation of the foot. Can J Surg 1961 4: 205-210. [PubMed]
3. Monson ST, Ryan JR. Subtalar dislocation. JBJS 1981 63A: 1156-1158. [PubMed]
4. Buckingham WW Jr, LeFlore I. Subtalar dislocation of the foot. J Trauma 1973 13: 753-765. [PubMed]
5. Smith H. Subastragalar dislocation: a report of seven cases. JBJS 1937 19B: 373-380.[Website]
6.  Leitner B. Obstacles to reduction in subtalar dislocations. JBJS 1954 36A: 299-306. [PubMed]
7. Gross RH. Medial peritalar dislocation, associated foot injuries and mechanism of injury. J Trauma 1975 15: 682-688.[PubMed]
8. Delee JC, Curtis R. Subtalar dislocation of the foot. JBJS 1982 64A: 433-437. [PubMed]
9. Bibbo C, Anderson RB, Davis WH. Injury characteristics and the clinical outcome of subtalar dislocations: a clinical and radiographic analysis of 25 cases. Foot Ankle Int 2003 24:158-163. [PubMed]
10. Lancaster S, Horowitz M, Alonso J. Subtalar dislocations: a prognosticating classification. Orthopedics 1985 8:1234-1240. [PubMed]
11. Goldner JL, Poletti SC, Gates HS 3rd, et al. Severe open subtalar dislocations. Long-term results. JBJS 1995 77A: 1075-1079. [PubMed]

Compartment Syndrome of the Foot associated with an Open Lisfranc Injury: A case report

by Travis Motley, DPM, FACFAS, Harold Cesar, DPM

The Foot and Ankle Online Journal 4 (10): 3

A high index of suspicion is required to recognize compartment syndrome in both closed and open fractures. [1] Compartment syndrome of the foot is well described with cases secondary to closed trauma. We present an unusual case of an open Lisfranc fracture with compartment syndrome. Diagnostic modalities are discussed and early diagnosis appears to improve the success of treatment.

Key words: Compartment Syndrome, Lisfranc joint, open fracture, closed fracture.

Accepted: September, 2011
Published: October, 2011

ISSN 1941-6806
doi: 10.3827/faoj.2011.0410.0003


Compartment syndrome is considered a surgical emergency. When not treated in a timely manner, compartment syndrome can lead to disastrous complications including muscle necrosis, Volkmann’s contracture, neurological deficit, crush syndrome, and even death. [1] There are reported cases of associated compartment syndrome in open fractures of the lower leg, however not in the foot. [2] As classically described, compartment syndrome results from increased interstitial pressure in a closed osseofascial compartment, which leads to micro vascular compromise. [3] Typically, interstitial pressures in a closed osseofascial compartment are less than intravenous pressures.

This pressure gradient allows the venous system to remain patent. When the interstitial pressure surpasses that of the intravenous pressure the veins collapse and capillary blood flow ceases. This results in swelling and increase compartment pressures. [4] Webber and Manoli identified nine compartments in the foot. Three compartments run the entire length of the foot, five compartments are contained within the forefoot, and a calcaneal compartment. [5] The consensus in the treatment of compartment syndrome is to reduce pressure in order to restore perfusion. Immediate fasciotomy is recommended to prevent long-term complications. [6,7]

Case report

A 48 year-old male was brought to the Emergency Department (ED) after a motor vehicle collision (MVC). After thorough evaluation by the trauma team, the patient was found to have suffered multiple rib fractures, a left knee laceration, and a left open Lisfranc fracture. The patient was admitted for a 23 hour observation and Foot and Ankle Surgery (FAS) was consulted for the management of the open Lisfranc fracture. The FAS evaluated the patient two hours after arriving to the ED. The patient’s past medical history was significant for diabetes mellitus, hypertension, and depression. He denied prior surgeries and known drug allergies. His current medications included metformin, lisinopril, and ZoloftTM or sertraline). The patient related to occasional alcohol consumption and occasional chewing of tobacco.

During the evaluation process by FAS, the patient complained of severe pain in the left foot. The patient reported unchanged pain level even after multiple doses of analgesics in the ED.

He was administered 4 mg of DilaudidTM or hydromorphone hydrochloride and 2 mg of morphine over a two hour period before he was evaluated by FAS. Pain was elicited with palpation of the dorsal aspect of the left foot and with passive range of motion of the metatarsophalangeal joints. The dorsal aspect of the foot was tense upon palpation. Both dorsalis pedis and posterior tibial arteries were palpable. There was a 2.5 cm to 3 cm longitudinal laceration over the third inter-metatarsal space of the patient’s left foot consistent with a grade II open injury.8 There was an associated +2 edema to the dorsal aspect of the left foot. The 3rd, 4th, and 5th digits of the patient’s left foot appeared dusky however capillary refill time was less than three seconds to all of the digits.

Radiographic examination revealed a first metatarsal base fracture with mild widening of the first tarsometatarsal joint. Computer Tomography (CT) revealed comminuted fractures of the proximal first and second metatarsals with intra-articular extension. The first and second metatarsal bases were displaced laterally.

There were intra-articular fractures of third and fourth metatarsal bases noted without displacement. There was diffuse soft tissue swelling with numerous scattered subcutaneous and intramuscular air bubbles. (Figs. 1, 2 and 3)

Figure 1 lateral view showing subcutaneous air dorsally.

Figure 2 Axial view shows the Lisfranc injury with subcutaneous and intramuscular air bubbles.

Figure 3 Frontal view also shows the Lisfranc injury with subcutaneous air.

The patient was taken to the operating room for decompression fasciotomy of the left foot. The laceration on the dorsal aspect of the patient’s foot was extended proximally and distally. Upon decompression of the interosseous compartment, there was approximately forty to fifty cubic centimeters of hematoma released from the wound. After evacuating this hematoma, the dorsal aspect of the left foot was soft upon palpation and there was hyperemic response to digits 3-5. The remaining compartments were soft and were not released. The wound was then subsequently irrigated with 6 liters of normal saline. A negative pressure therapy device was then applied to the patient’s left foot (Wound Vac KCI™, San Antonio).

Four days later, the patient underwent a repeated irrigation and debridement with percutaneus fixation of the Lisfranc joint with Kirschner-wires. (Fig. 4) After a granular bed was achieved, the wound was subsequently covered with a split thickness skin graft at about 4 weeks. [7]

Figure 4 Left foot with dorsal wound and percutaneous Kirschner wires.

Discussion

The diagnosis of compartment syndrome requires a high index of suspicion as this syndrome can occur after any injury regardless of mechanism.1 With open fractures however, it was postulated that compartment syndrome is not possible because there is an escape route for hematoma. Depending on the force, open fractures may decompress one or two compartments, but the remaining compartments may be at risk for developing compartment syndrome. [8] Certain wounds do not decompress the underlying muscle by splitting the overlying fascia and thus are not able to accommodate swelling and therefore are not sufficient to allow decompression of the compartment. [2] A hole in the compartment might not constitute adequate decompression because tissue does not flow like a liquid. [4]

There are multiple modalities available to objectively diagnose compartment syndrome such as intracompartment pressure monitoring (ICP), near infrared spectroscopy (NIRS), magnetic resonance imaging (MRI), scintigraphy, and laser Doppler flowmetry. There are several sources of error and learning curves associated with each of those modalities. [1] It is recommended that compartment syndrome be confirmed by measuring the intra-compartment pressure. [4] In our patient we did not feel the need to measure the intra-compartment pressure, as clinical signs for compartment syndrome were present. Our patient had pain that remain unchanged since arriving to the ED despite the administration of multiple doses of analgesics. The dorsal aspect of the foot was tense, pain was elicited with passive range of motion, and there was pallor of the digits 3-5. All reliable clinical findings for compartment syndrome. [6] In order to prevent disabling complications, early diagnosis derived from clinical findings is the key in the successful treatment of compartment syndrome.

References

1. Elliott K, Johnstone AJ. Diagnosing acute compartment syndrome. JBJS 2003 85B: 625-632.
2. Blick SS, Brumback RJ, Poka A, Burgess AR, Ebraheim NA. Compartment syndrome in open tibial fractures. JBJS 1996 68A: 1348-1353.
3. Mubarak SJ, Owen CA, Hargens AR, Garetto LP, Akeson WH. Acute compartment syndromes: diagnosis and treatment with the aid of the wick catheter. JBJS 1978 60A:1091-1095.
4. Matsen III, FA. Compartmental syndrome. A unified concept. Clin Orthop 1975 113:8-1114.
5. Manoli II A, Weber TG. Fasciotomy of the foot: an anatomical study with special reference to release of the calcaneal compartment. Foot Ankle 1990 10: 267-275.
6. Myerson M. Diagnosis and treatment of compartment syndrome of the foot. Orthopedics 1990 13: 711-717.
7. Myerson MS. Experimental decompression of the fascial compartments of the foot – the basis for fasciotomy in acute compartment syndromes. Foot Ankle 1988 8: 308-314.
8. Gustilo RB, Anderson JT. Prevention of infection in the treatment of one thousand and twenty-five open fractures of long bones. JBJS 1976 58A: 453-458.
9. Rorabeck CH, Macnab I. Anterior tibial compartment syndrome complicating fractures of the shaft of the tibia. JBJS 1976 58A: 549-550.


Address correspondence to: Travis Motley, DPM, MS, FACFAS. John Peter Smith Hospital, 1500 South Main Street, Fort Worth, TX 76104. Email: Tmotley@jpshealth.org

1  Associate Professor, University of North Texas Health Science Center. John Peter Smith Hospital, Department of Orthopedics, Podiatry Division, 1500 South Main Street, Ft. Worth, TX 76104.
2  PGY-2, John Peter Smith Hospital, Department of Orthopedics, Podiatry Division, 1500 South Main Street, Ft. Worth, TX 76104.

© The Foot and Ankle Online Journal, 2011

May-Hegglin and other Platelet Dysfunctions as Complications to Compartment Syndrome: A case report

by Jason R. Miller, FACFAS, FAPWCA1, Peter Moyer, DPM2

The Foot & Ankle Journal 1 (9): 1

Compartment syndrome is a well known surgical emergency encountered by physicians on trauma call. When compounded by platelet dysfunction, the management of a compartment syndrome becomes exponentially more difficult for the surgeon. The following case describes a twenty-four year old male who sustained multiple comminuted tarsal and metatarsal fractures after a crush injury that was further complicated by an existing platelet dysfunction known as May-Hegglin anomaly (MHA). This article reviews May-Hegglin and other rare hematological conditions that often obscure otherwise straightforward surgical cases.

Key words: May-Hegglin, MHA, compartment syndrome, external fixation, foot fractures

This is an Open Access article distributed under the terms of the Creative Commons Attribution License.  It permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. ©The Foot & Ankle Journal (www.faoj.org)

Accepted: August, 2008
Published: September, 2008

ISSN 1941-6806
doi: 10.3827/faoj.2008.0109.0001

May-Hegglin anomaly (MHA) is a familial disorder that is a rare type of autosomal dominant platelet disorder. From 2000-2005, only 85 families with MHA were reported. [6] It is associated with thrombocytopenia with varying degrees of purpura, bleeding, giant platelets, and cytoplasmic inclusion bodies that resemble Döhle bodies in the granulocytes (neutrophils, eosinophils, monocytes). [4,6] In these patients, neutrophil and platelet function is considered to be normal. Thrombocytopenia occurs in almost all patients and severe bleeding is rare but has been reported.

These patients may have a range of symptoms from asymptomatic to recurrent epistaxis, gingival bleeding, easily bruising to menorrhagia. MHA has not been associated with higher rates of infection. [4]

In 1909, German physician May described a young female patient who had leukocytic inclusions, who was asymptomatic. [6] In 1945, Swiss doctor Hegglin described a father and his two sons who had a triad of thrombocytopenia, giant platelets, and leukocytic inclusions. [6]

These patients have a mutation of the MYH9 gene, encoding non-muscle myosin heavy chain IIA, present in chromosomal region 22q12-13. [4,6] This mutation results in disordered production of non-muscle myosin heavy-chain type IIA.

The result is macrothrombocytopenia, secondary to defective megakaryocyte maturation and fragmentation. Other associated syndromes are Sebastian, Fechtner, or Epstein syndromes. Differential diagnosis associated with thrombocytopenia and large platelets include Alport syndrome, Bernard-Soulier syndrome, Montreal platelet syndrome, immune thrombocytopenia, and gray-platelet syndrome. [6] The differential diagnosis for leukocytic inclusions includes septicemia, myeloproliferative disorders, and pregnancy.

A case report describes a twenty-four year old male who sustained multiple comminuted tarsal and metatarsal fractures after a crush injury that was further complicated by an existing platelet dysfunction known as May-Hegglin anomaly (MHA).

Case Report

A twenty-four year old deaf man was transported from a local hospital to our Level 1 trauma center for evaluation. He was at work when a steel industrial loading dock door came crashing down and landed across his left foot. (Fig. 1)

Figure 1 Initial presentation after crush injury of the left foot. 

Initial evaluation in the trauma bay was significant for left foot swelling, pain, and mottled skin. His sensory function was compromised while gross motor function remained intact. He presented with stable vital signs.

His past medical history was positive for the May-Hegglin anomaly. He reported living with his parents, denied allergies, and had an otherwise unremarkable review of systems. A full physical exam was normal with the exception of his left lower extremity.

The lower extremity exam was positive for: diminished pulses, exquisite pain on palpation of the mid-foot area, pain with range of motion of digits 1,2,3 and 4, decreased temperature, color changes, and swelling. Arterial line pressure monitoring revealed compartment pressures between 75 mmHg and 100 mmHg in the foot, therefore the operating room was called and prepared for emergent surgery.

Plain film and CT scan revealed the following fractures: comminuted intra-articular fracture of the calcaneus, comminuted fractures of the navicular, cuboid, proximal portions of the cuneiforms and fractures at the base of the second and third metatarsals. (Figs. 2,3)

Figure 2 Radiograph reveals first and second metatarsal crush fracture. 

Figure 3  Sagittal CT view of crush injury.  Displaced metatarsal, calcaneal, and cuneiform fractures are evident. 

Stat labs revealed the following abnormalities: WBC 4.9, HgB 8.6, HCT 25.9 and platelets were 39,000mm3. He was then typed and crossed for surgery.

Surgical Procedure

In the operating room, general anesthesia was administered and an emergent fasciotomy was preformed following typical sterile preparation. His left foot was noted to be severely cyanotic, mottled, and cool to touch. An 8-10 cm medial incision was made to the level of the deep fascia.

After the deep fascia was penetrated via blunt dissection, copious amounts of dark, non-coagulated blood flowed from the incision site. (Fig. 4)

Figure 4  Surgical exploration shows dark, non-coagulated blood and hematoma associated with the compartment syndrome. 

Both the medial and plantar compartments were explored through this incision. Approximately one to two minutes after initial incision was made, the hallux changed from a mottled, blanched, cyanotic color to a healthy pink hue with appropriate capillary refill time. A second incision was then made between the shafts of the second and third metatarsals. Blunt dissections in to the deep fascia revealed additional copious amounts of dark blood that was evacuated from the compartment. A third incision was placed between the fourth and fifth metatarsals, and again this compartment was relieved of congestion. Within five minutes after initial incision, the entire foot was pink and warm with a dramatic decrease in the swelling. Further evaluation noted that the rear-foot remained mottled and cyanotic. At that point a fourth incision was made anterior to the Achilles tendon into the deep fascia, and approximately 5 cc of dark blood was evacuated from the calcaneal compartment. The incisions were flushed and packed with saline soaked nu-gauze packing. Attention was then paid to the medial aspect of the calcaneus where a closed reduction of the sustentacular fragment was performed under fluoroscopy.

An external fixator device was placed in triangular fashion under fluoroscopy to maintain proper alignment of the destabilized midfoot and forefoot fractures.

Post-operatively, a posterior splint with a mild compressive dressing was applied and CBC was collected. Medical and hematology consults were activated, neurovascular evaluations were ordered every two hours, cefazolin 1g every 8 hours was started, and repeat radiographs and CT scans were performed.

On post-operative day number one (POD #1), hematology recommended transfusions of both platelets and packed red blood cells prior to the surgical procedure scheduled for POD #5. While they recommend the use of SCD’s, compression stockings, and out of bed to chair three times per day, they discouraged the use of heparin or enoxaparin for DVT prophylaxis. Hematology also recommended that in monitoring the patient for active bleeding, the hemoglobin, hematocrit and platelet count should be drawn every 12 hours and to consider desmopression (DDAVP) if the labs worsened.

On POD #4, he was transfused with four units of platelets, two units of packed red blood cells, and was given prophylactic diphenhydramine.

The patient tolerated the transfusion well with no evidence of reaction. On POD # 5, he was taken back to the operating room for a successful wash out, minor debridement and primary delayed closure. The patient was discharged on POD #6 after two normal CBC evaluations.

His uneventful postoperative course was interrupted on his second office visit when it was noticed that there was some displacement at the comminuted first metatarsal-cuneiform joint. He was taken back to the operating room for a possible fusion or re-manipulation/stabilization procedure. Intra-operatively, the joint was easily manipulated back into place, and small Steinman pins were introduced for stability. Additionally, the sustentacular fragment of the calcaneal fracture was definitively fixated with 4.0mm cannulated screw fixation under fluoroscopy by percutaneous technique. The fixation pins and external fixator were removed six weeks later and he has since returned to regular employment approximately 8 months following this injury. He reports no residual deformity or pain and is able to ambulate freely in regular shoegear. (Fig. 5)

Figure 5 Patient post reduction with functional left foot and no residual pain or deformity.

Discussion

It is important to note that platelets play a central role in normal hemostasis and thrombosis. Platelets originate from pluripotent stem cells that undergo differentiation to the megakaryoblast and then to platelets. Normal platelet counts are between 150,000 to 300,000mm3, with thrombocytopenia being defined as a platelet count less than 100,000mm3. Spontaneous bleeding typically becomes evident after counts drop below 20,000mm3 (spontaneous head bleeds < 5,000mm3). [6]

In the circulating form, platelets appear as a smooth discs enclosed within a plasma membrane. This membrane contains a number of receptor glycoproteins that are responsible for platelet function. Within the platelet are two specific types of granules.

The first, alpha granules contain fibrinogen, fibronectin, factors V and VIII, platelet factor 4 and platelet-derived growth factor and transforming growth factor beta. The second type of granule is for non-metabolic pool adenine nucleotides (ADP & ATP), ionized calcium, histamine, serotonin, and epinephrine.6 When a vessel wall is damaged, platelets undergo three reactions: (1) adhesion and shape change, (2) secretion, and (3) aggregation collectively referred to as platelet activation. [4] (Fig. 6)

From May-Hegglin Anomaly, eMedicine, 2008.

Figure 6 2000x blood smear of a MHA patient demonstrating a typical giant platelet with ill defined granulation.  A normal sized platelet is also seen here.  The cytoplasmic inclusion body represents a Dohle body.

 

A cell blood count is essential in starting a workup in these patients. The platelet count is decreased, usually between 40,000-80,000mm3. The platelets are enlarged up to 15mm3 in diameter, with normal morphology. [4] Evaluation at the electron microscopy level reveals normal cell organelles with an increased amount of disorganized microtubuli.

The Wright-stained peripheral blood smear shows cytoplasmic inclusion bodies, most dominant in the neutrophils, but some are present in the eosinphils, monocytes, and basophils.

The inclusions are up to 5µm in size, they are spindle shaped, pale, blue-staining bodies that consist of ribosomes, endoplasmic reticulum, and microfilaments. [4] The inclusions are similar to Döhle bodies and are found in the periphery of the cytoplasm. [4] Bleeding time is typically prolonged in concordance with the degree of thrombocytopenia.

Since patients with MHA do not have significant bleeding problems, treatment should be based on clinical evaluation, laboratory evaluation and following recommendations from a hematologist pre- and post operatively. Though it is rare for a MHA patient to develop severe bleeding intra- and post operatively, the skilled foot and ankle surgeon should be aware of the risk of bleeding requiring transfusions. [5]

Desmopressin acetate (DDAVP), is a synthetic vasopressin analogue that has been used peri-operatively in patients with MHA. It is an altered form of vasopressin in which deamination of hemicysteine at position 1 and substitution of D-arginine for L-arginine at position 8 has occurred. [2] Desmopressin binds to the V2 receptor in renal collecting ducts, increasing water resorption. It also stimulates release of factor VIII from endothelial cells due to stimulation of the V1a receptor. [2] This change in stereochemistry eliminates vasopressor (V1) receptor agonist activity and enhances the antidiuretic (V2) receptor agonist action and prolongs duration of action from 2-6 hours to 6-24 hours. [2]

Desmopressin stimulates the endothelial release of factor VIII and von Willebrand factor into the plasma (V2 receptor effect).

After a slow infusion of 0.3mcg/kg, plasma concentrations of factor VIII and von Willebrand factor is 2-4x greater. [2]  Although it can be unpredictable, desmopressin has been shown to shorten bleeding time in a variety of platelet dysfunctional diseases.

DDAVP has become the drug of choice for prevention and treatment of bleeding in patients with mild hemophilia A and von Willebrand’s disease because of the increase in factor VIII and von Willebrand factor, but its mechanism in platelet disorders is still one of debate. [5]

Sehbai, et al., reported a case where 34-year old woman with known MHA underwent a craniotomy secondary to an intractable seizure disorder since childhood. [5] After an extensive family history, past medical history of the patient, and extensive workup which included; magnetic resonance imaging (MRI) of the brain, positron emission tomography (PET) scan, and 24 hour video EEG, the woman underwent craniotomy and resection of the temporal lobe foci of seizure activity. She was admitted one day prior to surgery and transfused with 6 units of platelets, and one hour before surgery was given DDAVP. Platelets were on standby if needed intra or post operatively. Her postoperative course was uneventful except for mild hyponatremia secondary to the DDAVP. [5]

Chabane, et al., reported a 24 year old female that was diagnosed with severe thrombocytopenia after giving birth. She was later diagnosed with MHA. She later went on to have a second and third child via cesarean section, and she did not receive platelets for either. The third child was affected by the MHA with a platelet count of 49,000mm3 as well as inclusion bodies on blood smear. [1]

Matzdorff, et al., reported on a patient with Fechtner syndrome that underwent a tonsillectomy and was given DDAVP pre-operatively. [3]

The patient was a 24 year old woman with a past medical history of thrombocytopenia and bruised easily in childhood. She had been diagnosed with Sebastian platelet syndrome, had also noted a impairment with her hearing as well as mild hematuria. After a detailed family history it was noted that some relatives had thrombocytopenia and hearing impairment. At the time, a blood smear was obtained and evaluated with electron-microscope, which confirmed that the inclusions were consistent with Fechtner syndrome. The woman underwent extensive laboratory evaluation: modified Ivy bleeding test, platelet aggregation studies with ADP, collagen, and ristocentin, and standard coagulation test. The patient also had a bone marrow biopsy. The pertinent test in this case was the bleeding test which was greater than thirty minutes, normal being 5-8 minutes. [3] The test was repeated after DDAVP was given, and her bleeding time normalized to 7 minutes 30 seconds, and her von Willebrand factor (her base line was above average) antigen had increased from 150% to 282%. On the day of surgery the women received DDAVP 0.4 µg/kg over 30 minutes 1 hour before the start time, and the surgery went uneventful. [3]

Conclusion

May-Hegglin is a rare platelet disorder associated with macrothrombocytopenia, leukocyte inclusions, deafness and nephritis. Patients may experience easy bruising, recurrent epistaxis, gingival bleeding, menorrhagia, and excessive bleeding associated with surgical procedures. A patient that presents with MHA and an un-witnessed fall should get a CT scan to rule out intracranial hemorrhage and internal bleeding. Patients that present with MHA should be evaluated by a hematologist to recommend DDAVP and platelet transfusions when necessary. In this case, MHA likely played a compounding role in the rapid development of the foot compartment syndrome encountered and could have certainly compounded the post-operative course.

This case demonstrates the need for a multi-disciplinary approach to patients exhibiting May Hegglin anomaly and expeditious surgical intervention when this rare patient population experiences a traumatic event. Additionally, it demonstrates the need to take a thorough history to reveal rare disorders, like this one, in an elective surgery population. A lack of proper treatment in patients with rare platelet disorders can certainly lead to devastating complications. It is our sincere hope that this article will serve to guide the foot and ankle surgeon to appropriately recognize and treat complicating disease processes when they present.

References

1. Chabane H, Gallais Y, Pathier D, Tchernia G, Gaussem P. Delivery management in a woman with thrombocytopenia of the May-Hegglin anomaly type. Eur J Obstet Gynecol and Reproduction Bio 99:124-25, 2001.
2. Mahdy A.M., Webster N.R. Perioperative systemic haemostatic agents. British J Anaesthesia 93(6):842-58, 2004.
3. Matzdorff AC, White JG, Malzahn K, Greinacher A. Perioperative management of a patient with Fechtner syndrome. Ann Hematol 80:436-439, 2001.
4. Noris P, Spedini P, Belletti S, Magrini U, Balduini C. Thrombocytopenia, giant platelets, and leukocyte inclusion bodies (May-Hegglin anomaly): clinical and laboratory findings. Am J Med 104:355-60, 1998.
5. Sehbai A, Abraham J, Brown V. Perioperative management of a patient with May-Hegglin anomaly requiring craniotomy. Am J Hematol 79:303-08, 2005.
6. Shafer FE. May-Hegglin Anomaly. eMed J [online], 2003.


 
Address correspondence to: Jason R. Miller, FACFAS, FAPWCA
Chief, Foot and Ankle Surgery, Pennsylvania Orthopaedic Center
Adjunct Associate Professor, Dept. of Surgery, TUSPM
Office: 215-644-6900 , FAX: 215-644-7160
Email: jrmiller71@pol.net

1Chief, Foot and Ankle Surgery, PA Orthopaedic Center. Adjunct Associate Professor, Dept. of Surgery, TUSPM, Philadelphia, Pa. 19107.
2PGY-4, Foot and Ankle Surgery, Temple University Hospital, Philadelphia, PA, 19140.

© The Foot & Ankle Journal, 2008