Tag Archives: foot fractures

Protected Weight Bearing During Treatment of Acute Charcot Neuroarthropathy: A case series

by Jeremy J. Cook, DPM,MPH,CPH, Emily A. Cook, DPM,MPH,CPH

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

Standard of care in the treatment of acute Eichenholtz stage I Charcot neuroarthropathy includes complete non-weight bearing immobilized with total contact casting. This small case series of three patients focuses on patients with acute phase midfoot Charcot neuroarthropathy treated with non-casting immobilization therapy. All patients were male with a mean age of 48.7 (range 46-53) years. Patients were instructed to assume complete non-weight bearing during treatment. Due to financial restrictions, all patients reported fully weight bearing in the non-removable immobilization boot because of work related obligations. Immobilization therapy lasted a mean duration of 90.3 days (range 76 – 133 days) and was discontinued once there was clinical resolution of inflammation and osseous stability. Serial radiographs revealed absence of deformity progression and eventual consolidation in all cases. All patients remained ulcer and callus free during immobilization therapy, without progression of a rocker-bottom deformity, while fully weight bearing and maintaining full-time manual labor employment. This preliminary case series adds to the evidence base that it may be possible to allow protected weight bearing during acute phase Charcot neuroarthropathy with adequate immobilization of the foot at all times.

Key words: Diabetes, Charcot neuroarthropathy, Foot Deformity, Casting, Foot fractures, Diabetic Foot.

Accepted: June, 2011
Published: July, 2011

ISSN 1941-6806
doi: 10.3827/faoj.2011.0407.0001

Charcot neuroarthropathy is an increasingly common clinical entity encountered by foot and ankle professionals. In the early decades of the last century syphilis was the most commonly associated etiology. That has changed with the advent of insulin and the resulting extended survival of patients suffering from diabetes mellitus.

Delay in diagnosis and patient non-compliance can result in severe destruction of the foot and ankle with permanent disability from ulceration, infection, and eventual amputation. [1-14]

The standard of care for treatment of Eichenholtz stage 115 Charcot neuroarthropathy has been immobilization in a total contact cast and complete non-weight bearing. [16-20] The period of non-weight bearing immobilization should last until erythema, edema, and warmth subside and the foot becomes stable and consolidated enough to prevent anatomic destruction while ambulating. This process has been reported to last anywhere from a few months to over two years. [20-26]

Total contact casts (TCC) have been shown in numerous studies to be an effective immobilization device in the treatment of acute Charcot neuroarthropathy. [5,7-14,16-20]

It is recommended that TCCs be changed frequently in order to prevent cast irritation, ulceration and to maintain immobilization as edema subsides. Minor complications such as skin irritation are anticipated with TCC. The risk of major complications such as ulceration and infection can be minimized with proper application techniques, as well as frequent casts changes, which permit careful monitoring, and adequate patient education. [27] Many centers have specially-trained orthotists who apply TCCs on a routine basis. Most studies support changing TCCs for the treatment of Stage I Charcot neuroarthropathy every 1-2 weeks. [11,13,17,19,22,27-31] Some institutions have allowed weight bearing in the TCC due to its inherent stability with success in preliminary reports. [9,27-31]

Although weight bearing during stage I of Charcot neuroarthropathy is controversial, many patients tend to be non-compliant. This is because this period of prolonged non-weight bearing may be detrimental in quality of life and may pose to be an unacceptable disability. [32,33] While the alternative may be amputation, advances in immobilization technology may allow protected weight bearing during the early stages of Charcot without the development of severe deformity. [34,35] The purpose of this study was to report results of acute Stage 1 Charcot neuroarthropathy in individuals immobilized in a vacuum stabilization boot that maintained full weight bearing.

Case Series

Three consecutive patients presented with acute Stage I Charcot neuroarthropathy over a three month period (November 2009 to January 2010). All three patients had Brodsky type I deformity involving the tarsometatarsal and naviculocuneiform joints. [18] Patients were referred for examination and treated within two weeks of symptom onset. Clinical examination revealed erythema, warmth, and edema involving the midfoot with gross instability, crepitation with midfoot range of motion, and bounding pedal pulses. One patient had diabetic neuropathy while the other two were diagnosed with alcoholic neuropathy. Peripheral neuropathy was confirmed by the absence to detect the Semmes-Weinstein 10gm monofilament.

Two of the three patients reported a minor injury preceding the Charcot event. The third patient had had previous amputations of digits two and three for localized osteomyelitis secondary to contiguous digital ulcerations. All three patients were male with a mean age of 48.7 (range 46-53) years. All patients presented within two weeks of first symptoms and were ulcer free at the time of initial presentation with this being their first occurrence of Charcot neuroarthropathy. Radiographs were obtained with findings consistent with early signs of Charcot neuroarthropathy. (Fig. 1A and 1B) Magnetic resonance (MR) imaging further confirmed the diagnosis with diffuse bone marrow edema adjacent to the Lisfranc joint.


Figure 1A and 1B  Initial anterior posterior (AP) (A) and lateral (B) radiographs demonstrating soft tissue edema with early signs of osteolysis, cortical thickening, fragmentation, and osseous destruction within the tarsometatarsal and naviculocuneiform joints.

All three patients were treated with immobilization in a vacuum stabilization boot (VACOcast®, OPED Inc, Framingham, MA) with instructions to remain strictly non-weight bearing. (Fig. 2) Despite these recommendations, all three patients reported bearing weight on the affected limb in order to prevent loss of their job. All three patients were sole providers in their household with jobs that required extensive manual labor. The patients were compliant in wearing the boot at all times as this was verified through inspecting the undamaged compliance locks on the boot.

Figure 2   In this boot, by removing air from a vacuum cushion, small beads contour around the lower limb and create vacuum stabilization.

Serial monitoring was conducted by clinical examinations and plain radiographs. Patients were kept immobilized in the vacuum stabilization boot until resolution of edema, warmth (examined by palpation with back of hand and fingers and comparing to contra-lateral limb), and clinical stability was achieved. Successive radiographs were taken to ensure the absence of deformity progression every 3-4 weeks. (Fig. 3A, 3B and 3C) Throughout the treatment period each patient maintained normal full weight bearing in the conduct of their full-time jobs.


Figure 3A, 3B and 3C  Progression of acute phase of Charcot neuroarthropathy.  AP (A), Oblique (B) and lateral (C) views demonstrate increased osseous destruction and osteolysis.

Patients wore the vacuum stabilization boot for a mean of 90.3 days (range 76 – 133 days). One patient developed a superficial abrasion on the dorsal proximal interphalangeal joint of the second digit. This healed after two weeks of wound care and the additional of padding to the boot in this area. There were no other complications experienced. During the treatment of acute Stage I Charcot neuroarthropathy, all three patients remained ulcer and callus free while ambulating in the immobilization boot. Once the Charcot events had progressed to the consolidation phase, patients were transitioned to accommodative shoes or boots with supportive inserts.

Two of the three patients were compliant with accommodative shoes and molded insoles. After 16 months from the initial presentation, both patients have not developed ulcers, callus, or progression of deformity. (Fig. 4A, 4B and 4C) During the 12 weeks that the third patient was wearing the immobilization boot, the deformity did not progress and the patient remained ulcer and callus free.


Figure 4A, 4B and 4C  AP (A), Oblique (B) and lateral (C) views showing progression into chronic Charcot neuroarthropathy with maintenance of anatomic alignment with consolidation of osseous destruction.

However, the third patient did not obtain prescribed accommodative shoes or inserts citing financial limitations. He was subsequently lost to follow-up for five months after completing 12 weeks of immobilization therapy. His Charcot neuroarthropathy had developed a rocker bottom foot deformity and plantar midfoot ulcer after five months of interrupting care.


Management of Charcot neuroarthropathy is a complex process which requires flexibility and constant attention. This small case series demonstrates that despite the overt disregard for non-weight bearing management instructions, all patients were able to maintain employment and prevent progression of rocker bottom midfoot deformities during acute Eichenholtz stage I Charcot neuroarthropathy as there was continuous utilization of the vacuum immobilization boot.

Patients were continuously immobilized in a vacuum stabilizing below-knee boot with compliance confirmed by boot locks. There were minimal complications during the acute phase treatment with one patient developing a superficial digital abrasion from the boot. This was identified immediately and rectified by adjusting the boot. Despite fully weight-bearing, a rocker bottom deformity was prevented with adequate and constant immobilization.

Standard of care for acute Eichenholtz stage I traditionally includes total contact casting and complete non-weight bearing to prevent progression of deformity. This has been recently challenged by allowing weight bearing in the total contact cast in combination with frequent cast changes and close monitoring. Two prospective case series have reported successfully preventing deterioration of osseous alignment from acute phase Charcot deformity with weight-bearing total contact casts. [28,29]

The amount of non-restrained cumulative load forces across acute Charcot joints is also believed to increase the amount of deformity progression. By immobilizing the foot with a walking total-contact cast, the acute phase resolved and further progression towards a rocker bottom foot was prevented. [30]

The immobilization boot reported in this study was chosen for several reasons. Total contact casts require frequent changes and proper construction to prevent complications related to this casting technique. This immobilization boot had the advantage of clinical efficiency as no time was necessary beyond properly sizing and fitting the patient and providing instructions on its use. The vacuum boot can be adjusted to accommodate changes in edema. The removable sole allows patients to sleep with the boot without dirtying the linens. It also has a radiolucent frame that permits radiographic evaluation without removal. Finally, the compliance locking straps prevent unknown patient removal. Although none of the affected limbs had an open ulcer necessitating daily care, had local wound care been necessary by a visiting nurse an additional key would have been provided.

Limitations of this study include its retrospective nature. The initial treatment plan did not permit patients to weight bear during acute phase Charcot neuroarthropathy, however, weight bearing did not adversely impact the treatment outcome. Both mechanical and comparative studies are needed to further investigate the ability of nontraditional immobilization devices to effectively prevent osseous deformity in a disease which can cause permanent disability and eventual amputation. Future prospective studies with a larger sample size are needed to assess the long-term outcomes of this immobilization technique. Comparison studies of different immobilization techniques would also be very useful. Finally, the definition of adequate immobilization needs further investigation in order to achieve a balance of prevention of serious Charcot-related complications and quality of life.


Patients with acute Eichenholtz stage I midfoot Charcot neuroarthropathy were able to fully weight bear and maintain manual labor employment without development of a rocker bottom foot deformity while wearing a vacuum stabilization below-knee boot. Advances in immobilization therapy may allow improvement in the quality of life in acute phase Charcot neuroarthropathy.


1. Holewski, J, Moss KM, Stess RM, Graf PM, Grunfeld C. Prevalence of foot pathology and lower extremity complications in a diabetic outpatient clinic. J Rehab Res and Devel 1989 26: 35-44.
2. Sohn MW, Stuck RM, Pinzur M, Lee TA, Budiman-Mak E. Lower-extremity amputation risk after charcot arthropathy and diabetic foot ulcer. Diabetes Care 2010 33: 98-100.
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4. Sohn MW, Lee TA, Stuck RM, Frykberg RG, Budiman-Mak E. Mortality risk of Charcot arthropathy compared with that of diabetic foot ulcer and diabetes alone. Diabetes Care 2009 32: 816-821.
5. Boulton AJ, Jeffcoate WJ, Jones TL, Ulbrecht JS. International collaborative research on Charcot’s disease. Lancet 2009 J373 (9658): 105-106.
6. Shibuya N, La Fontaine J, Frania SJ. Alcohol-induced neuroarthropathy in the foot: a case series and review of literature. J Foot Ankle Surg 2008 47: 118-124.
7. Nielson DL, Armstrong DG. The natural history of Charcot’s neuroarthropathy. Clin Podiatr Med Surg 2008 1: 53-62.
8. Frykberg RG, Belczyk R. Epidemiology of the Charcot foot. Clin Podiatr Med Surg 2008 1:17-28.
9. Pinzur MS. Current concepts review: Charcot arthropathy of the foot and ankle. Foot Ankle Int 2007 8: 952-959.
10. Sanders LJ. What lessons can history teach us about the Charcot foot? Clin Podiatr Med Surg 2008 1: 1-15.
11. Wukich DK, Sung W. Charcot arthropathy of the foot and ankle: modern concepts and management review. J Diabetes Complications 2009 23: 409-426.
12. Jeffcoate WJ. Charcot neuro-osteoarthropathy. Diabetes Metab Res Rev 2008 24 (Suppl 1): S62-65.
13. Petrova NL, Edmonds ME. Charcot neuro-osteoarthropathy-current standards. Diabetes Metab Res Rev 2008 24 (Suppl 1): S58-61.
14. Chantelau E. The perils of procrastination: effects of early vs. delayed and treatment of incipient Charcot fracture. Diabet Med 2005 22: 1707–1712.
15. Eichenholtz SN. Charcot Joints. Springfield, Illinois: Charles C. Thomas, 1966.
16. Shaw JE, His WL, Ulbrecht JS. The mechanism of plantar unloading in total contact casts: implications for design and clinical use. Foot Ankle Int 1997 18: 809-817.
17. Pinzur MS, Shields N, Trepman E, Dawson P, Evans A. Current practice patterns in the treatment of Charcot foot. Foot Ankle Int 2000 21: 916–920.
18. Brodsky JW. The diabetic foot. In: Coughlin MJ, Mann RA, editors. Surgery of the Foot and Ankle. Vol 2. 7th ed. St. Louis, Mosby. 1999, 895-969.
19. Pinzur MS, Shields N, Trepman E, Dawson P, Evans A. Current practice Patterns in the treatment of Charcot foot. Foot Ankle Int 2000 21: 916-920.
20. Armstrong DG, Todd WF, Lavery LA, Harkless LB, Bushman TR. The natural history of acute Charcot’s arthropathy in a diabetic foot specialty clinic. Diabetic Med 1997 14: 357-363.
21. Molines L, Darmon P, Raccah D. Charcot’s foot: newest findings on its pathophysiology, diagnosis and treatment. Diabetes Metab 2010 36: 251-255.
22. Pinzur, MS. Surgical vs. accommodative treatment for Charcot arthropathy of the midfoot. Foot Ankle Int 2005 25: 545-549.
23. Myerson MS, Henderson MR, Saxby T, Short KW. Management of midfoot diabetic neuroarthropathy. Foot Ankle Int. 1994 15: 233-241.
24. Alpert SW, Koval KJ, Zuckerman JD. Neuropathic arthropathy: review of current knowledge. J Am Acad Orthop Surg. 1996 4: 100-108.
25. Fabrin J, Larsen K, Holstein PE. Long-term follow-up in diabetic Charcot feet with spontaneous onset. Diabetes Care 2000 23: 796-800.
26. Schon LC, Easley ME, Weinfeld SB. Charcot neuropathy of the foot and ankle. Clin Orthop Relat Res 1998 349: 116-131.
27. Wukich DK, Motko J. Safety of total contact casting in high-risk patients with neuropathic foot ulcers. Foot Ankle Int 2004 25: 556-560.
28. Pinzur MS, Lio T, Posner M. Treatment of Eichenholtz stage I Charcot foot arthropathy with a weightbearing total contact cast. Foot Ankle Int 2006 27: 324-329.
29. de Souza LJ. Charcot arthropathy and immobilization in a weight-bearing total contact cast. JBJS 2008 90A:754-759.
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35. Stöckle U, König B, Tempka A, Südkamp NP. Cast immobilization or vacuum stabilizing system? Early functional results after osteosynthesis of ankle fractures. Unfallchirurg 2000 103: 215-219.

Address correspondence to: Harvard Medical School, Beth Israel Deaconess Medical Center, Boston, MA.
Email: jeremycook@post.harvard.edu

1,2  Clinical Instructors in Surgery at Harvard Medical School, Division of Podiatric Surgery, Department of Surgery.
185 Pilgrim Road, PB Span 3, Beth Israel Deaconess Medical Center, Boston, MA. 617-632-7098

© The Foot and Ankle Online Journal, 2011

The Clinical Importance of the Os Peroneum: A Dissection of 156 Limbs Comparing the Incidence Rates in Cadavers versus Chronological Roentgenograms

by Brion Benninger, MD 1 , Jessica Kloenne 

The Foot and Ankle Online Journal 4 (2): 2

Introduction: The purpose of this study was to assess radiograph incidence versus cadaver incidence rates of the os perineum (OP) within the fibularis (peroneus) longus tendon (FLT) and to assess why broad variants have occurred in previous radiograph and cadaver studies. The OP or sesamoid bone in the FLT has a history of controversy regarding the terminology and frequency. Recent histological studies have proven sesamoid terminology. Cadaver studies have revealed high incidence rates (IR), yet virtually all texts and atlases exclude it. Clinicians recognize it in routine foot radiographs. No studies have compared IRs between cadavers and injured patients of the general population.
Methods: A literature search of texts, atlases, journals and websites was conducted identifying incidence of OP within the FLT. Dissection of 82 embalmed cadavers (M 52, F 30) identified the IR of the OP. Oblique foot radiographs from 1,025 individuals were examined.
Results: A literature review revealed OP in 20% of atlases, 7.69% in texts, and previous cadaver study results are 46%, 90%, and 14.9%. This study’s cadaver results reported an IR of 88.46% with an average age of 78.1 (45 – 89yrs). Radiographic results revealed 15.12% incidence with an average age 41.97 (10 – 89yrs). The average IR from 10 to 70 years was 13.32%. From 70 onwards it increased to 32.98%. The p value per decade from radiographic analysis was 0.0005.
Conclusion: This study suggests there is a high IR of an OP in cadavers (88.46%). This may be a result of the average age of cadavers 78.1 and the technique used to locate the OP. Radiographic results were significantly lower and may be explained by an age factor. Radiographs reviewed were from an emergency room where the majority of patients receiving foot radiographs were between the ages of 19 – 45. The clinical importance has been understated regarding the area of the os peroneum, which can be mistaken for styloid and Jones’ fractures.

Key words: Os peroneum, incidence, foot injury, sesamoid bone, Jones fracture, styloid process.

Accepted: January, 2011
Published: February, 2011

ISSN 1941-6806
doi: 10.3827/faoj.2011.0402.0002

The purpose of this study was to examine the important clinical relevance of the ‘os peroneum’ (OP) within the fibularis longus (FLT) by investigating the incidence of the OP between cadaveric specimens and radiological images.

The ‘os peroneum’ within the fibularis (peroneus) longus tendon has a history of controversy regarding its incidence in both individual and combined radiographic and cadaver studies. Radiographic and cadaveric studies have reported incidence rates of the OP within the FLT, however, a limited number of anatomical textbooks and atlases used by healthcare professionals and trainees mention or illustrate the OP.

Research to date reports a wide variation of incidence rates (IR), which are different in radiographic and cadaveric research. Previously Cilli, et al., in 2005 conducted a radiographic study on males only reporting 31.8% IR. A cadaveric study was conducted by Muehleman, et al., in 2009 which reported a 46% IR. Despite the various studies conducted thus far, no previous studies have conducted a comparison of incidence rates between radiographs and cadavers from separate populations. The objective of this research project was to assess radiograph incidence versus cadaver incidence rates of OP within the FLT and to assess why broad variants have occurred in previous radiograph and cadaver studies. (Fig. 1 and Fig. 2)

Figure 1 Os peroneum in the fibularis longus tendon. (Reproduced with kind permission from Lippincott Williams & Wilkins, Grant’s Anatomy, 12th edition.)

Figure 2 Location of os peroneum in the foot. (Reproduced with kind permission from Elsevier, Gray’s Anatomy, 40th edition.)


A literature search was conducted of anatomical texts and atlases, specialty texts, journals and websites regarding the presence or incidence of the OP within the FLT. Oblique foot radiographs from 1,025 individuals (range 10-89) were examined to identify OP within the FLT. (Fig. 3A and 3B) Dissection of 82 (156 sides) embalmed cadavers (52M, 30F) with a mean of 78.1 years (range 45- 89) was performed to identify the incidence rate of the OP within the FLT. A skin incision was made from the fifth toe to the styloid process distal to proximal along the lateral border of the foot. Then an oblique incision was made to the lateral malleolus to expose the FLT. (Fig. 4A)

Figure 3A and 3B Oblique radiographs of the OP in the FLT. (A and B)  (Thanks to the OHSU Radiology Department for radiograph.)

At the styloid process a horizontal incision was made along the surface of the foot to the opposite side, then exposing the FLT within the tunnel it traverses. The FLT was freed from its attachment point distally and reflected back. Palpation of the FLT was performed to identify the OP. A longitudinal incision was performed 2cm proximal and distal to the OP and then opened to reveal the ‘sesamoid bone’s’ existence or not. (Fig. 4B) A second examiner palpated and analyzed the longitudinal incision and reported their findings. A paired t-test was conducted on the radiographic data.

Figure 4A and 4B OP within the FLT in a cadaver.


The literature search revealed the OP within the FLT was discussed in anatomy texts (7.69%), contemporary atlases (20%) and specific imaging texts (16.6%). This study’s radiographic evaluation of OP within the FLT from 1,025 individuals with a mean age of 41.97 years had an incidence rate of 15.12% overall.

Incidence by ten-year increments revealed 12.16% for 10-19 years, 11.31% for 20-29 years, 13.87% for 30-39 years, 16.17% for 40-49 years, 15.15% for 50-59 years, 10% for 60-69 years, 41.38% for 70-79 years, and 19.44% for 80-89 years. The number of radiographs analyzed per ten-year increment was from approximately 100 individuals. (Graphs 1 and 2) The p value for the radiographic images was 0.0005. In this study, the incidence rate of the cadaver dissections was 88.46% with a mean age of 78.1.

Graph 1 Radiographic incidence of the OP within the FLT per ten-year increment.

Graph 2 Identification of the OP within the FLT in educational texts.


The OP is a sesamoid bone that is located within the FLT. [12] The shape of the OP can be round, oval, triangular, irregular and can also be found as bipartite or multipartite. [6,7]

The etiology of the OP is unknown; however, it has been thought that it arises from both mechanical and genetic factors. [7,11] A literature search of contemporary anatomical texts, atlases and specialty radiographic texts revealed incidence rates of 7.69%, 20% and 16.6%, respectively. This lack of recognition of the OP in commonly used texts and atlases contradicts radiographic and cadaver evidence from our study.

Our study’s incidence rate of identifying the OP within the FLT from radiographs (15.12%) was consistent with other radiographic studies. (Table 1). Radiographic studies report incidence rates of 31.8%, 4.7%, 14%, 14% and 9%. [3,4,1,11,13] The reason the incidence of the OP within the FLT in the images of our study was less than cadaveric results may be due to the average age of the individuals from the radiographs (41.97 years).

Table 1 Review of OP within the FLT in radiographic and cadaveric studies.

The radiographs reviewed were from an emergency room where on average the majority of patients receiving foot radiographs are between the ages of 19-61. [9] The incidence rate might have been higher if the average age of the individuals was higher. Another study had a mean age of 51 years. [11] Two other studies only provided the range of their subjects and none were greater than 72 years. [3,4] Two other radiographic studies did not provide any information on the mean age or range. [1,13] To collect comprehensive research on the radiographic incidence of the OP within the FLT, further data is required in the age range of 60 and up.

The IR of the OP within the FLT in the cadavers (88.46%) was consistent with one cadaveric study with an incidence rate of 90%.8 This consistency may be related to similar methods of identification of the OP within the FLT. Recent cadaveric studies have reported incidence rates of 46% and 14.9%. [7,10]

One study assessed radiographic imaging from the 33 cadavers dissected, but did not look at separate populations for radiographic and cadaveric data. [7] Furthermore, that study did not report the incidence rate of the OP within the FLT when solely palpating the FLT on cadavers; their incidence rate was reported after radiographic and histologic investigation. A combined radiographic (500 individuals) and cadaveric (20 cadavers) study showed 12.3% incidence rate; this study did not separate incidence rates for radiographic and cadaveric results. [5]

In our study, the average age of the cadavers (78.1 years) is much older than the average age of the individuals in the radiographs, which may contribute to the high incidence rate of the OP within the FLT in the cadavers. Other studies that researched incidence rate of the OP within the FLT in cadavers had mean ages that were consistent with our study (81.0, 75.2 and 77.7 years). [7,8,10] However, the age range (33-97 years) was only given for one of these studies. [8]

The method of identification may also contribute to the high incidence rate of the OP within the FLT because our study palpated and dissected open the OP, but did not use histology or radiology to confirm presence of OP from cadavers.

A conflict between cadaveric and radiographic results is the fact that cadaver incidence can be based on a partially ossified OP. Therefore a partially ossified OP could be recorded as positive. In contrast, an incomplete ossification may not be obvious or present on typical radiographs. A partially ossified OP can be cartilaginous and at times cartilage cannot be recognized on ordinary (not over or under penetrated images) radiographic images. [12]

A possible factor affecting the radiographic results of our study is that we used only those patients who presented to the emergency room with foot pain. It is not common for people over age 60 to present to the emergency room with sprained ankles or fractured 5th metatarsals. The over 60 age group population present acutely with hip fractures or with gout of the great toe. There may have been different results if we had randomly chosen from the general population for OP in the FLT.


The clinical importance has been understated regarding the area of the os peroneum, which can be mistaken for styloid and Jones’ fractures. The radiograph IR was always over 10% regardless the age group while the cadaveric incidence rate was 88.46%. This suggests that teaching the OP in the FLT is clinically relevant because lower limb injuries are common.


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3. Cilli F, Akcaoglu M. The incidence of accessory bones of the foot and their clinical significance. Acta Orthop Traumatol Turc 2005 39: 243-246.
4. Coskun N, Yuksel M, Cevener M, Arican RY, Ozdemir H, Bircan O, Sindel T, Ilgi S, Sindel M. Incidence of accessory ossicles and sesamoid bones in the feet: a radiographic study of Turkish subjects. Surg Radiol Anat 2009 31: 19-24.
5. Le Minor JM. Comparative anatomy and significance of the sesamoid bone of the peroneus longus muscle (os peroneum). J Anat 1987 151: 85-99.
6. Mellado JM, Ramos A, Salvadó E, Camins A, Danús M, Saurí A. Accessory ossicles and sesamoid bones of the ankle and foot: imaging findings, clinical significance and differential diagnosis. Eur Radiol 2003 13: L164-L177.
7. Muehleman C, Williams J, Bareither ML. A radiologic and histologic study of the os peroneum: prevalence, morphology, and relationship to degenerative joint disease of the foot and ankle in a cadaveric sample. Clin Anat 2009 22: 747-754.
8. Oydele O, Maseko C, Mkasi N, Mashanyana M. High incidence of the os peroneum in a cadaver sample in Johannesburg, South Africa: possible clinical implication? Clin Anat 2006 19: 605-610.
9. Reason for Visits to Emergency Room – National Hospital Ambulatory Medical Care Survey 1998-2006. U.S. Department of Health and Human Services; Centers for Disease Control and Prevention; National Center for Health Statistics.
10. Rühli FJ, Solomon LB, Henneberg M. High prevalence of tarsal coalitions and tarsal joint variants in recent cadavers sample and its possible significance. Clin Anat 2003 16: 411-415.
11. Sarin VK, Erickson GM, Giori NJ, Bergman AG, Carter DR. Coincident development of sesamoid bones and clues to their evolution. Anat Rec 1999 257: 174-180.
12. Stranding, S. Gray’s Anatomy: The Anatomical Basis of Clinical Practice, 40th ed. Philadelphia: Elsevier 2005, pg 1420.
13. Tsuruta T, Shiokawa Y, Kato A, Matsumoto T, Yamazoe Y, Oike T, Sugiyama T, Saito M. Radiological study of the accessory skeletal elements in the foot and ankle (abstract). J Jap Orthop Assoc 1981 55: 357-370.

Address correspondence to: Oregon Health & Science University
611 SW Campus Drive, Portland, OR 97239.

1 Department of Surgery, Department of Integrative Biosciences, Department of Orthopaedic Surgery & Rehabilitation, Department of Oral Maxillofacial Surgery, Oregon Health & Science University, Portland, OR.
2 Department of Integrative Biosciences, Oregon Health & Science University, Portland, OR.

© The Foot and Ankle Online Journal, 2011

Rigid Stabilization of Partial Incongruous Lisfranc Dislocations: A Cannulated Solid Screw Technique

by Dane K. Wukich, MD1 , Dekarlos M. Dial, DPM2

The Foot and Ankle Online Journal 2 (6): 4

For several years, there has been much controversy over the optimal form of fixation in the operative treatment of Lisfranc injuries. Both cortical and cannulated screws have been widely used in the treatment of these injuries. The level of technical difficulty and reproducible accuracy of the cannulated screw system has gained much popularity. In comparison, the rigid stability of cortical screws appears more favorable. The authors present a cannulated technique utilizing a single 4.0 mm cortical screw (Synthes USA Paoli, Pa.). In Lisfranc injuries with partial incongruity, this method allows precise screw placement while maintaining rigid solid screw stabilization. The technique is minimally invasive, provides anatomical restoration and allows early return to functional activity.

Key Words: Lisfranc injury, midfoot fracture, cannulated screw, foot sprain, dislocation.

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 and Ankle Online Journal (www.faoj.org)

Accepted: May, 2009
Published: June, 2009

ISSN 1941-6806
doi: 10.3827/faoj.2009.0206.0004

Lisfranc fracture dislocations account for 0.2 % of all fractures.[1,2] In 1909, Quenu and Kuss described the first classification system for Lisfranc injuries. [3] This classification system was modified by Hardcastle in 1982. [2] In 1986, Myerson, et al., [5] further modified the Hardcastle classification into medial and lateral dislocations. Type A is a total incongruent tarsometatarsal joint complex. Types B1 is a partial incongruity with medical displacement affecting the first ray or first metatarsal, and B2 is partial incongruity resulting in lateral displacement of one or more lesser metatarsals. The most common Lisfranc injury in the athlete is the partial incongruous injury resulting in medial or lateral dislocation. [4,17,18,19]

Types C1 and C2 injuries result in partial or total displacement respectfully. Such classification systems allow communication between surgeons, but offer no prognostic value.

Stable anatomic reduction and internal fixation for Lisfranc fracture dislocations has been described using both screw and Kirchner wire fixation. Pin migration, infection and loss of reduction have been reported with Kirchner wire fixation. [11] Over the years, screw fixation has emerged as the superior fixation method for stabilizing the medial three tarsometatarsal joints. [1,6,7,8,9,10] Some disadvantages of screw fixation include articular cartilage damage and screw breakage. [12]

The authors share their surgical technique for addressing partial incongruous Lisfranc injuries; lateral displacement injuries affecting only the second metatarsal.

The technique is also useful for patients with a symptomatic subtle diastasis. This technique allows direct visualization of the injury, anatomical reduction, articular cartilage preservation, and rigid solid screw stabilization. The technique is avoided in patients with neuropathic arthropathy, peripheral vascular disease, insensate feet, open physis, complex dislocations, and open fracture dislocations.

Surgical Technique

Patients with partially incongruent Lisfranc injuries (B2) were treated with internal fixation utilizing 4.0 mm solid screw (Synthes®, Paoli, Pa. USA).

Pre-operatively, all patients receive a regional popliteal and femoral block. With the patient lying supine, a stress abduction stress is applied to the midfoot as described by Coss, et al.13 Stress radiography may obviate the need for stabilization of the entire tarsometatarsal joint complex. (Figs. 1 and 2)

Figure 1 Bilateral comparison weightbearing anteroposterior radiographs demonstrating partial incongruity of the second tarsometatarsal joint. Note the arrow identifying the first and second metatarsal base diastasis.

Figure 2  Oblique foot radiograph. Note the collinear relationship at the adjacent tarsometatarsal joints.

The neurovascular bundle is identified by palpating the dorsalis pedis artery prior to inflation of the calf tourniquet. The interval between the first and second metatarsal bases is verified under fluoroscopy. (Fig. 3) A single 6cm longitudinal skin incision is made medial to the neurovascular bundle.

Figure 3 Freer elevator used prior to guide incision placement to identify the interval between the first and second metatarsal cuneiforms.

Blunt dissection is continued down to the level of the extensor hallucis brevis muscle tendon (EHB). The EHB tendon is retracted laterally.

Subperiosteal dissection at the intercuneiform level is extended just distal to the first and second metatarsal base articulation. The Lisfranc ligament integrity is evaluated. A ruptured Lisfranc ligament, fibrotic tissue and or bone fragments can impede anatomical reduction and must be debrided and or excised. Care is taken to protect the neurovascular bundle and deep plantar artery.

Reduction is performed by placing a bone tenaculum around the medial cuneiform and second metatarsal base. The second metatarsal is reduced to the medial border of the middle cuneiform. The reduction is verified under fluoroscopy. (Fig. 4) If a diastasis remains present, first and second metatarsal base interval is inspected and the bone reduction tenaculum is adjusted. Once reduction of the diastasis is achieved, a 1.2 mm guide wire is obtained from the cannulated 3.5/4.0mm Synthes screw set.

Figure 4  A bone tenaculum is utilized to reduce the first and second metatarsal base diastasis.

The 1.2 mm guide wire is placed obliquely from the medial cuneiform (plantar proximal) into the second metatarsal base (distal dorsal) penetrating the lateral cortex. (Fig. 5)

Figure 5  The 1.2 mm guide wire is placed from medial to lateral just penetrating the second metatarsal base lateral cortex.

The knee is then flexed and the guide wire position is verified under fluoroscopy on both an anteroposterior and lateral projection. The medial cuneiform is then countersunk to prevent screw irritation. Next, the cannulated depth gauge is used to determine the screw length. The 1.2mm guide wire is advanced until it exits the foot dorsolaterally. (Fig. 6A and 6B).


Figures 6A and 6B The 1.2 mm guide wire is advanced obliquely until exiting the foot laterally allowing retrieval if breakage occurs. (A)  Note the direction of the guide wire in both the transverse and sagittal planes. (B)

By exiting the foot in this fashion, it allows easy retrieval of the guide wire if breakage occurs. The cannulated 2.5 mm drill bit is utilized to drill over the guide wire and penetrates the lateral second metatarsal base cortex. (Fig. 7) The appropriate length 4.0 mm screw is obtained and inserted in line with the guide wire. (Fig. 8A and 8B) To prevent toggle or misdirection during insertion, the guide wire is removed simultaneously.

Figure 7 The 2.5 mm cannulated drill is used to drill over the 1.2mm guide wire penetrating the lateral 2nd metatarsal base cortex.


Figures 8A and 8B 4.0 mm cortical screw (Synthes® Paoli, Pa. USA) (A)  Simultaneous placement of the 4.0mm solid screw and removal of the 1.2mm guide wire. (B)

The bone reduction tenaculum is disengaged and removed. Screw placement and stability are verified under fluoroscopy. (Fig. 9) The calf tourniquet is deflated and hemostasis achieved prior to closure.

Figure 9 Intraoperative anteroposterior image demonstrating diastasis reduction and 4.0mm solid screw fixation.

Postoperatively, patients are placed into a Jones compression dressing with a posterior splint for until postoperative day seven. The patient is then transferred into a short leg non-weightbearing cast for 14 days. At day 21 the sutures are removed and another short leg cast is applied. Patients are non-weightbearing for a further 6 weeks, followed by protected weight bearing in a walking boot with progression to normal shoe gear as tolerated.


The standard treatment for Lisfranc joint injuries is to achieve anatomic reduction with internal fixation. Stable anatomical reduction results in more favorable long-term outcomes. [4,7,14] Buzzard, et al., reported that optimal results are obtained if precise anatomical reduction is achieved.1 Kuo, et al., reported that stable anatomic reduction lead to better long-term outcomes with higher AOFAS midfoot scores. [7]

Current recommendations support screw fixation in treatment of Lisfranc injuries. However, screw fixation is not without complications. Some authors believe that screw fixation results in articular cartilage damage during and that there is added risk for screw breakage. [15] Our technique preserves cartilage congruity and provides stabilization until ligamentous healing is restored. Thodarson, et al., found that absorbable screw fixation (PLA screws) was safe and eliminated the need for removal. [5]

In a cadaveric study, dorsal plating versus screw fixation showed no difference in resisting tarsometatarsal joint displacement. [15]

Ly, et al., recommended primary stable arthrodesis of purely ligamentous Lisfranc dislocations. The authors reviewed 41 patients with an average follow-up of 42 months. The average post-operative AOFAS score was 68.6 points in the open reduction and internal fixation group and 88 points for the arthrodesis group. The authors believe that stable arthrodesis prevented loss of correction and degenerative changes. [16]

The cannulated solid screw technique is an alternative treatment option for partial incongruous injuries or subtle dislocations. Excessive fixation is avoided while preserving articular congruity. The rigidity of the 4.0 mm solid screw allows early functional rehabilitation. Retrospectively, the authors have treated 10 patients using this technique with a zero incidence of hardware fatigue or breakage.

In conclusion, our technique has proven to be effective for stabilizing partial incongruous Lisfranc injuries. The technique is reproducible, accurate and allows for early function rehabilitation. The rigidity of the 4.0 mm solid screw provides optimal stability. However, care must be taken to individualize this treatment based on the extent of tarsometatarsal joint displacement.


1. Buzzard BM, Manos, RE, Buoncristiani A, Mills WJ: Surgical management of acute tarsometatarsal fracture dislocation in the adult. Clin Orthop 353: 125 – 133, 1998.
2. Hardcastle PH, Reschauer R, Kutscha-Lissberg E, Schoffmann W: Injuries to the tarsometatarsal joint. Incidence, classification and treatment. J Bone and Joint Surg 64B (3): 349 – 356, 1982.
3. Quenu E, Kuss G: Etude sur les luxations du metatarse (luxations metatarso-tariennes). Rev Chir 39: 281 – 336, 1909.
4. Myerson MS, Fisher RT, Burgess AR, Kenzora JE. Fracture dislocations of the tarsometatarsal joints: end results correlated with pathology and treatment. Foot Ankle Clinics 6: 225 – 242, 1986.
5. Thodarson DB, Hurwitz G: PLA screw fixation of Lisfranc Injuries. Foot Ankle Int 23: 1003 – 1007, 2002.
6. Myerson MS: The diagnosis and treatment of injury to the tarsometatarsal joint complex. J Bone Joint Surg 81B: 756 – 763, 1999.
7. Kuo R, Tejwani N, DiGiovanni CW, Holt SK, Benirschke SK, Hansen ST Jr, Sangeorzan BJ: Outcome after open reduction and internal fixation of Lisfranc joint injuries. J Bone Joint Surg 82A: 1609 – 1618, 2000.
8. Arntz CT, Hansen ST: Dislocations and fracture dislocations of the tarsometatarsal joints. Orthop Clin North Am 18: 105 – 114, 1987.
9. Bloome DM, Clanton TO: Treatment of Lisfranc injuries in the athlete. Tech. Foot Ankle Surg 1: 94 – 101, 2002.
10. Chiodo C, Myerson M: Developments and advances in the diagnosis and treatment of injuries to the tarsometatarsal joint. Orthop Clin North Am 32: 11 – 20, 2001.
11. Arntz CJ, Veith RG, Hansen Jr. ST: Fractures and fracture dislocations of the tarsometatarsal joints. J Bone Joint Surg 70A (2): 173 – 181, 1988.
12. Alberta FG, Aronow MS, Barrero M, Diaz-Doran V, Sullivan RJ, Adams DJ: Ligamentous Lisfranc joint injuries: A biomechanical comparison of dorsal plate and transarticular Screw Fixation. Foot Ankle Int 26: 462 – 473, 2005.
13. Coss HS, Manos RE, Buoncristiani A, Mills W: Abduction stress and AP weightbearing radiography of purely ligamentous injury in the tarsometatarsal joint. Foot Ankle Int 19: 537 – 541, 1998.
14. Goosens M, De Stoop N: Lisfranc’s fracture-dislocations: etiology, radiology, and results of treatment. Clin Orthop 176: 165 – 162, 1983.
15. Alberta FG, Aronow MS, Barrero M, Diaz-Doran V, Sullivan RJ, Adams DJ: Ligamentous Lisfranc joint injuries: a biomechanical comparison of dorsal plate and transarticular screw fixation. Foot Ankle Int. 26: 462 – 472, 2005.
16. Ly V, Coetzee JC: Treatment of primary ligamentous Lisfranc joint injuries: primary arthrodesis compared with open reduction and internal fixation. J Bone Joint Surg 88A: 514 – 520, 2006.
17. Davies MS, Saxby TS: Intercuneiform instability and the “gap sign”. Foot Ankle Int 20: 606 – 609, 1999.
18. Meyer SA, Callaghan JJ, Albright JP, Crowley ET, Powell JW: Midfoot sprains in collegiate football players. Am J Sports Medicine 22: 392-401, 1994.
19. Shapiro MS, Wascher DC, Finerman GA: Rupture of Lisfranc’s ligament in athletes. Am J Sports Med 22: 687 – 691, 1994.

Address correspondence to: Dekarlos M. Dial, DPM, Cornerstone Foot and Ankle Specialists, 1814 West Chester Drive, Suite 300, High Point, North Carolina 27262

Chief, Foot and Ankle Division, Department of Orthopaedic Surgery; University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
3rd year resident, Department of Graduate Medical Education; University of Pittsburgh Medical Center Surgery, Pittsburgh, Pennsylvania.

© The Foot and Ankle Online Journal, 2009

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.


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]


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.


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