20 Vascular Pathology of the Hand and Upper Extremities, including Kienbock’s Disease

Ariel Williams and Lauren Lee Smith

Abstract

Vascular pathology in the hand and upper extremity encompasses a broad range of conditions, some resulting in carpal osteonecrosis and mechanical wrist pain, others in insufficient blood flow to the digits with resultant ischemia and ulceration. Although hand surgeons have gained a far better understanding of the pathophysiology of these conditions over the last few decades, many mysteries remain.

This chapter provides an overview of the most notable vascular conditions affecting the hand, from the very common (Raynaud’s disease) to the very rare (digital artery aneurysm). Specific subjects include Kienbock’s disease, Preiser’s disease, hypothenar hammer syndrome, Raynaud’s phenomenon, thromboangiitis obliterans or “Buerger’s disease,” and digital artery aneurysm.

Peripheral vascular disease – Kienbock’s disease – Preiser’s disease – avascular necrosis – hypothenar hammer – Raynaud’s disease – thromboangiitis obliterans – aneurysm

20 Vascular Pathology of the Hand and Upper Extremities, including Kienbock’s Disease

I. Kienbock’s Disease

A. Introduction

Kienbock’s disease = lunate osteonecrosis.
Results in predictable sequence of lunate necrosis, fragmentation, and collapse, eventually leading to carpal malalignment and arthritis.
Most commonly seen in 20- to 40-year-old males, although has been described in pediatric patients and older adults.
Although it is less common, prognosis best in children and elderly.

B. Pathophysiology

True cause unknown—likely multifactorial.
A number of biomechanical and anatomic factors have been identified as playing a possible role although their true contribution is unknown:
- History of repetitive wrist trauma.
- Negative ulnar variance.
- Decreased radial inclination.
- Lunate geometry.
- Differences in lunate vascularity.

C. Examination

Dorsal wrist pain related to activity.
Tenderness over lunate (palpate dorsally).
Wrist swelling.
Decreased wrist range of motion (ROM).
Decreased grip strength.

D. Imaging

1. Radiographs

Three views of wrist should be obtained.
Radiographs demonstrate a predictable pattern of disease evolution over time.
The Lichtman’s classification is the most frequently used staging system (► Table 20.1). Stages 0 and IIIC have recently been added.
Correct staging of the disease is critical to guide treatment choice.
Stage II and stage III are distinguished by the collapse of the lunate in stage III.
The scaphoid ring sign (► Fig. 20.1) and radioscaphoid angle (► Fig. 20.2) may be useful in differentiating between stages IIIA and IIIB.

**Fig. 20.1** Patient with stage IIIB Kienbock’s disease and a scaphoid ring sign, which indicates flexion and malrotation of the scaphoid.

**Fig. 20.2** Measurement of the radioscaphoid angle (RS) in a patient with Kienbock’s disease. A radioscaphoid angle of greater than 60 degrees is an indicator of stage IIIB disease.

Table 20.1 Lichtman’s classification of Kienbock’s disease
Stage	Radiographic findings
0	X-ray and MRI normal
I	X-ray normal; edema within lunate on MRI
II	Lunate sclerosis
IIIA	Lunate collapse without malalignment
IIIB	Lunate collapse; scaphoid malrotation and proximal capitate migration
IIIC	Lunate is fragmented or fractured in coronal plane
IV	Arthrosis
Abbreviation: MRI, magnetic resonance imaging.

2. Magnetic Resonance Imaging (MRI)

May be performed with or without contrast.
Changes correspond to Lichtman’s staging system.
Detects early disease while X-rays are still negative (Lichtman stage I):
- Findings: Lunate signal decreases on T1 sequences, variable on T2 sequences.
Gadolinium can be helpful. Unlike necrotic tissue, neovascular tissue enhances on T1 images when gadolinium is used. When observed, this may indicate a better prognosis for healing than when such enhancement does not occur.
MRI can also demonstrate integrity of lunate cartilage shell.

3. Arthroscopy

Can evaluate the articular surfaces of the lunate, radius, and capitate to support the decision to proceed with reconstructive versus salvage surgery.

E. Treatment

Treat children nonoperatively as outcomes are excellent.
For adults, treatment depends on disease stage.
Multiple treatment options exist. However, there is controversy regarding which procedures are best, especially in stage III disease.
Nonsteroidal anti-inflammatory drug (NSAIDs), activity modification, and immobilization are all useful initial management at any stage.
Surgical treatments include revascularization procedures, unloading procedures, and salvage procedures.
Revascularization and unloading procedures are most effective in Lichtman stage 0, I, and II disease. They are less successful in stage III disease and contraindicated in stage IV disease.
Revascularization procedures:
- Aim to facilitate lunate recovery by restoring blood supply.
- Distal radius core decompression:
  - Drill distal radius to increase local blood flow.
- Lunate decompression:
  - Drill lunate to relieve venous congestion.
- Vascularized bone grafts:
  - Pedicled: 4 and 5 extensor compartment artery or 2nd or 3rd dorsal metacarpal arteries.
  - Free: Vascularized iliac crest, medial femoral trochlea.
- An unloading procedure can be performed at the same time to optimize conditions for lunate recovery. Alternatively, K-wires or an external fixator can be used to temporarily unload the lunate while healing takes place.
Unloading procedures:
- Aim to facilitate lunate recovery by altering biomechanical forces.
- Radial shortening osteotomy:
  - Ulnar negative patients only.
- Capitate shortening osteotomy:
  - Patients who are ulnar neutral or ulnar positive.
Salvage procedures:
- Lichtman stages III and IV.
- Aim to reduce pain once the lunate is no longer viable.
  - Procedure choice based upon patient’s age and extent of surrounding deformity, arthrosis.
- Proximal row carpectomy.
- Partial wrist arthrodesis (lunate excision scaphocapitate fusion or scaphotrapeziotrapezoid [STT] fusion).
- Pyrocarbon lunate replacement.
- Wrist fusion or arthroplasty.
  - Pan-carpal arthrosis.