Calcifying Tendonitis

Dupuytren contracture results from contracture of the palmar fascia within the hand, possibly resulting in a fixed flexion deformity of the metacarpophalangeal (MCP) joints and the proximal interphalangeal (PIP) joints. This condition usually affects the fourth and fifth digits (the ring and small fingers). (See the images below.)
Arrow denotes the cord often present in Dupuytren contracture. Metacarpophalangeal joint and proximal interphalangeal joint contractures are also present. This photo shows a patient with an inability to extend the fourth and fifth digits. The differential diagnosis includes Dupuytren contracture, which is a flexion contracture most commonly involving digits 4 and/or 5. The condition is a fibrosing disorder that results in slowly progressive thickening and shortening of the palmar fascia, leading to the debilitating digital contractures. Dupuytren contracture belongs to the group of fibromatoses that include plantar fibromatosis (Ledderhose disease), penile fibromatosis (Peyronie disease), and fibromatosis of the dorsal proximal interphalangeal (PIP) joints (Garrod nodes or knuckle pads).^[1] Although many cases appear to be idiopathic and without coexisting conditions, a variety of associated diseases have been reported. (See Etiology.)
Dupuytren contracture is most commonly observed in persons of Northern European descent and affects 4-6% of whites worldwide.^[2] Many individuals have bilateral disease (45%); in unilateral cases, the right side is more often affected.^[3] The ring finger is most commonly involved, followed by the fifth digit and then the middle finger. The index finger and the thumb are typically spared. (See Epidemiology and Presentation.)
Although the cause of Dupuytren disease is unknown, a family history is often present. Males are 3 times as likely to develop disease and are more likely to have higher disease severity.^{[4, 5]} Male predominance may be related to expression of androgen receptors in Dupuytren fascia. (See Etiology and Epidemiology.)^[6]
Additional risk factors include manual labor with vibration exposure, prior hand trauma, alcoholism, smoking, diabetes mellitus, hyperlipidemia, Peyronie disease, and complex regional pain syndrome.^[7] Rheumatoid arthritis seems to protect against the development of Dupuytren disease. (See Etiology.)
Therapies include conservative medical and surgical modalities. Although the condition is not fatal, significant morbidity can occur if patients remain untreated. (See Prognosis, Treatment, and Medication.)

Stages of Dupuytren disease

Dupuytren disease occurs in the following 3 stages:

• Proliferative phase - Local fascial fibroplasia and development of a nodule, in which myofibroblasts proliferate, occur (see the image below), with palmar skin blanching on finger extension; in early disease, some patients may report tenderness and discomfort associated with the nodules
• Involutional phase - Contracture develops, with associated nodular thickening of the palmar fascia; myofibroblasts are predominant during this phase and align themselves along tension lines within the nodule
• Residual phase - The nodular tissue disappears, leaving acellular tissue and thick bands of collagen; the ratio of type III collagen to type I collagen increases, which is the reverse of the normal pattern in the palmar fascia^[8]

Grades of severity

The grading system for Dupuytren disease severity is as follows (see the images below)^[9] :

• Grade 1 - Thickened nodule and band in the palmar aponeurosis; may have associated skin abnormalities
• Grade 2 - Development of pretendinous and digital cords with limitation of finger extension
• Grade 3 - Presence of flexion contractureArrow denotes the cord often present in Dupuytren contracture. Metacarpophalangeal joint and proximal interphalangeal joint contractures are also present. Arrow denotes the typical cords of Dupuytren contracture. These cords are usually painless. Note the metacarpophalangeal joint contracture. This photo demonstrates the presence of a nodule as well as skin blanching with extension of the affected digits. Three clinical grades of Dupuytren disease.

Overview of Rheumatoid Spondylitis

The most common sites of rheumatoid arthritis (RA) are the metatarsophalangeal joints, followed by the metacarpophalangeal joints and the cervical spine (ankylosing spondylitis, rheumatoid spondylitis). Much of the understanding of spinal afflictions in rheumatoid arthritis was advanced by studies published in the 1950s and 1960s.^[1] In 1951, Davis and Markley detailed medullary compression as a cause of death in patients with rheumatoid arthritis.^[2] In 1969, Mathews reported that 25-30% of patients with rheumatoid arthritis who were admitted to the hospital had radiographic evidence of cervical spine involvement.^[3]
Rheumatoid spondylitis (ankylosing spondylitis) primarily affects the cervical spine. Affliction of the thoracic or lumbar spine is rare. The anatomic abnormalities occur as a consequence of the destruction of synovial joints, ligaments, and bone. Abnormalities of the rheumatoid cervical spine generally can be grouped into 3 categories that may be seen in isolation or combined involvement, of which atlantoaxial instability (AAI) or atlantoaxial subluxation (AAS) is the most common abnormality (see the image below). AAS can be a fixed deformity or can be partially or fully reducible.
Rheumatoid spondylitis. Depiction of anterior subluxation of C1 on C2, retrodental pannus, and osseous erosions; the spinal cord is compressed between the pannus anteriorly and the posterior arch of the atlas. Superior migration of the odontoid (SMO) is the next most common abnormality and has alternately been referred to as cranial settling, pseudobasilar invagination, or vertical/upward translocation of the odontoid (see the following images).^[4] The third and least common deformity is subaxial subluxation, which may be seen at multiple levels and produces a stepladder deformity.
Rheumatoid spondylitis. Depiction of superior migration of the odontoid into the foramen magnum with compression of the spinal cord. Rheumatoid spondylitis. Pertinent measurements of superior migration of the odontoid; cranial migration distance (CMD).

For patient education information, see eMedicine's Arthritis Center, as well as Rheumatoid Arthritis and Rheumatoid Arthritis Medications.

Go to Rheumatoid Arthritis and Ankylosing Spondylitis for more information on these topics.

Bursitis

Bursae are saclike structures between skin and bone or between tendons, ligaments, and bone. They are lined by synovial tissue, which produces fluid that lubricates and reduces friction between these structures. Bursitis occurs when the synovial lining becomes thickened and produces excessive fluid, leading to localized swelling and pain.^{[1, 2, 3]}
Olecranon bursitis, shown here with the elbow flexed. Image courtesy of UMDNJ-New Jersey Medical School, www.DoctorFoye.com, and www.TailboneDoctor.com. Olecranon bursitis aspiration of a hemorrhagic effusion. Image courtesy of UMDNJ-New Jersey Medical School, www.DoctorFoye.com, and www.TailboneDoctor.com. Location of pes anserine bursa on the medial knee. MCL is medial collateral ligament.

Recent studies

In a study of 25 cases of postarthroplasty trochanteric bursitis requiring corticosteroid injection, Farmer et al found that corticosteroid injections were effective treatment and that nonoperative management may be more likely to fail in young patients and patients with leg-length discrepancies. Of the 25 hips, 11 required multiple corticosteroid injections, and symptoms resolved in 20 but not in 5 cases.^[4]
Pretell et al described distal "Z" lengthening of the fascia lata in 13 hips and reported that 12 of the 13 patients reported good results. According to the authors, this technique is less aggressive and can be performed under local anesthesia with little morbidity and disability. Mean surgical time for the procedure was 15 minutes, and 1 seroma was reported as a complication.^[5]
According to Martinez-Taboada et al, in patients with severe septic bursitis, but without extensive cellulitis, aspiration plus I.V. cloxacillin may be sufficient treatment, and in patients with more severe cases of septic bursitis, aspiration along with cloxacillin plus gentamicin may be appropriate in the majority. The investigators studied 82 patients with severe septic bursitis, and the most frequent bacterium isolated was Staphylococcus aureus

Scleroderma

Scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin). Hippocrates first described this condition as thickened skin.^[1] Carlo Curzio (1752) offered the first detailed description of scleroderma in a patient with hard skin, which he described as woodlike or as containing a dry hide. In 1836, Giovambattista Fantonetti applied the term scleroderma to a patient's condition.^[2] He applied the term to describe a patient with dark leatherlike skin who exhibited a loss of range of joint motion due to skin tightening. Robert H. Goetz first described in detail the concept of scleroderma as a systemic disease in 1945; he introduced the term progressivesystemic sclerosis  to emphasize the systemic and often progressive nature of the disease.

Definition

The term systemic sclerosis is used to describe a systemic disease characterized by skin induration and thickening accompanied by various degrees of tissue fibrosis and chronic inflammatory infiltration in numerous visceral organs, prominent fibroproliferative vasculopathy, and humoral and cellular immune alterations.
The American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis require one major criterion or two minor criteria, as follows:

• Major criterion: Proximal scleroderma is characterized by symmetric thickening, tightening, and induration of the skin of the fingers and the skin that is proximal to the metacarpophalangeal or metatarsophalangeal joints. These changes may affect the entire extremity, face, neck, and trunk (thorax and abdomen; see images below). Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles. Sclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair.
• Minor criteria

  • Sclerodactyly is characterized by thickening, induration, and tightening of the skin, limited to only the fingers.
  • Digital pitting scars or a loss of substance from the finger pad: As a result of ischemia, depressed areas of the fingertips or a loss of digital pad tissue occurs.
  • Bibasilar pulmonary fibrosis includes a bilateral reticular pattern of linear or lineonodular densities most pronounced in basilar portions of the lungs on standard chest roentgenography. These densities may assume the appearance of diffuse mottling or a honeycomb lung and are not attributable to primary lung disea

Sjogren Syndrome

Sjögren syndrome is a systemic chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs. Most individuals with Sjögren syndrome present with sicca symptoms, such as xerophthalmia (dry eyes), xerostomia (dry mouth), and parotid gland enlargement, which is seen in the image below. (See Presentation.)
Marked bilateral parotid gland enlargement in a patient with primary Sjögren syndrome. Sicca syndrome is a common clinical finding. In addition, numerous extraglandular features may develop, such as arthralgia, arthritis, Raynaud phenomenon, myalgia, pulmonary disease, gastrointestinal disease, leukopenia, anemia, lymphadenopathy, neuropathy, vasculitis, renal tubular acidosis, and lymphoma. About 50% of patients with Sjögren syndrome have cutaneous findings, such as dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria.^[1] (See Etiology, Presentation, and Workup.)
Primary Sjögren syndrome occurs in the absence of another underlying rheumatic disorder, whereas secondary Sjögren syndrome is associated with another underlying rheumatic disease, such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), or scleroderma. Given the overlap of Sjögren syndrome with many other rheumatic disorders, it is sometimes difficult to determine whether a clinical manifestation is solely a consequence of Sjögren syndrome or is due to one of its overlapping disorders.
Importantly, classic clinical features of Sjögren syndrome may also be seen in viral infections with hepatitis C, human immunodeficiency virus (HIV), and human T-cell lymphotrophic virus (HTLV). Treatment for Sjögren syndrome is largely based on symptoms, but patients must be monitored carefully for the potential development of lymphoma. (See DDx.)

American-European Consensus Group classification

A number of classification criteria for Sjögren syndrome were designed primarily for clinical research studies but are also often used to help guide clinical diagnoses. The American-European Consensus Group’s criteria for the classification of Sjögren syndrome are outlined below.^{[2, 3]} These are the most currently accepted criteria for the diagnosis of Sjögren syndrome. These criteria allow a diagnosis of Sjögren syndrome in patients without sicca symptoms or who have not undergone a biopsy.
According to the American-European classification system (as modified by Tzioufas and Voulgarelis^[4] ), diagnosis of primary Sjögren syndrome requires 4 of 6 of the below criteria; in addition, either criterion number 5 or criterion number 6 must be included. Sjögren syndrome can be diagnosed in patients who have no sicca symptoms if 3 of 4 objective criteria are fulfilled. The criteria are as follows:

• Ocular symptoms - Dry eyes for more than 3 months, foreign-body sensation, use of tear substitutes more than 3 times daily
• Oral symptoms - Feeling of dry mouth, recurrently swollen salivary glands, frequent use of liquids to aid swallowing
• Ocular signs - Schirmer test performed without anesthesia (< 5 mm in 5 min), positive vital dye staining results
• Oral signs - Abnormal salivary scintigraphy findings, abnormal parotid sialography findings, abnormal sialometry findings (unstimulated salivary flow < 1.5mL in 15min)
• Positive minor salivary gland biopsy findings
• Positive anti–SSA or anti–SSB antibody results

Secondary Sjögren syndrome is diagnosed when, in the presence of a connective-tissue disease, symptoms of oral or ocular dryness exist in addition to criterion 3, 4, or 5, above.
Application of these criteria has yielded a sensitivity of 97.2% and a specificity of 48.6% for the diagnosis of primary Sjögren syndrome. For secondary Sjögren syndrome, the specificity is 97.2% and the sensitivity, 64.7%.^[5]
Exclusion criteria include past head-and-neck irradiation, hepatitis C infection, acquired immunodeficiency syndrome (AIDS), prior lymphoma, sarcoidosis, graft versus host disease, and the use of anticholinergic drugs.

Complications

Complications related to Sjögren syndrome include the following (see Prognosis, Treatment, and Medication):

• Emergence of disorders associated with Sjögren syndrome, such as SLE and RA
• Infection of the parotid gland, typically staphylococcal, streptococcal, or pneumococcal - Clues include unilateral worsening of symptoms, along with tenderness, warmth, and erythema
• Emergence of parotid tumors - Watch for unusually hard or unilateral parotid enlargement
• Pregnant patients with antiRo/SS-A antidodies are at risk for fetal loss, complete heart block in the fetus ,and neonatal lupus syndrome in the newborn
• Emergence of pseudolymphomas (pleomorphic cells that do not meet the criteria for malignancy) andnon-Hodgkin B-cell lymphomas (see the image below)^[4] Clinical photograph and photomicrograph of a 48-year-old man with Sjögren syndrome with a large left parotid mass. On biopsy, B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type was identified. Microscopic section of parotid biopsy, stained with immunoperoxidase for kappa light chains (brown-stained cells), showed monoclonal population of B cells, confirming the diagnosis.

Leukocytoclastic Vasculitis

Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis and hypersensitivity angiitis, is a histopathologic term commonly used to denote a small-vessel vasculitis (see image shown below). Leukocytoclastic vasculitis has many causes, but no cause is identified in up to 50% of patients with this condition.
Histopathology of leukocytoclastic vasculitis. Leukocytoclastic vasculitis may be localized to the skin or may manifest in other organs. The internal organs affected most commonly include the joints, the gastrointestinal tract, and the kidneys. The prognosis is good in the absence of internal involvement.
Leukocytoclastic vasculitis may be acute or chronic. Chronic disease that primarily involves the skin should be treated with nontoxic modalities whenever possible, avoiding the use of systemic corticosteroids and immunosuppressive agents.
For additional information on cutaneous manifestations of leukocytoclastic vasculitis, see the eMedicine articleHypersensitivity Vasculitis (Leukocytoclastic Vasculitis)  in the Dermatology volume.

Polyarteritis Nodosa

Classicpolyarteritis nodosa (PAN or c-PAN) is a systemic vasculitis characterized by necrotizing inflammatory lesions that affect medium-sized and small muscular arteries, preferentially at vessel bifurcations, resulting in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction.
Kussmaul and Maier first described PAN in 1866. The autopsy of a patient with fever, weight loss, abdominal pain, and polyneuropathy revealed areas of focal inflammatory exudations that gave rise to palpable nodules along the course of medium-sized arteries.^[1] PAN, like other vasculitides, affects multiple systems and has protean manifestations, although it most commonly affects skin, joints, peripheral nerves, the gut, and the kidney.^[2] The lungs are usually spared with PAN. A typical PAN patient might present with fever, night sweats, weight loss, skin ulcerations or tender nodules, and severe muscle and joint pains developing over weeks or months. (See Etiology, Clinical, and Workup.)
Nonspecific, firm, tender subcutaneous nodules without livedo reticularis and/or systemic involvement may be the first sign of polyarteritis nodosa (PAN). Insight into PAN requires some understanding of how this rare disease has been defined. Periarteritis nodosa was a term used from the mid 1800s to the 1900s to describe a spectrum of systemic vasculitic disorders, including diseases that manifested as arterial aneurysms, as well as those that caused diffuse necrotizing glomerulonephritis.^{[3, 4]} The term periarteritis nodosa was changed to polyarteritis nodosa in the mid 1900s to reflect the transmural inflammation of arteries caused by this disorder.^[5]
The understanding of vasculitides continued to increase by the 1980s with the discovery of antineutrophil cytoplasmic antibodies (ANCAs). Microscopic polyangiitis
 (MPA; formerly called microscopic polyarteritis) is an ANCA-associated systemic vasculitis that has some features similar to those of classic PAN, with the additional involvement of renal glomeruli and pulmonary capillaries.

Features of PAN

The American College of Rheumatology (ACR) established criteria for research purposes in order to differentiate PAN from other forms of vasculitis.^[6] A committee of ACR physicians selected 10 disease features of PAN; in order for PAN to be diagnosed, at least 3 of the 10 ACR criteria should be present when radiographic or pathological diagnosis of vasculitis is made^[6] (See Clinical and Workup.):

• Weight loss of 4 kg or more
• Livedo reticularis
• Testicular pain/tenderness
• Myalgia or leg weakness/tenderness
• Mononeuropathy or polyneuropathy
• Diastolic blood pressure greater than 90 mm/Hg
• Elevated blood urea nitrogen (BUN) or creatinine level unrelated to dehydration or obstruction
• Presence of hepatitis B surface antigen or antibody in serum
• Arteriogram demonstrating aneurysms or occlusions of the visceral arteries
• Biopsy of small- or medium-sized artery containing polymorphonuclear neutrophils

The strong association of MPA with ANCA, as well as the pathologic and clinical differences between MPA and PAN, demonstrate that PAN and MPA are likely separate disorders. It was not until 1994 that histologic criteria to distinguish PAN from MPA were defined at the international Chapel Hill Consensus Conference (CHCC).^[7] According to the CHCC criteria, the presence of vasculitis in arterioles, venules, and capillaries defines the diagnosis of MPA (although small- and medium-sized arteries may also be involved in MPA) and excludes the diagnosis of PAN. (See Clinical, Differentials, and Workup.)

Stages

PAN is divided into subacute, acute, and chronic stages. In the subacute stage, infiltration of mononuclear cells becomes more prominent, while in the acute stage, polymorphonuclear neutrophils infiltrate all layers of the vessel wall. (See Etiology.)
In the chronic stage, fibrinoid necrosis of the vessels causes thrombosis and tissue infarction. Aneurysmal dilatations of the involved arteries, as large as 1 cm in size, are characteristic findings of PAN. Kidney lesions show predominant arteritis without glomerulonephritis; however, in patients with severe hypertension, glomerulosclerosis may be superimposed with glomerulonephritis. Pulmonary arteries are not involved, and bronchial artery involvement is uncommon.

Patient education

Patients should understand that PAN can be a progressive systemic disease, and further complications and the involvement of other organ systems are quite common. Many patients attempt to discontinue their medications after initial symptomatic improvement, owing to the potential for adverse effects. Therefore, the benefits of medical treatments should be discussed clearly with the patient, in addition to the risks associated with the long-term use of immunosuppressants. The use of these medications necessitates close monitoring for many years to come. (See Treatment and Medications.)

Amyloidosis

Amyloid is defined as in vivo deposited material distinguished by fibrillar electron micrographic appearance, amorphous eosinophilic appearance on hematoxylin and eosin staining (see the first image below), beta pleated sheet structure as observed by x-ray diffraction pattern, apple-green birefringence on Congo Red histological staining (see the second image below), and solubility in water and buffers of low ionic strength. All types of amyloid consist of a major fibrillar protein that defines the type of amyloid.^{[1, 2]}
Amorphous eosinophilic interstitial amyloid observed on a renal biopsy. Congo Red staining of a cardiac biopsy specimen containing amyloid, viewed under polarized light. Amyloidosis is a clinical disorder caused by extracellular and or intracellular deposition of insoluble abnormal amyloid fibrils that alter the normal function of tissues. Approximately 10% of amyloidosis depots consist of components such as glycosaminoglycans (GAGs), apolipoprotein E (apoE), and serum amyloid P (SAP) component, while 90% of the depots consist of the amyloid fibrils that are formed by the aggregation of misfolded proteins. These proteins either arise from proteins expressed by cells at the deposition site (localized) or precipitate systemically after production at a local site (systemic).^[3] In humans, about 23 different unrelated proteins are known to form amyloid fibrils in vivo.^[4] Many mechanisms of protein function contribute to amyloidogenesis, including "nonphysiologic proteolysis, defective physiologic proteolysis, mutations involving changes in thermodynamic or kinetic properties, and pathways that are yet to be defined."^[4]

Acute Rheumatic Fever

The incidence of acute rheumatic fever (ARF) has declined in most developed countries, and many physicians have little or no practical experience with the diagnosis and management of this condition. Occasional outbreaks in the United States make complacency a threat to public health.
Diagnosis rests on a combination of clinical manifestations that can develop in relation to group A streptococcal pharyngitis. These include chorea, carditis, subcutaneous nodules, erythema marginatum, and migratory polyarthritis. Because the inciting infection is completely treatable, attention has been refocused on prevention. See the image below.
Clinical manifestations and time course of acute rheumatic fever.

Osteoporosis

Osteoporosis, a chronic progressive disease of multifactorial etiology (see Etiology), is the most common metabolic bone disease in the United States. It has been most frequently recognized in elderly white women, although it does occur in both sexes, all races, and all age groups.
This disease is considered a "silent thief" that generally does not become clinically apparent until a fracture occurs (see Clinical Presentation). Screening at-risk populations is, therefore, essential (see Workup).
Osteoporosis can affect almost the entire skeleton. It is a systemic skeletal disease characterized by low bone mass and microarchitectural deterioration of bone tissue, with a consequent increase in bone fragility.^[1] The disease often does not become clinically apparent until a fracture occurs.
Osteoporosis represents an increasingly serious problem in the United States and around the world. Many individuals, male and female, experience pain, disability, and diminished quality of life as a result of having this condition. The economic burden the disease imposes is already considerable and will only grow as the population ages.^[2]
Despite the adverse effects of osteoporosis, it is a condition that is often overlooked and undertreated, in large part because it is so often clinically silent before manifesting in the form of fracture. For example, a Gallup survey performed by the National Osteoporosis Foundation revealed that 75% of all women aged 45-75 years have never discussed osteoporosis with their physicians. Failure to identify at-risk patients, to educate them, and to implement preventive measures may lead to tragic consequences.
Medical care includes calcium, vitamin D, and antiosteoporotic medication such as bisphosphonates and parathyroid hormone. Antiresorptive agents currently available for osteoporosis treatment include bisphosphonates, the selective estrogen-receptor modulator (SERM) raloxifene, calcitonin, and denosumab. One anabolic agent, teriparatide (see Medication), is available as well. Surgical care includes vertebroplasty andkyphoplasty
 . (See Treatment and Management.)
Osteoporosis is a preventable disease that can result in devastating physical, psychosocial, and economic consequences. Prevention and recognition of the secondary causes of osteoporosis are first-line measures to lessen the impact of the disease (see the images below).
Osteoporosis of the spine. Observe the considerable reduction in overall vertebral bone density and note the lateral wedge fracture of L2. Osteoporosis of the spine. Note the lateral wedge fracture in L3 and the central burst fracture in L5. The patient had suffered a recent fall.
For more information, see Pediatric Osteoporosis, as well as Osteoporosis in Solid Organ Transplantation, Utility of Bone Markers in Osteoporosis, and Nonoperative Treatment of Osteoporotic Compression Fractures.

Gout and Pseudogout

Gout and pseudogout are the 2 most common crystal-induced arthropathies. They are debilitating illnesses in which recurrent episodes of pain and joint inflammation are caused by the formation of crystals within the joint space and deposition of crystals in soft tissue.^{[1, 2, 3]} If untreated, these disorders can lead to joint destruction and renal damage. Rarely, gout can produce significant ocular findings.^[4] The incidence of age-related macular degeneration (ARMD) is higher in patients with gout.^[5]
Gout is inflammation caused by monosodium urate monohydrate (MSU) crystals. Pseudogout is inflammation caused by calcium pyrophosphate (CPP) crystals and is sometimes referred to as calcium pyrophosphate disease (CPPD).
Gout-related images are provided below.
Gout. Acute podagra due to gout in an elderly man. Gout. Radiograph of erosions with overhanging edges. Gout. Needles of urate on polarizing microscopy. Although gout is associated with hyperuricemia, the level of uric acid does not itself precipitate gout; rather, acute changes in the level of uric acid cause gout. Most individuals with hyperuricemia do not have gout, but if high uric acid levels go untreated, 90% of patients develop gout within 30 years. Hyperuricemia is found in 90% of individuals with gout, but it is also found in patients taking diuretics and even in those taking low doses of aspirin.
Primary gout is related to underexcretion or overproduction of uric acid. Secondary gout is related to myeloproliferative diseases or their treatment, therapeutic regimens producing hyperuricemia, renal failure, renal tubular disorders, lead poisoning, hyperproliferative skin disorders, enzymatic defects (eg, deficient hypoxanthine-guanine phosphoribosyl transferase, and glycogen storage diseases).^[6]
Gout is definitively diagnosed based on the demonstration of urate crystals in aspirated synovial fluid. Classic radiographic findings may also be diagnostic. (See Workup).
Improvements in early diagnosis and the availability of definitive treatment have significantly improved the prognosis of gout, as evidenced by the declining incidence of disabling chronic tophaceous gout. However, tophaceous gout may still develop because of misdiagnosis, poor management, medication intolerances, and/or poor patient compliance.
Treatment of gout is important to relieve pain, prevent disease progression, and prevent tissue deposition of uric acid (eg, in the kidneys) that may produce kidney stones or urate nephropathy.^[7] (See Treatment.)
Treatment of the acute phase of pseudogout is identical to that of gout. Unlike gout, however, no specific therapeutic regimen exists to treat the underlying cause of pseudogout, but colchicine and hydroxychloroquine are effective for prophylaxis.

Historical background

Gout is one of the oldest diseases in the medical literature.^{[8, 9]} Since the time of the Greeks, many authors have written about gout as the result of personal excess; its association with a diet rich in meat and alcohol gained it the sobriquet, “the king of diseases and the disease of kings”. However, among the abstinent was John Milton, who lived a life of rigorous self-discipline and yet, to his anger and despair, suffered from what commonly was regarded as just punishment of the dissolute.
Lowenhook described symptoms of gout in the 1600s. In 1848, Sir Alfred Garrod linked gout with hyperuricemia, but the pathophysiology of acute gouty arthritis was not described fully until 1962.
Since then, gout has been associated with a large number of different autoimmune and metabolic disorders. Specific therapies and prophylactic measures have been developed to address the underlying problem.
Pseudogout, which may be clinically indistinguishable from gout, was recognized as a distinct disease entity in 1962.

Ankle Block

Regional anesthesia is increasing in its utility in the treatment of injuries, foreign body removal, and even in the operative setting. By providing a regional block, the affected anatomy can be properly cleaned, explored, and treated without causing unnecessary pain to the patient. In many settings, regional anesthetic is also preferred because it prevents the distortion of the anatomy commonly seen in local anesthesia.
Regional anesthesia of the ankle presents a challenge to the provider because of the complicated nerve supply to the foot and the varied locations of the nerves around the ankle. However, by adequately anesthetizing the foot, patients can be offered relief from often painful injuries while the complex anatomy of the foot is examined and repaired. See for more information.
Providing local anesthesia to the foot, particularly to the sole, presents many difficulties. First, the thick but sensitive skin must be penetrated with a needle, which often must be a larger gauge than might be used elsewhere to pass through the skin — this can be very painful. Second, the underlying soft tissue of the sole is tightly bound to the fascia and does not separate easily; injecting even small amounts of anesthesia locally into the sole can result in compression of blood vessels and vascular damage to the tissue that is already injured. But, with regional anesthesia, each of these difficulties can be avoided by blocking the pain sensation carried from the foot at the point where the nerves pass through a safe, readily accessible region of anatomy.^[1]
An image depicting a cross-section of the ankle can be seen below.
Cross-section of the ankle.

Bacterial Keratitis

Bacterial keratitis is a sight-threatening process. A particular feature of bacterial keratitis is its rapid progression; corneal destruction may be complete in 24-48 hours with some of the more virulent bacteria. Corneal ulceration, stromal abscess formation, surrounding corneal edema, and anterior segment inflammation are characteristic of this disease. (See image below.)
Human eye anatomy. Bacterial keratitis remains one of the most important potential complications of contact lens use and refractive corneal surgery. Keeping this in mind, early diagnosis and treatment are key to minimizing any visual-threatening sequelae. In addition, close follow-up, attention to laboratory data, and changing antimicrobials if no clinical improvement is evident are important elements for successful outcome.

Anthrax

Anthrax is a zoonotic infection caused by Bacillus anthracis (see the image below) . Most anthrax is cutaneous (95%). The remaining cases of the disease are inhalational (5%) and gastrointestinal (< 1%). Cutaneous anthrax results from exposure to the spores of B anthracis while handling sick animals or contaminated wool, hair, or animal hides. Pulmonary anthrax results from inhaling anthrax spores. GI anthrax results from ingesting meat products that contain anthrax. Anthrax is present in areas where animals, particularly herbivores, graze. Anthrax caused by inhalation is usually fatal, and symptoms usually begin days after exposure. This delay makes the initial exposure to B anthracis difficult to track.
Polychrome methylene blue stain of Bacillus anthracis. Image courtesy of Anthrax Vaccine Immunization Program Agency, Office of the Army Surgeon General, United States. Anthrax was described in the early literature of the Greeks, Romans, Egyptians, and Hindus. The term anthrakis means coal in Greek, and the disease is named after the black appearance of its cutaneous form.^[1] The fifth plague described in the Old Testament book of Genesis may be among the earliest descriptions of anthrax. At the end of the 19th century, Robert Koch's experiments with anthrax led to the original theory of bacteria and disease. John Bell's work in inhalational anthrax led to wool disinfection processes and the term woolsorter's disease.
A modern concern is use of anthrax as a biologic warfare agent. During the first Gulf War, Iraq reportedly produced 8500 L of anthrax. A total of 150,000 US troops were vaccinated with anthrax toxoid. In the weeks following the terrorist attacks of September 11, 2001, 22 confirmed or suspected cases of anthrax infection were disseminated via the US postal system; the spores mailed in these letters were ultimately traced to a US army medical research institute. Since there have been no cases of naturally occurring inhalational anthrax in the US since 1976, alarm should be raised for the occurrence of even a single infection.

Background

degenerative disc diseaseCervical disc disorders encountered in physiatric practice include herniated nucleus pulposus (HNP),  (DDD), and internal disc disruption (IDD). HNP (seen in the image below) implies extension of disc material beyond the posterior margin of the vertebral body. Most of the herniation is made up of the annulus fibrosus. DDD involves degenerative annular tears, loss of disc height, and nuclear degradation. IDD describes annular fissuring of the disc without external disc deformation.Cervical   radiculopathycan result from nerve root injury in the presence of disc herniation or stenosis, most commonly foraminal stenosis, leading to sensory, motor, or reflex abnormalities in the affected nerve root distribution.^{[1, 2]}
Sagittal magnetic resonance imaging (MRI) scan demonstrating cervical intervertebral disc protrusions at C3-C4 and C7-T1. Understanding cervical disc disease requires basic knowledge of anatomy and biomechanics. The intervertebral disc absorbs shock, accommodates movement, provides support, and separates vertebral bodies to lend height to intervertebral foramina. The disc consists of an eccentrically located nucleus pulposus and a surrounding annulus fibrosus separating each segmental level between the C2-T1 vertebrae. No disc exists between C1 and C2, and only ligaments and joint capsules resist excessive motion. Disc degeneration and/or herniation can injure the spinal cord or nerve roots and result instenosisand/or myofascial pain.

Background

Systemic lupus erythematosus (SLE) is an inflammatory connective tissue disease with variable manifestations (eg, rash; see the images below). SLE may affect many organ systems with immune complexes and a large array of autoantibodies, particularly antinuclear antibodies (ANAs).
The classic malar rash, also known as a butterfly rash, of systemic lupus erythematosus, with distribution over the cheeks and nasal bridge. Note that the fixed erythema, sometimes with mild induration as seen here, characteristically spares the nasolabial folds. Photosensitive systemic lupus erythematosus rashes typically occur on the face or extremities, which are sun-exposed regions. Photo courtesy of Dr. Erik Stratman, Marshfield Clinic.
Physical medicine and rehabilitation for SLE may involve physical therapy, occupational therapy, speech therapy, recreational therapy, or combinations thereof. Follow-up medical care of patients with SLE must be ongoing. One physician should coordinate the patient’s care. The physician and the patient must be alert to subtle changes in symptoms that may indicate a flare-up in the disease process.

Overview

Pneumonia is the sixth leading cause of death, and the number 1 cause of death from infectious disease, in the United StatesThe image below depicts typical bacterial pneumonia.
Image in a 49-year-old woman with pneumococcal pneumonia. The chest radiograph reveals a left lower lobe opacity with pleural effusion.

Typical versus atypical syndromes

The classification of pneumonias as either typical or atypical arose from the observation that the presentation and natural history of some patients with pneumonia were different compared with those with pneumococcal infection.
Pathogens like Haemophilus influenzae, Staphylococcus aureus, and gram-negative enteric bacteria cause clinical syndromes similar to that due to Streptococcus pneumoniae. However, other pathogens cause an atypical pneumonia syndrome, and this was initially attributed to Mycoplasma pneumoniae.
Other pathogens, including bacteria and viruses are now known to cause similar syndromes indistinguishable from that due to M pneumonia. Therefore, the term atypical pneumonia represents diverse etiologic entities and may have limited clinical value.

Preferred examination

Chest radiography with posteroanterior and lateral views is the preferred imaging examination for the evaluation of typical bacterial pneumonia

Gross Anatomy

The anal canal is the most terminal part of the lower GI tract/large intestine, which lies between the anal verge (anal orifice, anus) in the perineum below and the rectum above. Confusion and controversy exist regarding the anatomy of the anorectal region in anatomy and surgical texts. The description in this topic is from below upwards, as that is how this region is usually examined in clinical practice. Images depicting the anal canal can be seen below.^{[1, 2]}
Coronal section of rectum and anal canal. Coronal section through the anal canal.
The pigmented, keratinized perianal skin of the buttocks (around the anal verge) has skin appendages (eg, hair, sweat glands, sebaceous glands); compare this with the anal canal skin above the anal verge, which is also pigmented and keratinized but does not have skin appendages.^{[3, 4]}
In anatomy texts, the rectum changes to the anal canal at the dentate line. For surgeons, however, the demarcation between the rectum above and the anal canal below is the anorectal ring. The following is the description of the surgical anal canal.
The anal canal is completely extraperitoneal. The length of the (surgical) anal canal is about 3-5 cm, with two thirds of this being above the dentate line and one third below the dentate line (anatomical anal canal).
The epithelium of the (anatomical) anal canal (between the anal verge below and the dentate line above) is variously described as anal mucosa or anal skin. The author feels that it should be called anal skin (anoderm), as it looks like (pigmented) skin, is sensitive like skin, and is keratinized (but does not have skin appendages).
The dentate line (also called the pectinate line) is the site of fusion of the proctodeum below and the postallantoic gut above. It is a wavy demarcation formed by the anal valves (transverse folds of mucosa) at the inferior-most ends of the anal columns. Anal glands open above the anal valves into the anal crypts. The dentate line is not seen on inspection in clinical practice, but under anesthesia the anal canal descends down, and the dentate line can be seen on slight retraction of the anal canal skin.
From a surgical perspective, the anal canal just above the dentate line for about 1-2 cm is called the transition zone. Beyond this transition zone, the (surgical) anal canal is lined with columnar epithelium. Anal columns (of Morgagni) are 5-10 longitudinal (vertical) mucosal folds in the upper part of the anal canal.
At the bottom of these columns are anal crypts, or sinuses, into which open the anal glands and anal papillae. Three of these columns (left lateral, right posterior, and right anterior, at 3, 7, and 11 o’clock position in supine position) are prominent; they are called anal cushions and contain branches and tributaries of superior rectal (hemorrhoidal) artery and vein. When prominent, veins in these cushions form the internal hemorrhoids.
The anorectal ring is situated about 5 cm from anus. At the anorectal angle, the rectum turns backwards to continue as the anal canal.
Levator ani and coccygeus muscles form the pelvic diaphragm. Lateral to the anal canal are the ischioanal fossae (1 on either side), below the pelvic diaphragm. The anterior relations of the anal canal are, in males, the seminal vesicles, prostate, and urethra, and, in females, the cervix and vagina. In front of (anterior to) the anal canal is the rectovesical fascia (of Denonvilliers), and behind (posterior) is the presacral endopelvic fascia (of Waldeyer), under which lie a rich presacral plexus of veins. Posterior to the anal canal lie the tip of the coccyx and lower sacrum.

Blood supply and lymphatics

The anal canal above the dentate line is supplied by the terminal branches of the superior rectal (hemorrhoidal) artery, which is the terminal branch of the inferior mesenteric artery. The middle rectal artery (a branch of the internal iliac artery) and the inferior rectal artery (a branch of the internal pudendal artery) supply the lower anal canal.
Underneath the anal canal skin (below the dentate line) lies the external hemorrhoidal plexus of veins, which drains into systemic veins. Underneath the anal canal mucosa (above dentate line) lies the internal hemorrhoidal plexus of veins, which drains into the portal system of veins. The anorectum is, therefore, an important area of portosystemic venous connection (the other being the esophagogastric junction). Lymphatics from the anal canal drain into the superficial inguinal group of lymph nodes.

Embryology

The anal canal below the dentate line develops from the proctodeum, while that above the dentate line develops from the endoderm of the hindgut.

Bile Duct Tumors

Tumors of the biliary tract (see image below) are uncommon but serious problems. The spectrum of lesions ranges from benign tumors, such as adenomas, to malignant lesions, such as adenocarcinomas. This discussion excludes tumors of the gallbladder, which are discussed separately.
Distal common bile duct tumor excised by radical pancreaticoduodenectomy. The tumor measured 1.2 cm in diameter.
Most patients with bile duct tumors present with jaundice due to obstruction of the biliary tree by the tumor. Because the tumors are generally small, standard imaging studies, such as ultrasonography^[1] and computed tomography (CT) scanning, may fail to show the lesion. These techniques may, however, provide a clue to the level of the obstruction and help exclude metastatic disease.
Cholangiography via a transhepatic or endoscopic approach is required to define the biliary anatomy and extent of the lesion. Magnetic resonance cholangiography is a noninvasive alternative available in an increasing number of centers.
The anticipated course of most cases of bile duct tumors includes recurrent biliary obstruction with infectious complications, local spread, and death in 6-12 months. Treatment depends on the site and extent of the lesion, and surgical resection improves survival and prognosis.

Benign Gastric Tumors

With the advent of modern techniques and the widespread use of gastric endoscopy, benign gastric wall lesions are now diagnosed more frequently and can be studied using the tissue obtained by biopsy or polypectomy. In the past, the diagnosis of gastric tumors was based on x-ray examination, but, in 1922, Schendler was the first to make an endoscopic-based diagnosis.^[1] Note the image below.
Upper endoscopy showing multiple gastric polyps.

Acute Mesenteric Ischemia

Acute mesenteric ischemia (AMI) is a syndrome in which inadequate blood flow through the mesenteric circulation causes ischemia and eventual gangrene of the bowel wall. Broadly, AMI may be classified either as arterial or venous disease.
Arterial disease may be subdivided into nonocclusive mesenteric ischemia (NOMI; see the image below) and occlusive mesenteric arterial ischemia (OMAI). OMAI may be further subdivided into acute mesenteric arterial embolus (AMAE) and acute mesenteric arterial thrombosis (AMAT). Venous disease takes the form of mesenteric venous thrombosis (MVT). Thus, for practical purposes, AMI comprises 4 different primary clinical entities: NOMI, AMAE, AMAT, and MVT.
CT scan (with contrast) of nonocclusive mesenteric ischemia with resulting bowel wall edema (arrows). The 4 types of AMI have somewhat different predisposing factors, clinical pictures, and prognoses. A secondary clinical entity of mesenteric ischemia occurs because of mechanical obstruction, such as internal hernia with strangulation, volvulus, intussusception, tumor compression, and aortic dissection. Occasionally, blunt trauma may cause isolated dissection of the superior mesenteric artery (SMA) and lead to intestinal infarction.
Because the 4 types of AMI share many similarities and a final common pathway (ie, bowel infarction and death, if not properly treated), they are discussed together.
In 1930, Cokkinis remarked, “Occlusion of the mesenteric vessels is apt to be regarded as one of those conditions of which the diagnosis is impossible, the prognosis hopeless, and the treatment almost useless.”^[1] This quote indicates some of the extreme difficulties faced by physicians treating AMI. Symptoms are nonspecific initially, before evidence of peritonitis presents. Thus, diagnosis and treatment are often delayed until the disease is advanced.
Fortunately, since 1930, many advances have been made that allow earlier diagnosis and treatment. Whereas the prognosis remains grave for patients in whom the diagnosis is delayed until bowel infarction has already occurred, patients who receive the appropriate treatment in a timely manner are much more likely to recover

Abdominal Angina

Abdominal angina is defined as the postprandial pain that occurs in individuals with sufficient mesenteric vascular occlusive disease such that blood flow cannot increase enough to meet visceral demands. The mechanism is believed to be similar to the  that occurs in individuals with coronary artery disease or the intermittent claudication that accompanies peripheral vascular disease, as depicted in the image below.
The superior mesenteric artery and inferior mesenteric artery share collateral circulation near the splenic flexure of the colon. When dilated, this vessel is termed the meandering mesenteric artery. As seen on an angiogram, this is a sign of chronic mesenteric ischemia. The pancreaticoduodenal arcades are collateral pathways between the celiac artery and the superior mesenteric artery.
Although Schnitzler first described the clinical picture of postprandial clinical pain in 1901, description of the syndrome of postprandial abdominal angina generally is attributed to Baccelli or Goodman (1918). In 1936, Dunphy recognized that this syndrome was a precursor of fatal intestinal necrosis; however, not until 1957 did Mikkelsen propose surgical treatment of occlusive mesenteric vascular disease. Shaw and Maynard reported the first transarterial thromboendarterectomy of the superior mesenteric artery (SMA) in 1958, followed in rapid succession by Mikkelsen and Zarro in 1959. Numerous technical refinements followed

Familial Adenomatous Polyposis

^{Familial adenomatous polyposis (FAP) is the most common . It is an autosomal dominant inherited disorder characterized by the early onset of hundreds to thousands of throughout the colon. If left untreated, all patients with this syndrome develop colon cancer by age 35-40 years. In addition, an increased risk exists for the development of other malignancies. See the image below.
Colectomy specimen obtained from a patient with familial adenomatous polyposis. Note the presence of numerous synchronous adenomatous polyps lining the luminal surface. The genetic defect in FAP is a germline mutation in the adenomatous polyposis coli (APC) gene. Syndromes once thought to be distinct from FAP are now recognized to be, in reality, part of the phenotypic spectrum of FAP.
Syndromes with a germline mutation in the APC gene include FAP, , some families with Turcot syndrome, and attenuated adenomatous polyposis coli (AAPC).  is characterized by colonic polyposis typical of FAP, along with osteomas (bony growth most commonly on the skull and the mandible), dental abnormalities, and soft tissue tumors. Turcot syndrome is characterized by  typical of FAP, along with central nervous system tumors (medulloblastoma). AAPC is characterized by fewer  (average number of polyps, 30-35) as compared to classic FAP. The polyps also tend to develop at a later age (average age, 36 y), and they tend to involve the proximal colonic area.
In considering the spectrum of polyposis syndromes, patients with multiple adenomatous polyps most likely have FAP (or one of its variants), AAPC, or MYH-associated polyposis (MAP). If a patient with a suspected polyposis syndrome undergoes genetic testing and does not have an APC gene mutation, MYH gene testing should be performed to assess for MAP, as 10-20% of patients who do not have an APC gene mutation have biallelic MYH gene mutations]
The phenotype of MAP is often indistinguishable from FAP or AAPC, with patients having usually 10-100 polyps but sometimes more than 100. The age of onset of MAP is usually in patients older than 45 years, and patients often present symptomatically, with commonly found at the time of diagnosis. This is in part because there is usually no family history given the autosomal recessive inheritance pattern of MAP. Duodenal polyps can be found in up to one fifth of patientsThere is no increased risk of other types of cancers associated with this syndrome.}

Schatzki Ring

since the 1950s, several investigators have published reports of patients with dysphagia who had associated lower esophageal ringlike constrictions, but each investigator had a different opinion as to the cause and nature of these rings. In 1953, Ingelfinger and Kramer believed that these rings occurred as a result of a contraction by an overactive band of esophageal muscle ; however, Schatzki and Gary believed that these rings were fixed and not contractile Some of this controversy may be related to the confusion of categorizing muscular and mucosal rings under the same entity, as concluded by Goyal et al
Endoscopic appearance of the distal esophagus illustrating a Schatzki ring.
Two rings have been identified in the distal esophagus. The muscular ring, or A ring, is a thickened symmetric band of muscle that forms the upper border of the esophageal vestibule and is located approximately 2 cm above the gastroesophageal junction. The A ring is rare; furthermore, it is even more rarely associated with dysphagia. On the other hand, the mucosal ring, or B ring, is quite common and is the subject of discussion in this article. The B ring is a diaphragmlike thin mucosal ring usually located at the squamocolumnar junction; it may be symptomatic or asymptomatic, depending on the luminal diameter.
The pathogenesis is not clear, and patients typically present with intermittent nonprogressive dysphagia for solids. Fortunately, most patients respond well to initial and repeat dilatation therapy. A small number of patients may have stubborn rings that require more aggressive endoscopic or surgical intervention.

Sengstaken-Blakemore Tube

Balloon tamponade of bleeding esophageal varices was described as early as the 1930s. A double-balloon tamponade system originally developed by Sengstaken and Blakemore in 1950 has undergone relatively few changes to the current day. The 3 major components of a Sengstaken-Blakemore tube are a gastric balloon, an esophageal balloon, and a gastric suction port. The addition of an esophageal suction port to help prevent aspiration of esophageal contents resulted in what is called the Minnesota tube. Another nasogastric (NG) device with a single gastric balloon is most effective at terminating bleeding from gastric varices and is known as the Linton-Nachlas tube. The advent of endoscopy has reduced the use of balloon tamponade, but the use of such devices can still be temporizing or lifesaving, despite their potential for serious complication^]
See the images below.
Sengstaken-Blakemore tube. Image courtesy of Richard Treger, MD. Linton-Nachlas tube. Image courtesy of Richard Treger, MD

Cytomegalovirus Colitis

Cytomegalovirus (CMV) is a member of the Herpesviridae family, along with,  and . It is a double-stranded DNA virus with a protein coat and lipoprotein envelope. Similar to other herpesviruses, CMV is icosahedral and replicates in the host's nucleus. Replication in the host cell typically manifests pathologically with large intranuclear inclusion bodies and smaller cytoplasmic inclusions, and is accompanied by presence of CMV viral particles in the plasma.

Gross specimen of bowel showing ulceration secondary to cytomegalovirus colitis. Giant cell with inclusion body characteristic of cytomegalovirus colitis.

Between 50% and 80% of the world's population is seropositive for CMV. Initial CMV infection in the immunocompetent host typically is mild and goes undetected clinically. This is followed by a chronic latent state, during which the virus remains present within host cells, but viral proliferation is prevented by host cell-mediated immunity. Failure of immune containment may lead to reactivation with viral proliferation and severe systemic illness. Systemic CMV disease is characterized by fever, pancytopenia, and inflammatory changes in multiple organs including the liver and lungs, and in the retina. Colitis is a frequent manifestation of this acute systemic illness.

Patients are rendered susceptible to systemic CMV disease by treatment with immunosuppressive medications, or by illnesses that reduce cellular immunity, such as . Acute systemic illness caused by CMV is particularly common following initial exposure in an immune compromised individual (in particular, in a CMV-negative transplant recipient who receives an organ from a CMV-positive donor).

Hirschsprung Disease

Hirschsprung disease is a developmental disorder of the enteric nervous system and is characterized by an absence of ganglion cells in the distal colon resulting in a functional obstruction.^[1] See the image below.
Hirschsprung disease. Contrast enema demonstrating transition zone in the rectosigmoid region.
Although this condition was described by Ruysch in 1691 and popularized by Hirschsprung in 1886, the pathophysiology was not clearly determined until the middle of the 20th century, when Whitehouse and Kernohan described the aganglionosis of the distal intestine as the cause of obstruction in their series of patient
In 1949, Swenson described the first consistent definitive procedure for Hirschsprung disease, rectosigmoidectomy with coloanal anastomosis. Since then, other operations have been described, including the Duhamel and Soave techniques. More recently, advances in surgical technique, including minimally invasive procedures, and earlier diagnosis have resulted in decreased morbidity and mortality for patients with Hirschsprung disease.
Most cases of Hirschsprung disease are now diagnosed in the newborn period. Hirschsprung disease should be considered in any newborn who fails to pass meconium within 24-48 hours after birth. Although contrast enema is useful in establishing the diagnosis, full-thickness rectal biopsy remains the criterion standard. Once the diagnosis is confirmed, the basic treatment is to remove the poorly functioning aganglionic bowel and to create an anastomosis to the distal rectum with the healthy innervated bowel (with or without an initial diversion).

Pseudomembranous Colitis Surgery

Pseudomembranous colitis is an inflammatory disease of the colon (see image below). It has changed in the last 100 years from a fatal disease caused by a postoperative event to, in the era of antibiotics, a commonly occurring complication of antibiotic use that may lead to serious morbidity but that usually is treated easily.
Colonic pseudomembranes of pseudomembranous colitis. Photographs courtesy of Eric M. Osgard, MD.

• In the late 1800s, prior to the availability of antibiotics, Finney reported the first case of pseudomembranous colitis, calling it "diphtheritic colitis."
• Hall and O'Toole first described Clostridium difficile in 1935
• C difficile was first implicated as a causative factor in pseudomembranous colitis in the 1970s

Toxic Megacolon

 is the clinical term for an acute toxic colitis with dilatation of the colon. The dilatation can be either total or segmental. A more contemporary term for toxic megacolon is simply toxic colitis, because patients may develop toxicity without megacolon. For the purposes of this article, the term toxic megacolon (toxic colitis) is used, but either toxicity or megacolon can occur exclusively of each other.The hallmarks of toxic megacolon (toxic colitis), a potentially lethal condition, are nonobstructive colonic dilatation larger than 6 cm and signs of systemic toxicity. Toxic megacolon (toxic colitis)was recognized by Marshak and Lester in 1950 Jalan et al described the diagnostic criteria.The first criterion is radiographic evidence of colonic dilatation. The second criterion is any 3 of the following: fever (>101.5°F), tachycardia (>120 beats/min), leukocytosis (>10.5 10³/µL), or . The third criterion is any 1 of the following: dehydration, altered mental status, electrolyte abnormality, or hypotension.
Toxic megacolon (toxic colitis) was first thought to be a complication of . In fact, toxic megacolon (toxic colitis) may complicate any number of colitides, including inflammatory, ischemic, infectious, radiation, and pseudomembranousSee the image below.
A 22-year-old man presented with abdominal pain, passage of blood and mucus per rectum, abdominal distention, fever, and disorientation. Findings from sigmoidoscopy confirmed ulcerative colitis. Abdominal radiographs obtained 2 days apart show mucosal edema and worsening of the distention in the transverse colon. The patient's clinical condition deteriorated over the next 36 hours despite steroid and antibiotic therapy, and the patient had to undergo a total colectomy and ileostomy.
The incidence of toxic megacolon (toxic colitis) is expected to increase due to the rising prevalence of . Colonic dilatation may be present in other conditions, such as , idiopathic megacolon/, and intestinal pseudo-obstruction  However, these patients do not develop signs of systemic toxicity and, therefore, do not fall into the category of having toxic megacolon (toxic colitis). See the images below.
Gross pathology specimen from a case of pseudomembranous colitis demonstrating characteristic yellowish plaques. Computed tomography scan from a patient with pseudomembranous colitis demonstrating the classic accordion sign.