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Diabetology
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10.1 Diabetic Hand and Diabetic Foot – 393
Diabetic Angiopathy – 393
Diabetic Peripheral Neuropathy, Osteopathy, and Infections – 394
Diabetic Myonecrosis – 397
Diabetic Skin Changes and Infections – 397
The Role of Doppler Sonography in DM – 400
The Role of MRI in DM – 401
Diff erential Diagnoses and Related Diseases – 406
10.2 Diabetic Brain and Nervous System – 407
10.3 Diabetic Syndromes – 408
Alström Syndrome – 408
Bardet–Biedl Syndrome – 409
Leprechaunism (Donohue Syndrome) – 409
Prader–Willi Syndrome – 410
Wolcott–Rallison Syndrome – 410
Wolfram Syndrome (DIDMOAD) – 411
Rabson–Mendenhall Syndrome – 411
391
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10.4 Diabetes Insipidus – 412
10.5 Obesity, Gastric Banding, and Liposuction – 415
Hormonal Obesity – 415
Syndromic/Pathologic Obesity – 416
Drug-Induced Obesity – 419
Gastric Banding – 419
Liposuction – 422
10.6 Lipoatrophic–Lipodystrophic Syndromes – 423
Congenital Generalized Lipodystrophy (Seip–Berardinelli Syndrome) – 424
Familial Partial Lipodystrophy (Dunnigan–Kobberling Syndrome) – 425
Mandibuloacral Dysplasia – 425
Acquired Generalized Lipoatrophy (Lawrence–Seip Syndrome) – 425
Acquired Partial Lipoatrophy (Barraquer–Simons Syndrome) – 426
Parry–Romberg Syndrome (Progressive Facial Hemiatrophy) – 426
10.7 Diabetic Nephropathy – 427
Diabetic Nephropathy – 427
Renal Papillary Necrosis – 428
© Springer International Publishing Switzerland 2017
J.A. Al-Tubaikh, Internal Medicine, DOI 10.1007/978-3-319-39747-4_10
Diabetic Cystopathy (Neurogenic Bladder) – 430
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Calcifi cation of the Vas Deferens – 430
Emphysematous Cystitis – 430
Emphysematous Pyelonephritis – 431
Xanthogranulomatous Pyelonephritis – 432
10.8 Lipomatosis – 432
Intestinal Lipomatosis – 432
Pelvic Lipomatosis – 434
Epidural Lipomatosis – 434
Encephalocraniocutaneous Lipomatosis Syndrome
(Haberland Syndrome) – 434
Lipomatous Hypertrophy of the Interatrial Septum – 435
10.9 Hypoglycemia – 436
Diff erential Diagnoses and Related Diseases – 437
10.1 · Diabetic Hand and Diabetic Foot
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10.1 Diabetic Hand and Diabetic Foot
Diabetes mellitus (DM) is a chronic metabolic disease that
arises due to insulin de ciency (type 1 DM) or insulin receptor insensitivity (type 2 DM). Type 2 DM is more common
than type 1.
Diabetic complications arise due to cellular ischemia,
angiopathy, peripheral neuropathy, osteopathy, infections,
skin changes, and atherosclerosis. e hands and feet are
uncommonly a ected in diabetes, but when they are
a ected, it may be severe enough to cost the patient loss of a
limb.
Radiology o ers great tools for early detection of diabetic complications by ultrasound and MRI.For diabetic
foot screening, a Doppler scan is performed to detect arterial ow anomalies. If the Doppler scan shows abnormalities
in the vessels, MRI can be done to detect hidden signs of
diabetic foot complications. Adults with long-term DM
should be annually examined for lower limb vascular abnormalities.
Diabetic Angiopathy
Diabetic angiopathy is divided into two types: microangiopathy and macroangiopathy. Microangiopathy arises due to
chronic hyperglycemia that impairs the walls of the microvessels, causing leakage of exudates and blood. Later, these exudates may lead to obstruction of the microvessels causing
ischemia. is type is typically seen in diabetic retinopathy
and diabetic nephropathy. Macroangiopathy, on the other
hand, damages the arterial vessels due to atherosclerosis
a ecting the coronary, cerebral, and lower limb vessels.
Arteriosclerosis occurs 10 years earlier in diabetics than in
normal people.
Chronic limb ischemia and compromised vascular supply can lead to tissue necrosis and dry gangrene. is is
o en complicated by bacterial infection that may cause wet
gangrene; this scenario is o en seen in the feet. Amputation
is the tragic end of severe limb osteomyelitis, extensive
lower limb calci cations, and uncontrolled diabetes that
suppresses the immune system. Within 2 years of amputation of one leg, the other leg has a 50 % chance of complications that might lead to a 50 % chance of contralateral
amputation.
Gangrene can be divided into dry, wet, and infected.
Dry gangrene arises due to an occluded artery with a patent
vein; tissue liquefaction occurs at a very slow rate. It is seen
in senile gangrene (due to atherosclerosis and vascular stasis) and Buerger’s disease (thromboangiitis obliterans).
Senile gangrene is seen in 50 % of elderly patients wearing
tight shoes and commonly a ects the big toe. Wet gangrene
arises due to an occluded artery and vein, with rapid tissue
liquefaction and sever toxemia. is type is classically seen
in DM, crush injuries (accidents), and bedsores. Infected
gangrene arises due to bacterial infection and is typically
seen in lung abscess, necrotizing fasciitis, synergistic gangrene, and gas gangrene (due to muscular lesion with
anaerobic fermentation of the tissues with Clostridium dif-
cile ).
As previously mentioned, patients with gangrene are
treated by amputation of the gangrenous part of the lower
extremity, which can be above or below the knee, depending
on the extension of the compromised vascular supply. e
amputee may develop stump pain a er surgery, which can be
attributed to stump infection, in ammation, impaired vasculature, or development of neuromas. A neuroma is a focal,
nodular, noncapsulated so -tissue mass that forms at the distal segment of peripheral nerves a er surgery or traumatic
avulsion injury. Schwann cells regenerate the peripheral nervous system axons and myelin sheath a er trauma. In an
amputated limb, regeneration of the nerve axon is unstoppable, because there is no distal end pathway for the regenerated nerve axon to fuse with, resulting in aggregation of the
Schwann cells at the stump end, forming a mass of nerve tissue. Postamputation neuromas are usually multiple and may
appear 1 month a er amputation. Patients typically present
with stump pain, usually in the absence of in ammation or
stump infection.
Signs on Radiographs
Calcifi cation of pedal vessels occurs in 24 % of diabetic
patients, and it is seen radiologically as classic “tramline”
or “pipestem” calcifi cation (
. Fig. 10.1.1 ).
. Fig. 10.1.1 A lateral plain radiograph of a patient with
severe diabetic foot shows calcaneal ulcer ( hollow arrowhead ),
osteomyelitis causing bone resorption and necrosis ( solid
arrowhead ), and calcifi ed arteries due to macroangiopathy
( arrows )
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Chapter 10 · Diabetology
Signs on MRI
Postamputation neuromas are detected as ovoid,
bulbous, or rounded soft-tissue mass expansions at the
end of a proximally transected nerve (e.g., peroneal
nerve in above-knee amputation) that classically
measure 1–2 cm in diameter. The mass shows low T1 and
moderately high T2 signal intensity, with a characteristic
dark rim seen on both T1W and T2W images due to focal
fi brous tissue condensation around the neuroma.
Diabetic Peripheral Neuropathy,
Osteopathy, and Infections
Diabetic peripheral neuropathy o en a ects both hands and
feet in a bilateral symmetrical fashion ( glove and stocking
phenomenon ). Loss of the deep knee tendon re ex is the earliest sign of diabetic neuropathy, even before any sensory or
motor disturbances manifest. Diabetic neuropathy is attributed to metabolic abnormalities a ecting Schwann cells, the
myelin-forming cells of the peripheral nervous system.
In the neuropathic diabetic foot, sympathetic denervation
is the main pathological injury. Somatic and autonomic denervation causes numbness and loss of heat and pain sensation,
along with reduction in the sensation of touch and vibration.
Sympathetic denervation causes arteriovenous shunts within
hands and feet, causing abnormal increase in the venous ow
within the limbs. Moreover, the intracutaneous pressure causes
the development of calci cation within the medial layer of the
arterial vascular wall ( Monckeberg ’ s sclerosis ).
ere are two types of neuroarthropathies in DM: atrophic
and hypertrophic ( Charcot ’ s joint ). Atrophic neuroarthropathy
is characterized by osteoporosis, bone resorption, and dislocation. In contrast, Charcot’s joint is characterized by the 5Ds: d is-
tention, d islocation, d isorganization, d ebris, and increased bone
d ensity. In the absence of diabetes, atrophic neuroarthropathy is
commonly caused by syrinx in the cervical spine, while Charcot’s
joint is commonly caused by neurosyphilis of the posterior columns of the spinal cord ( tabes dorsalis ). A syrinx is also the com-
monest cause of Charcot’s joint of the shoulder.
Diabetic peripheral neuropathy a ects 10–15 % of
patients, and it can be di use or focal. e di use form
presents in the form of bilateral, symmetrical denervation
and sensory de cits of the hands and feet (glove and stocking phenomenon). In contrast, the focal form presents in
the form of “mononeuritis,” commonly a ecting the cranial
nerves CN III, CN IV, CN VI, and CN VII.Involvement of
both sympathetic and sensory bers leads to mechanical
overuse, loss of the protective joint pain, proprioceptive
sensation, and active hyperemia due to loss of vasoconstrictive neural impulses, which all result in atrophic neuroarthropathy. In contrast, sensory ber denervation in the
absence of sympathetic ber involvement results in the
development of Charcot’s joint. e atrophic joint tends to
involve the forefoot, while Charcot’s joint tends to a ect the
mid- or hindfoot.
Diabetic lumbosacral radiculoplexus neuropathy ( DLRPN ),
also known as Bruns – Garland syndrome , is an uncommon
condition, characterized by asymmetric lower extremity
pain, weakness, and muscle atrophy commonly a ecting the
thigh muscles. e mechanism of injury is thought to be a
result of microvasculitis and resultant ischemic injury to the
sacral plexus and/or peripheral nerves. Patients with DLRPN
are commonly between 46 and 71 years of age, o en
presenting with acute or subacute onset of severe asymmetric
lower limb pain and paresthesia involving the anterolateral
thigh region. e pain is described as aching and burning
and tends to be worse at night or in contact with cloths or bed
sheets (contact allodynia). DLRPN pain is usually followed
by limb weakness, evolving over weeks or months, and
commonly a ects the quadriceps and iliopsoas muscles.
Wasting of the quadriceps muscle and absence or reduction
in the knee jerk re ex are classic features. DLRPN is
commonly preceded by unintentional weight loss. Laboratory
ndings in DLRPN include high erythrocytes sedimentation
rate, occasional positive rheumatoid factor (RF) and
antinuclear antibody (ANA), and elevated cerebrospinal
uid protein content.
Osteomyelitis occurs in up to 90 % of cases in the diabetic
foot, due to neurotropic pedal ulcers. Diabetic ulcers tend to
occur at the sites of pressure over bony or joint protuberance
(e.g., metatarsal heads or the calcaneus).
e diabetic foot can be rarely associated with tarsal
tunnel syndrome. Tarsal tunnel syndrome is a condition
characterized by entrapment of the posterior tibial nerve as it
passes beneath the exor retinaculum. e condition is
analogous to carpal tunnel syndrome in the wrists. Patients
o en present with a burning sensation and paresthesia in the
toes, sole of the foot, or medial heel, aggravated by weight
bearing.
Uncommonly, Freiberg’s disease may arise in patients
with diabetic foot. Freiberg ’ s disease is a disease characterized
by infarction of the metatarsal heads. e disease typically
develops 3–4 times more frequently in women than men,
during late childhood or adolescence. Patients present clinically in the acute phase with local foot pain with tenderness,
con ned to the area of the metatarsal heads. In the chronic
phase, which is characterized by osteonecrosis and repair,
patients are typically asymptomatic.
Signs on Plain Radiographs
5 Charcot’s joint is destruction of the aff ected with
sclerosis (increased bone density), osteophytes
(debris), dislocation, and destruction (
5 Calcified vessels may be seen as radio-opaque
tubular structures.
5 An atrophic joint often shows osteoporosis with
resorption of the metatarsal distal ends resulting
in “pencil and cup” or “sucked candy stick”
deformities, similar to those seen in leprosy.
. Fig. 10.1.2 ).
10.1 · Diabetic Hand and Diabetic Foot
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5 Osteomyelitis is seen as cortical bone destruction
with a moth-eaten appearance of the affected
bone (
. Figs. 10.1.1 and 10.1.3 ).
5 Lisfranc fracture is a clinical condition where the
entire forefoot is displaced laterally. Lisfranc
fracture is diagnosed radiographically when the
second metatarsal bone is displaced laterally
>2 mm from its articulation with the intermediate
cuneiform bone (
5 Charcot’s joint of the hip can result in osteolysis of
the acetabulum with loss of its boarders
( wandering acetabulum ) and hypertrophic sclerosis
of the femoral head resulting in a “drumstick”
appearance.
5 The talonavicular joint is a preferred site for
Charcot’s joint in the hindfoot.
. Fig. 10.1.4 ).
a
b
. Fig. 10.1.2 Anteroposterior plain knee radiograph of a
patient with sever Charcot’s knee joint demonstrates
disorganization, debris, and increased bone density
. Fig. 10.1.3 Plain foot radiographs of a patient with diabetic
foot show acute osteomyelitis. In ( a ), the patient was
investigated for a pain in the fi fth toe, which shows mild
osteoporosis compared to the rest of the metatarsals (note the
third toe amputation). After 3 months ( b ), the patient showed
moth-eaten osteomyelitis bone destruction of the fi fth
metatarsal bone, with complete cortical destruction
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Chapter 10 · Diabetology
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Signs on MRI
5 In DLRPN, the scan show enhancement of the
lumbosacral nerve roots and plexus after contrast
injection.
5 I n Freiberg ’ s infarction , the metatarsal head shows
low T1 signal intensity and high T2 signal intensity,
with contrast enhancement (
. Fig. 10.1.5 ) .
. Fig. 10.1.4 Plain radiograph of the forefoot show Lisfranc
fracture, with lateral displacement of the metatarsals
( arrowhead )
a
b
c
. Fig. 10.1.5 Plain foot radiograph ( a ), T1W ( b ), and sagittal short tau inversion recovery (STIR) ( c ) foot MRI of a patient show the signs of
Freiberg’s infarction. In ( a ), there is mild fl attening and sclerosis of the second metatarsal head ( arrowhead ). Later, the patient underwent a
foot MRI that confi rmed bone infarction of the second metatarsal head seen as low T1 signal intensity in ( b ) and high signal intensity in ( c ).
The MRI shows also fracture of the third metatarsal neck ( arrows ), which was not well appreciated in the plain radiograph ( a )
10.1 · Diabetic Hand and Diabetic Foot
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Diabetic Myonecrosis
Diabetic myonecrosis is a rare complication of diabetes,
characterized by muscle infarction. Most patient a ected
with diabetic myonecrosis are patients with type 1 DM (74 %)
and type 2 DM (26 %) or patients with prolonged poorly controlled diabetes. Diabetic myonecrosis occurs in association
with diabetic retinopathy (60 %), nephropathy (80 %), or
neuropathy (64 %). It almost always occurs in the lower
extremities and o en a ects the quadriceps muscles.
Patients with diabetic myonecrosis commonly present
with a painful limb, swelling, and resting pain that is aggravated by walking. If one limb is a ected by diabetic myonecrosis, the contralateral limb may be involved up to 2 years
a er the initial manifestation.
e main di erential diagnosis of diabetic myonecrosis
includes deep venous thrombosis (DVT) and pyomyositis.
DVT can be ruled out by Doppler sonography. Pyomyositis is
a severe muscle infection with formation of an intramuscular
abscess. In 90 % of cases, it is caused by Staphylococcus . In
contrast, diabetic myonecrosis does not show positive culture
of Staphylococcus , because it is mostly caused by ischemia
and infarction rather than infection.
Signs on MRI
The aff ected muscle shows extensive edema and
swelling, with high signal intensity on T2W images
involving the muscle and the subcutaneous tissues.
Diabetic Skin Changes and Infections
Sudeck’s atrophy ( shoulder – hand disease ). Sudeck’s atrophy is
a disease characterized by osteoporosis and swelling in one
limb, especially the ankles, wrists, and elbows, a er a minor
trauma. It results from abnormal sympathetic innervations
and secondary vascular changes a er minor trauma and typically a ects the distal part of a limb below the trauma.
Tropical diabetic hand syndrome , a terminology used to
describe a speci c infection of the hands in diabetics, usually
occurs in tropical areas and is characterized by progressive
synergistic gangrene ( Meleney ’ s gangrene ) of the hand fol-
lowing minor trauma. e cause of this syndrome is a progressively severe form of cellulitis caused by multibacterial
infection, usually a er a history of minor trauma or a scratch
(
. Fig. 10.1.6 ).
Necrobiosis lipoidica diabeticorum ( NLD ) is a rare,
degenerative, granulomatous skin disease that o en a ects
the lower extremities in diabetic patients (0.3 % of diabetics).
Lesions are red papules or oval plaques that grow peripherally and become atrophic and yellowish at the center, with
elevated and erythematous edges (. Fig. 10.1.7 ). With time,
these lesions become more brownish-yellow, telangiectatic,
and porcelain-like. In most cases they are bilateral. Ulceration,
the most common complication of NLD (35 %), usually
arises a er a minor trauma. Lesions in NLD are granulomatous, mainly a ecting the subcutaneous tissues and the
dermis, and the epidermis is o en normal or atrophic. NLD
is classically found in young Caucasian diabetic patients,
with female predominance (80 %); however, it may occur also
with sarcoidosis, rheumatoid arthritis, and in ammatory
bowel disease.
When normal skin is stroked with a dull object, it rises and
swells to assume the shape of the stroke, due to edema and
Diabetic hand lesions are not as common as diabetic foot
lesions, perhaps due to the stress load on the feet compared
to the hands. e main lesions of the hands in diabetes are
related to dermatological diseases rather than neuroosteopathic diseases such as those of the feet.
Diabetic dermopathy is characterized by the formation of
multiple skin thickening on the back of the ngers ( nger
pebbles), scleroderma-like skin and sti joints of the ngers
and dorsum of the hand, and brown atrophic macules over
the shin. Acanthosis nigricans is hyperpigmentation and
velvety brown thickening of the major skin exures, which is
o en seen with type 2 DM and obese patients.
Diabetic hand syndrome refers to a condition of
neuropathy denervation of the hand. It is characterized by
intrinsic wasting of the hand muscles and atrophy of the palmar tissues, with exion contractures of the ngers that may
mimic Dupuytren’s contracture. Patients with diabetic hand
syndrome o en complain of carpal tunnel syndrome , with
paresthesia in the palmar distribution of the median nerve
(the rst three ngers) and positive Tinel ’ s sign (pain and par-
esthesia initiated in the palmar sensory distribution of the
median nerve by tapping over the palmar aspect of the wrist).
Moreover, sever neuropathic denervation may lead to
. Fig. 10.1.6 An illustration demonstrates severe synergistic gangrene
of tropical diabetic hand syndrome
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Chapter 10 · Diabetology
. Fig. 10.1.7 An illustration demonstrates necrobiosis lipoidica
diabeticorum lesions on both shins
local erythema. In rare situations, exaggeration of this response
may be seen in diabetic patients, a condition known as derma-
tographism ( mechanical urticaria ). Skin stroke erythema in
normal skin develops and subsides in less than 5–10min,
whereas in dermatographism, it can last up to 30min.
Fournier ’ s gangrene , also known as necrotizing fasciitis of
the scrotum, is a medical emergency that is characterized by
rapidly progressing gangrene of the penis and scrotum, usually
in diabetic males aged 50–70 years. Fournier’s gangrene is
commonly seen a er perineal trauma, urinary tract infection,
or urological surgical procedures. Fournier’s gangrene is initiated by perianal, perirectal, and ischiorectal abscesses, ssures,
or urinary extravasation. Systemic ndings include leukocytosis, fever, hypoglycemia, tachycardia, and dehydration.
e skin of the back of the neck is surrounded by tough
deep fascia that attaches to the epidermis layer by brous
bands, creating separated compartments. In diabetics, subcutaneous infection on the back of the neck is localized by these
brous bands laterally and inferiorly, forcing the abscess to
spread to the surface via a sinus. Multiple intercommunicating
abscesses that open into the surface via multiple sinuses in diabetic patient is a special type of abscess called “ carbuncle .”
Signs on Plain Radiographs
Changes in the hand due to Sudeck’s atrophy are
typically seen as severe osteoporosis, which occurs at the
ends of all the phalanges and up to 70 % of metatarsal
heads (
. Fig. 10.1.8 ). Pseudoperiostitis may be seen as
striation of the cortices due to new bone formation.
Severe subluxation of the phalangeal joints may occur
later in the course of the disease.
. Fig. 10.1.8 Plain hand radiograph of a patient with
Sudeck’s atrophy shows marked osteoporosis of the hand that is
localized to the phalanges and the metatarsal heads
( arrowheads )
Signs on US
5 Carpal tunnel syndrome can be diagnosed with
wrist ultrasound by identifying the nerve below
the flexor retinaculum. Diagnosis of nerve
entrapment is achieved when the nerve transverse
diameter exceeds 10 mm due to edema or when
the nerve fails to return to its normal position
when performing the flexion pinch maneuver
(
. Fig. 10.1.9 ).
5 Fournier’s gangrene is characterized by thickening
of the scrotal skin, with gas formation within the
subcutaneous skin, seen as hyperechoic foci
surrounded by dirty shadowing.
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. Fig. 10.1.9 Median nerve ultrasound in a healthy volunteer
shows the normal median nerve ( arrowhead ) seen below the
fl exor retinaculum ( arrow ) as a hypoechoic structure in ( a ) and
( b ). The median nerve transverse diameter was 4 mm. In the
fl exion pinch maneuver, the patient is asked to fl ex his wrist,
forcefully oppose the thumb to the index fi nger, hold the
position for 3–5 s, and then release. In this maneuver, the
median nerve moves in a sagittal motion deep into the carpal
tunnel ( arrowhead in b ) and then returns to its normal position.
Failure of the nerve to return to its normal position or to move
deep into the carpal tunnel with this maneuver is a sign of
entrapment
a
b
Signs on CT
Fournier’s gangrene is seen as thickened scrotal and/or
penile skin with hypodense soft-tissue fl uid collection
surrounded by rim contrast enhancement (abscess). Air
within the mass and the subcutaneous tissues is a typical
sign of necrotizing fasciitis (
. Fig. 10.1.10 ).
. Fig. 10.1.10 Axial postcontrast CT of the scrotum and the upper thighs shows scrotal abscess with areas of ring contrast enhancement
( arrows ) and gas formation ( arrowhead ); a radiological stigma of Fournier’s gangrene
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Chapter 10 · Diabetology
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Signs on MRI
In patients with carpal tunnel syndrome, there is a
typical fl attening of the median nerve, with high signal
intensity in T2W images with contrast enhancement, due
to infl ammation.
The Role of Doppler Sonography in DM
Doppler sonography is used to detect stenosis within the
arterial system of the lower extremities. Arteriosclerosis is
the most common cause of arterial stenosis with the formation of atheromas and calcium plaques within the arterial
walls. Analysis of the Doppler wave spectrum is essential to
detect the hemodynamic abnormalities of circulation in the
lower limbs. Di erent spectral waves are observed, according
to the degree of stenosis.
Signs of Peripheral Vascular Disease on Doppler Scan
(Can Be Detected Even Before the Appearance of
Clinical Symptoms)
5 Medial arterial wall calcification with acoustic
shadowing string of beads sign (
5 Increased diastolic flow with reduced resistance
index (RI) in the spectral flow analysis
(
. Fig. 10.1.12 ). The increase in diastolic flow is
due to arteriovenous shunting.
5 Spectral flow abnormalities.
. Fig. 10.1.11 ).
. Fig. 10.1.11 Sagittal ultrasound image of the superfi cial
femoral artery in a diabetic shows multiple dense calcifi cations
of the arterial wall ( arrowheads )
. Fig. 10.1.12 Sagittal ultrasound image of the superfi cial femoral artery in a patient with peripheral vascular disease due to diabetes
mellitus (DM) shows monophasic arterial spectral wave with increased diastolic fl ow ( arrowhead )
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