Добавил:
kiopkiopkiop18@yandex.ru t.me/Prokururor I Вовсе не секретарь, но почту проверяю Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз: Предмет: Файл:

Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2690_Библиотеки_им_академика_М_И_Перельмана

.pdf
Скачиваний:
0
Добавлен:
31.08.2026
Размер:
38 Мб
Скачать
10.5 Obesity, Gastric Banding, and Liposuction 375
https://t.me/medicina_free
Madelung’s disease (Benign symmetric lipomatosis/ Launois-Bensaude syndrome) is a rare condition, characterized by massive symmetric deposits of non­encapsulated adipose tissue in the head, the neck, and the upper trunk.
Madelung’s disease (MD) is typically seen in middle­aged males of Mediterranean origin with a history of excessive alcohol consumption (90% of cases). Patients with MD typically consume more than 80 g of alcohol per day for more than 10 years. MD is considered a “sight diagnosis” because of the typical patterns of fat distribution in the head and neck region. Lipomatosis is typically seen accumulating in both parotid regions (hamster cheeks appearance), cervical region (horse collar appearance), and the posterior neck region (buf­falo hump appearance) (
Fig. 10.5.5 ). The disease is
divided into two types: type 1 MD is characterized by symmetric body lipomatosis that gives the patient a “pseudo-athletic” appearance (Fig. 10.5.5 ). Type 2 MD is characterized by diffuse lipomatosis that gives the patient a generalized obese appearance.
Sensory, motor, or autonomic polyneuropathy is
seen in up to 85% of patients with MD. Rarely, the
tongue or the mediastinum is involved in lipomatosis, resulting in dysphagia and dyspnea.
Metabolic syndrome (Syndrome X) is defi ned by the
WHO as the presence of impaired glucose regulation or diabetes, with two of the following risk factors: hypertension, dyslipidemia, central obesity, and microalbuminuria. There is a strong association between the development of metabolic syndrome and increase in visceral adipose tissue (VAT). Fröhlich syndrome is a rare hypothalamic disorder, characterized by obesity, stunned growth, and geni­tal hypoplasia. Obesity-hypoventilation (Pickwickian) syndrome ) is a disease characterized by the triad of morbid obe­sity, hypoventilation, and irresistible hypersomno­lence (drowsiness). Hypoventilation in Pickwickian syndrome is defi ned clinically by an arterial blood gas as a PaCO 2 > 45 and/or a PO 2 < 55 in the pres­ence of morbid obesity. This hypoventilation is attributed to obstructive sleep apnea in these patients, and by the ventilation/perfusion mismatch that results from the irregular shallow breathing with or without compression by a thick chest wall.
Fig. 10.5.5. An illustration demonstrates the typical clinical signs of Madelung’s disease: ( a ) hamster cheeks appearance, ( b ) horse collar appearance, ( c ) buffalo hump appearance, and ( d ) pseudo-athletic appearance
376 Chapter 10 Diabetology
https://t.me/medicina_free
10.5
Drug-Induced Obesity
Glucocorticoids use : glucocorticoids cause fat accu- mulation in the body in a similar fashion to people with Cushing’s syndrome. Glucocorticoid obesity is seen in patients with chronic prednisolon intake >10 mg/day or its equivalent.
Cessation of smoking : Patients who quit smoking
can gain up to 4–5 kg on average, and it is partly mediated by nicotine withdrawal.
Evaluation of Fat within the Body
There are several techniques used to estimate body fat. Anthropometry is a technique used to estimate BMI by measuring waist circumference and skinfold thick­ness. BMI is a good indicator of obesity, but it provides indirect measurement of body fat, and is unable to differentiate lean body mass from body fat. The inabil­ity of BMI to discriminate lean body mass from fat body mass leads to defi ning lean, muscular individuals as obese.
The metabolism of adipocytes varies with fat sites. Fat deposits in the abdomen and fl ank are more meta­bolically active than fat deposits in the buttocks or thighs. Upper-body obesity is more associated with hypertension, glucose intolerance, and serum choles­terol levels than is lower-body obesity. Recent research emphasizes the high risks of cardiovascular diseases and metabolic abnormalities associated with increased intra-abdominal VAT. VAT is defi ned as the fatty tis­sue that accumulates beneath the abdominal muscle wall and surrounding the abdominal viscera. VAT mass is pathologically more important than subcuta­neous fat thickness over the trunk. Also, visceral fat is a major determinant of whole body insulin resistance.
Adipose tissue is a specialized loose connective tis­sue that is laden with adipocytes. It works as a source of energy, thermal insulation (e.g., brown fat), and as a mechanical cushion in mammals. A 70-kg man nor­mally has 15 kg of adipose tissue, representing 21% of body mass. In contrast, fat is a term used to describe the chemical lipid component in the form of triglycer­ides. It can be found within adipocytes, or in
pathological conditions like fatty liver. Modern meth­ods use ultrasound, CT, and MRI as useful and accu­rate tools for VAT quantifi cation.
Quantitative Assessment of Visceral Fat by US
Ultrasound can be used as a fast and easy method to quantify visceral and subcutaneous fat. Although it is not as standard as CT quantifi cation, it is reported by many investigators to be an accurate method to quan­tify the abdominal wall fat thickness and preperitoneal fat thickness.
Quantitative Assessment of Visceral Fat by CT and MRI
An axial body section is taken at the level of the navel or (L4/L5), and assessment is done via a computer software program to calculate the percentage of vis­ceral fat distribution (Figs. 10.5.6 and 10.5.7 ). Excess visceral fat typically separates the intra-abdominal structures. The drawbacks of the CT method are the use of radiation and the limited gantry diameter to patients less than 70 cm wide (the standard CT gantry diameter). MRI, on the other hand, is radiation-free, but more expensive in its use as a regular monitoring method. The MRI method uses the same procedures as the CT method of quantifi cation.
Gastric Banding
Gastric banding is a widely performed surgical proce­dure as a surgical therapy for morbid obesity. The pro­cedure consists of placing a silicon band around the upper part of the stomach to create a small gastric pouch that works as a stomach (neostomach). The pouch is connected to the rest of the stomach via a nar­row stoma. The silicon band contains an adjustable inner balloon that can be infl ated with air or fl uid up to
3
5 cm
, and it is connected to a reservoir that is typically sutures to the anterior rectus sheath. The stoma width normally should be within 3–4 mm.
10.5 Obesity, Gastric Banding, and Liposuction 377
https://t.me/medicina_free
complications may arise later in up to 35% of cases. Additional surgery may be required in up to 11% of cases.
Early complications of gastric banding include esophageal perforation (0.5% of cases), dysphagia (14% of cases), gastroesophageal refl ux disease, and early slippage of the band (1% of cases). Late complica­tions include eccentric pouch dilatation (25%), slippage of the band (24%), intra-gastric band migration, and gastric necrosis (<0.3%). Patients with diabetes show early gastric dilatation due to diabetic gastroparesis.
Fluoroscopic barium meal examination is per­formed to detect abnormalities and complications of gastric banding. Initially, a scout supine abdominal
Fig. 10.5.6. An axial CT section obtained at the level of L4/L5 vertebra
image is taken to identify the place of the band and the reservoir. Water-soluble contrast media is initially administered in the early stages to confi rm the absence of leakage. The normal images should reveal
The gastric banding procedure controls obesity by restricting food administration to the stomach into a small gastric pouch and narrow stoma, which creates early satiety when the pouch is full. Although gastric banding is a relatively safe procedure, several
a small upper gastric pouch, a narrow stoma extend­ing through the gastric band, and opacifi cation of the rest of the stomach (
Fig. 10.5.8 ). CT can be per-
formed after the fl uoroscopic examination to detect other abnormalities.
Fig. 10.5.7. Computer­generated histogram segmentation of the same CT section as (Fig. 10.5.6 ) done by using image analysis software (Image J ). The image in ( a ) refl ects fat density (repre­sented in white ) and the other nonfatty tissues are removed (represented in black ). Image ( b ) represents total body volume, image ( c ) represents subcutaneous tissue volume (in back after inversion of the original white color ), and image ( d ) represents VAT volume (in black ). The volume of VAT or subcutaneous tissue in this image is gained in cm The volume must be multiplied by the slice thickness of the original image to get the fat volume in cm
2
3
.
378 Chapter 10 Diabetology
https://t.me/medicina_free
10.5
Fig. 10.5.8. Barium meal illustration shows the normal radio­graphic fi ndings in gastric banding: the gastric pouch ( solid
arrowhead ), the stoma ( solid arrow ), the gastric band ( hollow arrowhead ), and the gastric fundus ( hollow arrow )
Fig. 10.5.9. Barium meal illustration shows eccentric pouch dilatation
Signs on Barium Meal
Eccentric pouch dilatation is seen as an abnormally dilated gastric pouch. It is usually seen in patients with dietary noncompliance (Fig. 10.5.9 ). The gastric pouch is dilated due to chronic overfi lling. The stoma is usually in its normal position. Pouch dilatation may also occur due to narrow stoma (Fig. 10.5.10 ). The scan shows pouch dilatation, with narrow stoma (2–3 mm). Patients with this kind of obstruction often present with esophageal dysmotility, vomiting, and pseudo­achalasia. Band slippage is seen as stomach herniation above the band, resulting in pouch dilatation (Fig. 10.5.11 ). Patients may be asymptomatic (20%), or may present with epigastric pain, vomiting, and progressive gastroesophageal refl ux disease. If not corrected, band slippage can lead to gastric volvulus and gastric necrosis. Intragastric migration of the band is a serious complication, in which the band gradually erodes into the gastric wall until it perforates the stomach. It is a rare complication, seen in 0.2–2% of cases. Patients present with nonspecifi c gastric pain, gastrointestinal bleeding, and perotinitis when perforation occurs. The herniated band edge is seen as a barium fi lling defec t within the stomach on barium examination (pathognomonic).
Fig. 10.5.10. Barium meal illustration shows pouch dilatation due to narrow stoma ( arrowhead )
10.5 Obesity, Gastric Banding, and Liposuction 379
https://t.me/medicina_free
equals the amount of fat to be removed (Illouz tech­nique). The dissecting fl uid is composed of isotonic saline, hyaluronidase, and perhaps lidocain, epineph­rine, and sodium bicarbonate.
Liposuction is a relatively safe procedure, with over­all complications occurring in approximately 5–10% of patients. Most of these complications include the for­mation of hematoma, seroma, edema, and skin pigmen­tation problems. Removal of more 3,000 mL of fat may carry the risk of severe systemic complications that require resuscitation and blood transfusion. The amount of fat removed should not exceed 6–8% of the patient’s body weight and 30% of patient’s body surface area. Also, blood loss should not exceed 1–1.5 units.
Causes of severe complications and death in lipo­suction include the development of crush syndrome and fat embolism syndrome (FES). Crush syndrome is a pathological condition characterized by severe shock and hypotension, anuria, coma, and death. Classically, the disease is caused by muscle disintegration that
Fig. 10.5.11. Barium meal illustration shows band slippage into the dilated gastric pouch ( arrowhead )
releases myoglobin into the circulation, which has toxic effect over the renal glomerule. During liposuction, the numerous tunnels created through the fat create a mechanism of subcutaneous injury similar to crush syndrome. Also, uncontrolled cannula movements may
Liposuction
cause muscle injury, creating crisscrossed tunnels that may produce large cavities. These large cavities result
Liposuction is a procedure that allows surgical removal of excess adipose fat in healthy individuals. It is a very common and popular aesthetic surgical procedure that is performed in many countries around the world.
The main complications in fat removal procedures lies in the degree of hemostasis achieved after detach­ment of the subcutaneous fat layer from the skin in the area of desired fat removal. Poor hemostasis results in the formation of hematoma, seroma, infection, necro­sis, and even shock and death, which was commonly seen in the old fat removal techniques that used cutting instruments. In modern liposuction techniques, the detachment of subcutaneous fat is accomplished by injecting fl uid under the skin in the desired region through a blunt-edged cannula (hydrodissection). The cannula is used to create multiple tunnels within the subcutaneous fat, inject the dissecting fl uid, and then pulled (rather than cut) from the neighboring struc­tures. The liquefi ed dissected fat is then sucked into a container via the same cannula that injects the dis­secting fl uid. Classically, the amount of injected fl uid
in third space fl uid loss, which produces severe symp­toms. FES is a pathological situation characterized by the development of metastatic fat emboli in multiple body organs, resulting in a triad of pulmonary insuffi ­ciency, cerebral involvement, and petechial rashes. FES can result due to fat droplets leaking into the systemic circulation via ruptured veins at the site of cannula tun­neling (mechanical theory), or due to the liberation of free fatty acids such as “chylomicrons” from the detached adipocytes. These free fatty acids are toxic to the pneumocytes and the capillary epithelium, produc­ing chemical pneumonitis. FES typically manifests within 24–72 h after trauma or liposuction procedure. Neurological manifestations due to fat emboli to the brain include seizures, altered level of consciousness, focal neurological defi cits, and even coma. Patients develop petechial skin rash on the head and neck region and on the upper body, which is believed to be the only pathognomonic feature (seen in 50% of cases). Laboratory fi ndings show decreased hemat­ocrit level, increased serum lipase level, hypoxemia,
380 Chapter 10 Diabetology
https://t.me/medicina_free
and hypokalemia. These fi ndings are observed during the fi rst 24–72 h. Fluminant FES is a term reserved for severe manifestations of cardio-pulmonary obstruction by fat produced by a sudden intravascular liberation of a large amount of fat. Severe heart failure, shock and
10.5
Signs on Radiographs
Pulmonary edema may be seen on chest radiograph if a large amount of fl uid is injected subcutaneously or intravenously, or if the patient develops ARDS due to multiple pulmonary fat emboli. When pulmonary manifestations develop, the radiological signs may remain for up to 3 weeks.
Signs on US
Seromas are detected as localized fl uid (anechoic) collections below the skin, whereas hematoma is seen as hypoechoic to hyperechoic masses, depending on the age of the hematoma.
Fig. 10.5.12. Axial brain DW-illustration of a patient with fat embolism syndrome shows the starfi eld pattern
Signs on MRI
When FES aff ects the brain, it usually results in multiple diff use foci of hyperintensity located in the white matter of the subcortical, periventricular, and centrum semiovale regions. Characteristic multiple hyperintense foci lesions in the centrum semiovale may be seen in DWI, resulting in a “starfi eld pattern” (Fig. 10.5.12 ).
For Further Reading
1 . Bray GA. Obesity. Dis. Mon. 1979;26(1):1–85 2 . Buckley O et al European obesity and radiology depart-
ment. What can we do to help? Eur Radiol (2009);19: 298–309
3 . Koda M et al Sonographic subcutaneous and visceral fat
indices represent in the distribution of body fat volume. Abdom Imaging. 2007;32:387–92
4 . Valchos IS et al Sonographic assessment of regional adi-
posity. AJR Am J Roentgenol. 2007;189:1545–53
5 . Bulum T et al Madelung’s disease: case report and review of
the literature. Diabetologia Croatica. 2007;36-2, 25–30
10.5 Obesity, Gastric Banding, and Liposuction 381
https://t.me/medicina_free
6 . Lange U et al Dercum’s disease (lipomatosis dolorosa): suc-
cessful therapy with pregabalin and manual lymphatic drain­age and a current overview. Rheumatol Int. 2008;29:17–22
7 . Yoshida T et al Weber-Christian disease presenting with
ocular manifestations. Clin Rheumatol. 2003;22:339–42
8 . Nakai M et al Weber-Christian disease presenting with ret-
roperitoneal panniculitis. Eur J Radiol Extra. 2006;60: 89–92
9 . Raguse JD et al Benign symmetric lipomatosis (Madelung’s
disease) complicated by involvement of the facial nerve. Eur J Plast Surg. 2004;27:306–308
10 . Verna G et al Launois-Bensaude syndrome: an unusual
localization of obesity disease. Obes Surg. 2008;18:1313–17
11 . Adami GF et al Metabolic syndrome in severely obese
patients. Obes Surg. 2001;11:543–45
12 . Shen W et al Adipose tissue quantifi cation by imaging meth-
ods: a proposed classifi cation. Obes Res. 2003;11(1): 5–16
13 . Carucci LR et al Adjustable laparoscopic gastric banding
for morbid obesity: imaging assessment and complica­tions. Radiol Clin N Am. 2007;45:261–74
14 . Landen S et al Complications of gastric banding presenting
to the ED. Am J Emerg Med. 2005;23:368–70
15 . Wiesner W et al Adjustable laparoscopic gastric banding in
patients with morbid obesity: radiographic management, results, and post-operative complications. Radiology. 2000; 216:389–94
16 . Trekner SW et al Imaging of morbid obesity procedures
and their complications. Abdom Imaging. 2008. doi:
10.1007/s00261–008–9389–3
17 . Pomerri F et al Radiological assessment of complications after
laparoscopic suprabursal adjustable gastric banding for mor­bid obesity. Obes Surg (2009);19: 146–152
18 . Illouz YG. Complications of liposuction. Clin Plast Surg.
2006;33:129–63
19 . Wang HD et al Fat embolism syndromes following liposuc-
tion. Aesth Plast Surg. 2008;32:731–36
20 . Chen JJS et al MR imaging of the brain in fat embolism syn-
drome. Emerg Radiol. 2008;15:187–92
21 . Reddix RN Jr. et al Crush syndrome presenting three days
after injury. Injury Extra. 2004;35:73–75
382 Chapter 10 Diabetology
https://t.me/medicina_free
10.6
10.6
Lipoatrophic–Lipodystrophic Syndromes
Lipoatrophy syndromes are a wide group of disorders, characterized by diffuse or focal paucity of fatty tissue within the body.
Loss of fat is known as “ lipoatrophy ,” whereas abnormal fat distribution is known as “ lipodystrophy .” When diabetes mellitus occurs with lipoatrophy, it is called “ lipoatrophic diabetes .”
Patients with lipoatrophy syndromes are clinically characterized by focal or diffuse loss of fatty tissue, diabetes mellitus, acanthosis nigricans, hyperandro­genism and amenorrhea in females, cardiomyopathy and muscular hypertrophy, increased appetite and high basal metabolic rate, and nonalcoholic liver steatosis or cirrhosis.
Focal lipoatrophy refers to a condition where the loss of fat involves a single region in the body. An example of focal lipoatrophy is loss of fat in the gluteal area, usu­ally following intramuscular injection (Fig. 10.6.1 ).
Laboratory investigations in lipoatrophy syndromes typically show hyperinsulinemia, hyperglycemia, hyper­triglyceridemia, abnormal cholesterol profi le, elevated free fatty acids, and low leptin and other adipocytes hormones.
In general, radiological features show normal bone mineral density on DEXA scan and hepatic steatosis on ultrasound. However, the conventional radiographic and MRI features depend on the syndrome. Different lipoatrophy syndromes and their characteristic radio­logical features are discussed below.
Congenital Generalized Lipodystrophy (Seip-Berardinelli Syndrome)
Seip-Berardinelli syndrome (SBS) is a disease charac­terized by generalized absence of fat within the fi rst year of life (primarily affects neonates).
As neonates grow up, they develop diabetes melli­tus type 2 before the teenage years. Acanthosis nigri­cans, hypertriglyceridemia, and frequent bouts of pancreatitis are other common features.
The syndrome has an autosomal recessive mode of inheritance; with females showing more severe lipid profi le abnormalities than males. Features of gigantism are often found in patients with SBS.
Talon cusp has been reported in cases with SBS. Talon cusp , also known as “eagle cusp,” is an extra cusp of an anterior tooth. In the premolar teeth, an extra tooth-cusp is referred to as “ dens envaginatus .” When dens envaginatus occurs in an anterior tooth such as an incisor or a canine, it is referred to as a talon cusp (
Fig. 10.6.2 ).
Fig. 10.6.2. An illustration demonstrates talon cusp
Fig. 10.6.1. Axial T1W ( a )
and T1W postcontrast fat-saturation ( b ) images show focal fatty necrosis with enhancement due to previous intramuscular injection ( arrowhead )
10.6 Lipoatrophic–Lipodystrophic Syndromes 383
https://t.me/medicina_free
Signs on Plain Radiographs
Skeletal radiographs usually show advanced bone age plus loss of subcutaneous fat. Craniosynostosis may be seen in some patients (e.g., dolichocephaly).
Signs on CT and MRI
Transverse sections of the torso at the level of the fi fth lumbar vertebra often show markedly defi cient subcutaneous fat and loss of the visceral fat. Liver cirrhosis or steatosis is a common feature of SBS.
Fig. 10.6.3. Axial abdominal CT illustration demonstrates pro­found proliferation of the visceral fat in the retroperitonium ( arrow ), with almost complete absence of subcutaneous fatty tissue ( arrowheads ), which is a typical fi nding in a patient with familial partial lipoatrophy
Familial Partial Lipodystrophy (Dunnigan-Kobberling Syndrome)
Dunnigan-Kobberling syndrome (DKS) is a rare auto­somal dominant disease, characterized by normal fat distribution at birth, with progressive loss of subcuta­neous fat, mainly that located in the extremities and the trunk, as the patient reaches puberty. The loss of subcutaneous fat from the extremities and the trunk is associated with increased fat deposition in the face and the neck as puberty is complete.
Like SBS, DKS shows a more severe course in females than in males. Diabetes and dyslipidemia are seen in much earlier ages in females compared to males. Polycystic ovary disease is often seen in females with DKS.
The presentation of DKS may be clinically mis­taken for Cushing’s syndrome. The absence of fat and the well-reserved muscles in the extremities enable establishing the correct diagnosis.
Signs on CT and MRI
The patient’s body shows loss of the subcutaneous fat in the extremities and increased subcutaneous fat in the head and neck region. Transverse sections of the torso at the level of the fi fth lumbar vertebra often show markedly reduced subcutaneous fat and increased visceral fat (Fig. 10.6.3 ).
Mandibuloacral Dysplasia
Mandibuloacral dysplasia (MAD) is a very rare, multi­systemic, autosomal recessive disease, characterized by mandibular and clavicular hypoplasia (Fig. 10.6.4 ), joint contractures, acro-osteolysis, joint and skin prob­lems, and lipodystrophy.
Patients with MAD present with loss of the subcu­taneous fat of the extremities with increased visceral fat deposition.
Fig. 10.6.4. An illustration demonstrates the clinical fi ndings in a patient with mandibuloacral dysplasia. Notice the hypoplastic mandible (micrognathia)
384 Chapter 10 Diabetology
https://t.me/medicina_free
Signs on Plain Radiographs
Hypoplastic clavicle and mandible (micrognathia).
Loss of the terminal phalangeal tufts (acro-osteolysis).
10.6
Widened skull sutures.
Acquired Generalized Lipoatrophy (Lawrence-Seip Syndrome)
Lawrence-Seip syndrome (LSS) is an acquired gener­alized lipodystrophy disorder. Its clinical features are similar to SBS (congenital form), but it often arises after a triggering event (e.g., infection). The key diag­nosis between the two conditions is the age of presen­tation. SBS typically starts in neonates, while LSS starts in much older patients after a triggering event.
Fat loss in LSS is usually severe, and may lead to a dramatic change in the physical appearance. Patients often develop diabetes mellitus within 4 years from the time they starting losing fat. The fat loss is profound, and it may affect the retro-orbital fat, hands, feet, the genital area, and the bone marrow. Nephrotic syn­drome is often seen in patients with LSS.
Signs on Plain Radiographs
Skeletal radiographs usually show advanced bone age plus loss of subcutaneous fat.
Signs on CT and MRI
Almost the same radiological features as SBS.
Acquired Partial Lipoatrophy (Barraquer­Simon Syndrome)
Barraquer-Simon syndrome (BSS) is a disease with a wide-range of variations, with a few shared features. Patients with BSS are typically women in their second or third decade of life, who also have autoimmune disorder (e.g., scleroderma), presenting with partial loss of fat.
Fig. 10.6.5 . Axial abdominal CT illustration demonstrates pro­found proliferation of the subcutaneous fat in the buttocks ( arrow ) compared to the visceral fat and the anterior abdominal wall subcutaneous fat ( arrowheads ), which is a characteristic fi nding in acquired partial lipoatrophy
The loss of fat in BSS typically starts from the face and descends downward until the gluteal line, with increased fat deposition in the lower extremities. Not all patients with BSS develop diabetes mellitus and dyslipidemia (only 50%).
Signs on CT and MRI
A body section of the upper abdomen shows marked reduced subcutaneous fat compared to the lower pelvis and the lower extremities (Fig. 10.6.5 ).
Parry-Romberg Syndrome (Progressive Facial Hemiatrophy)
Parry-Romberg syndrome (PRS) is a sporadic disease of unknown origin, characterized by slow, progressive atrophy of the face with all its components, involving the skin, subcutaneous tissues, muscles, cartilage, and bones (Fig. 10.6.6 ). Some authors consider PRS a focal form of lipoatrophy affecting the face, while other authors consider it a form of phakomatosis.
PRS starts at a young age, and slowly progresses as the patient gets older. Patients commonly present with epilepsy and brain abnormalities ipsilateral to the facial atrophy. Bilateral facial atrophy is seen in 5–10% of cases, and atrophy of the ipsilateral eye or enopthal­mus is seen in 10–35% of cases.