(Lawrence syndrome, Lawrence-Seip syndrome)

Rare. Associates loss of adipose tissue and insulin resistance. The loss of adipose tissue is extended to large areas of the body: face, legs, arms... . The phenotype is close to Berardinelli syndrome except that the clinical signs appear at the end of childhood, during adolescence or adulthood. This condition is most common in women: sex ratio of 3: 1.

There are 3 types of acquired generalized lipodystrophy:

1)        panniculitis: inflammatory nodules above the lipoatrophy (25%)

2) autoimmune: associated with other autoimmune diseases: Hashimoto thyroiditis, hepatitis, dermatomyositis

3) idiopathic

The anti-HIV (stavudine) treatment can also cause generalized or localized lipodystrophy (face).

Other clinical signs:

-        insulin-resistant diabetes

-        voracious appetite

-        acanthosis nigricans: brown or black rough lesions, at the level of the flexion creases (neck, underarms, groin).

-        hepatomegaly with steatosis and risk of cirrhosis

-        girls: hirsutism, polycystic ovary syndrome

-        severe hypertriglyceridemia with risk of acute pancreatitis

-        proteinuria following glomerulosclerosis or membrano-proliferative glomerulonephritis.

Treatment: metformin, insulin, hypolipidemic agents

Anesthetic implications:

management of the insulinotherapy and cardiovascular risk (hypertension, angina). Monitor the BP, ECG, cardiac ultrasound. Check hepatic and renal functions. In case of hypertriglyceridemia, avoid total intravenous anesthesia with propofol: risk of acute pancreatitis.

One case rport of delayed awakening after a 70 minutes long sevoflurane anesthesia. In another case, it has been shown that delayed awakemening (and induction) is directly linked to the increased solubility of sevoflurane in the presence of hyperlipidemia.

References : 

• Capeau J, Magré L, Lascols O, Caron M, Béréziat V, Vigouroux C. 
  Lipodystrophies primitives. 
  Encyclopédie Orphanet février 2007
• Bustamante SE, Appachi E. 
  Acute pancreatitis after anesthesia with propofol in a child with glycogen storage disease type IA. 
  Pediatr Anesth 2006; 16: 680-3.
• Steen ER, De Baerdemaeker LEC, Van Limmen E, Wouters P. 
  Some pharmacokinetics of sevoflurane in a child with severe lipodystrophy. 
  A & A Case Reports 2014; 2: 61-4.
  
  

Updated: January 2019