MIAMI - The plan had been to remove only part of the large growth distending Marlie Casseus' upper
face and leave the rest for future surgeries, but the operation went so well that doctors just kept
Marie Casseus before surgery
By the time they had tied off the last of about 2,500 stitches, surgeons had opened the 14-year-old
Haitian girl's face like a book and excised 9 pounds of jelly-like fibrous tissue, bone fragments,
teeth and fluid, doctors reported Friday.
With titanium mesh, they constructed a new framework for her facial features, which, lacking the
support of the underlying growth, would have collapsed into an empty cavity.
Surgeons, who began the operation early Wednesday and completed it nearly a day later, formed an
upper lip, reduced the size of her overstretched mouth by 70 percent and - having been surprised to
find her nasal septum intact - reopened her crushed nostrils with plastic "trumpets."
She lost 40 percent of her blood during surgery at the University of Miami/Jackson Memorial Medical
Center's Holtz Children's Hospital, and received six units of replacement blood.
After surgery she looks like the picture below
Marlie has McCune-Albright syndrome, a genetic condition that includes the uneven bone growth of
polysostic fibrous dysplasia, and endocrine abnormalities.
The condition affects much of her bone structure and could return to her face, doctors cautioned.
She appeared to be growing normally for the first few years of her life, but by age 9, the mass had
so distorted her face that she quit school.
By the time she came to Miami 10 weeks ago, she could barely breathe.
McCune Albright Syndrome
It is possible that the main title of the report McCune Albright Syndrome is not the name you
expected. Please check the synonyms listing to find the alternate name(s) and disorder
subdivision(s) covered by this report.
Osteitis Fibrosa Disseminata
Polyostotic, Fibrous Dysplasia
Precocious Puberty with Polyostotic Fibrosis and Abnormal Pigmentation
McCune-Albright Syndrome (MAS) is a rare multisystem disorder characterized by (1) replacement of
normal bone tissue with areas of abnormal fibrous growth (fibrous dysplasia); (2) patches of
abnormal skin pigmentation (i.e., areas of light-brown skin cafe-au-lait spots with jagged borders);
abnormalities in the glands that regulate the body's rate of growth, its sexual development, and
certain other metabolic functions (multiple endocrine dysfunction). Depending on the number and
location of the skeletal abnormalities, mobility may be impaired, as well as vision and/or hearing,
and the individual may experience substantial pain. Malfunctioning endocrine glands can result in
the development of secondary sexual characteristics at an age younger than normal (precocious
McCune-Albright Syndrome is the result of a genetic change (mutation) that occurs randomly, for no
apparent reason (sporadic). In individuals with the disorder, this sporadic genetic mutation is
present in only some of the body's cells (mosaic pattern). The symptoms and physical characteristics
associated with the disorder vary greatly from case to case, depending upon the specific body cells
and tissues that are affected by the genetic mutation. This mutation occurs after fertilization
(postzygotic somatic mutation). It is not inherited from the parents.
The range of severity of the disorder is very broad: some children are diagnosed in early infancy
with obvious anomalies of bone and increased hormone production by one or more of the endocrine
glands; others show no evidence of bone, skin or endocrine malfunction in childhood and may enter
puberty at an appropriate age.
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