Editorial
An Update on the Surgical Classification of Brainstem
Tumors
Glenn Morrison, MD
Miami Children's Hospital
Neuroimaging of
Phakomatoses
Nolan Altman, MD
Abstract
The phakomatoses are a group of disorders
characterized as dysplasias or neoplasms of organs derived from the embryonic ectoderm.
They have common features of neuroepidermal maldevelopment and undifferentiated cells with
disturbed patterns of cell migration. Neuroimaging, primarily MRI plays an important role
in the diagnosis, followup and treatment of these disorders. This article will discuss and
illustrate the imaging findings for the most commonly encountered phakomatoses
(neurofibromatosis tuberous sclerosis and Sturge-Weber syndrome) as well as some of the
more unusual disorders (ataxia telangiectasia, neurocutaneous melanosis, Proteus syndrome,
basal cell nevus syndrome, hypomelanosis of Ito, linear sebaceous nevus syndrome and
cutaneous hemangioma vascular complex). Int Pediatr. 2000;15(1):4-14.
Key words: phakomatoses, pediatric
neuroradiology
The Surgical Classification and Management of
Braintem Tumors in Children
John
Ragheb, MD; Fred J. Epstein, MD
Abstract
Brainstem tumors in children are a
heterogeneous group of neoplasms that may be classified according to clinical and
radiographic criteria. We have described a simple anatomic classification that has
significance pathologically, surgically, and prognostically. Although there is no surgical
option forthe most common diffuse brainstem neoplasms, the other subtypes may be operated
upon if the clinical and radiographic information warrants. Int Pediatr.
2000;15(1):15-20.
Key words: brainstem, tumors,
surgery
Juvenile Dermatomyositis: A Review
Geetha
Chari, MD; Teresita A. Laude, MD
Abstract
Juvenile
dermatomyositis is a systemic vasculopathy, affecting mainly the skin and muscle. In the
United States, it is seen in more than three per million children per year. It is
diagnosed on the basis of the criteria set by Bohan and Peter. The following review
describes the characteristic clinical manifestations, the pathophysiology and immunology
of the disease. The various treatment modalities are discussed. Int Pediatr.
2000;15(1):21-25.
Key words: juvenile
dermatomyositis, vasculopathy
When to Start and When to Stop Anti-Epileptic
Drugs in Chhildhood Epilepsy
Peter Camfield, MD; Carol Camfield, MD
Abstract
Anti-epileptic medications
do not cure epilepsy – they reduce the number of seizures without apparently having
an effect on long-term remission. Reasons for treatment include – fear of brain
damage from seizures, fear of kindling, fear physical injury, fear of death and fear of
social consequences. None of these reasons is so compelling as to require routine
medication. Some children may not require treatment at all (Benign epilepsies and those
with excellent prognosis). Drug choice in part depends on the epilepsy syndrome. If the
child becomes seizure free for only one year, treatment may be discontinued successfully
in about 60-70%. Some children may benefit from treatment for less than one year. Those
with Juvenile Myoclonic Epilepsy and some with poor prognostic indicators may be
considered for longer treatment. Treatment decisions can be safely individualized. Int
Pediatr. 2000;15(1):26-29.
Key words: Anti-epileptic medications,
epilepsy, Juvenile Myoclonic Epilepsy
Current Diagnosis and Management of Appendicitis in Children
Juan E. Sola, MD;
Whitney McBride, MD; Juan Rachadell, MD
Abstract
Appendicitis is a
common condition affecting children. Nevertheless, up to half of all pediatric patients
continue to be diagnosed late in their disease process once perforation has occurred with
its attendant higher morbidity and mortality. Early surgical consultation and use of
diagnostic imaging modalities such as ultrasound, computed tomography, and laparoscopy may
decrease the perforation rate in equivocal cases. Preoperative preparation consists of
aggressive fluid resuscitation and antibiotic therapy. Appendectomy can then be performed
either open or laparoscopically depending on the surgeon’s or patient’s
preference. Interval appendectomy may be a less morbid and more cost-effective way to deal
with perforated appendicitis in children over 6 years of age.Int Pediatr.
2000;15(1):30-32.
Key words: appendicitis, perforated
appendicitis, interval appendectomy, laparoscopy, children.
Congenital Toxoplasmosis
M. Pataki, MD; Z. Meszner, PhD; R. Todorova, MD
Abstract
The aim of study was
to make an overview of clinical signs caused by congenital toxoplasmosis and methods of
diagnosis in Budapest, Hungary. The most frequent sign of congenital infection among our
patients were cerebral ultrasonographic changes (calcification, hydrocephalus,
microcephaly), neurologic disturbances (muscle tone disturbances, seizures) and
chorioretinitis. We observed hepatic failure rarely. The differencial diagnosis of
ultrasonographic changes included as other causes of hydrocephalus (hemorrhage, CMV or
other infections) as other causes of calcification (CMV or other infections) too. The
differential diagnosis of eye lesions included colobomatous defect, other inflamatory
lesions (HSV, CMV), birth injury (intraocular hemorrhage) circulatory disturbances or
neoplasm. Int Pediatr. 2000;15(1):33-36.
Key words: congenital toxoplasmosis,
calcification, hydrocephalus, microcephaly, seizures, chorioretinitis
A New Case of Interstitial 12q Deletion
Marta Gallego, PhD; Cristina Barreiro, MD; Miriam Pérez, PhD; Hugo Arroyo, MD;
Jorge Menehem, MD
Abstract
We report on a 20
month-old child with an interstitial deletion of the long arm of chromosome 12 with
breakpoints q11 to q13. To our knowledge this is the fifth reported case with deletion of
the long arm of chromosome 12. His clinical manifestations include mild psychomotor
retardation, minor congenital anomalies with mental and growth retardation, and strabismus
due to limited abduction probably associated with congenital fibrosis of the extraocular
muscles. The association of deletion of 12q and strabism has previously been reported in
one case. Int Pediatr. 2000;15(1):37-40.
Key words: deletion (12q); mental
retardation, congenital minor anomalies, strabismus
Persistent Müllerian Duct Syndrome
Alejandro Diaz, MD; Adriana Carrillo, MD; Mislen Bauer, MD; Andrew Labie, MD; Marco Danon,
MD
Abstract
Persistent
Müllerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism defined by
the presence of Müllerian derivatives in otherwise normally virilized XY subjects. We
report a case of a male infant with initial bilateral undescended testes diagnosed with
PMDS. The molecular alterations in the Müllerian Inhibiting Factor (MIF) and in its
receptor have been described. It is possible to determine serum MIF levels, allowing an
accurate study of babies with cryptorchidism. Int Pediatr.2000;15(1): 41-43.
Key words: Müllerian duct syndrome
(PMDS), pseudohermaphroditism, Müllerian Inhibiting Factor (MIF), cryptorchidism
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