4.

Arnold-Chiari Malformation: Report of a Case with Contamination of Ventricular and

Subarachnoid Spaces by Amniotic Sac Contents

Dimitris P. Agamanolis,Marvin S. Platt &John H. Vollman

Pages 89-94 | Accepted 15 Feb 1983, Published online: 09 Jul 2009

Cite this article https://doi.org/10.3109/15513818409041190

Abstract

An infant with the Arnold-Chiari malformation had a lumbosacral amniotic-central nervous system fistula
that allowed massive amounts of squamous cells and lanugo hair to enter the ventricular and
subarachnoid spaces causing blockage, gliosis, and fibrosis. This rare complication can be diagnosed by
cytologic examination of cerebrospinal fluid and should be considered in the prognosis of Arnold-Chiari
malformation and selection of cases for treatment.

The exact cause of Chiari malformations is not known, but often the cavity near the base of the skull
(posterior fossa) is narrow and abnormally small in relation to the size of the cerebellum, which this
portion of the skull encloses. Researchers believe that in some cases the small posterior fossa may cause
the developing brain, specifically the cerebellum and the brainstem, to be pushed downward. Part of the
cerebellum (known as the cerebellar tonsils) may protrude (herniate) through the foramen magnum,
which is the normal opening found in the occipital bone at the base of the skull. The tonsils may thus
interfere with the flow of cerebrospinal fluid (CSF) to and from the skull and spinal canal, potentially
leading to accumulation of cerebral spinal fluid in the subarachnoid spaces of the brain and spine. In
relation to the findings of Agamanolis, Platt and Vollman (2014), another form of malformation shows a
lumbosacral amniotic-central nervous system fistula that allowed massive amounts of squamous cells
and lanugo hair to enter the ventricular and subarachnoid spaces causing blockage, gliosis, and fibrosis
with specific signs and symptoms, requiring a specific type surgical and medical treatment.

Cross Reference Chiari Malformations https://rarediseases.org/rare-diseases/chiari-malformations/

5. Pathology of Suspected Acquired Immune Deficiency Syndrome in Children: A Study of Eight Cases

V. V. Joshi,J. M. Oleske,A. B. Minnefor,R. Singh,T. Bokhari &R. H. Rapkin

Pages 71-87 | Received 13 Apr 1983, Accepted 01 Aug 1983, Published online: 09 Jul 2009

Cite this article https://doi.org/10.3109/15513818409041189

Abstract

Biopsy and/or autopsy material from lymphoreticular and other organs was studied in 8 children with
suspected acquired immune deficiency syndrome (AIDS). One or both parents of each of these children
had one or more of the recognized risk factors for AIDS, such as intravenous drug abuse, prostitution,
Haitian origin. The following histologic patterns were noted in the lymph nodes: (1) follicular hyperplasia
with normocellular paracortex, (2) follicular hyperplasia with depletion of paracortex, and (3) atrophy of
follicles with depletion of paracortex. Lymphoid interstitial pneumonitis (LIP), a previously unreported
lesion in AIDS, was present in 4 cases. It is suggested that the pulmonary lymphoid lesion may be part of
a more generalized lymphoid hyperplasia involving B cells. The gross and microscopic features of the
thymus, available in 2 of the 8 cases, indicated that the immunologic defect in these children was not of
congenital type. Pathologic findings can be helpful in the diagnosis of the syndrome when correlated
with clinical and immunologic features of suspected cases and of the pulmonary lesion. The latter is of
importance in deciding the type of therapy to be given for the pulmonary disease process.

Cross Reference

Vijay V Joshi Pathology of Acquired Immunodeficiency Syndrome (AIDS) in Children

The Keio Journal of Medicine 1996 Volume 45 Issue 4 Pages 306-312
https://doi.org/10.2302/kjm.45.306

Acquired Immunodeficiency Syndrome (AIDS) was first described to occur in children in 1983. With
experience of increasing number of cases of AIDS, pathologic lesions in various organs and tissues such
as lungs, brain, G.I. tract, heart, blood vessels, lymph nodes, spleen, bone marrow, etc. became evident
in autopsy and biopsy specimens. In recent years the emphasis of pathologic study is on the reactive and
neoplastic proliferative disorders. These disorders include nodal and extranodal lymphoproliferative
lesions (such as myoepithelial sialadenitis, malignant lymphomas, etc) In terms of pulmonary lymphoid
lesion, it specifically identified a more generalized lymphoid hyperplasia involving B cells. The gross and
microscopic features of the thymus, available in 2 of the 8 in the above cases, indicate that the
immunologic defect in these children was not hereditary or congenital in nature, which is significant in
the type of therapeutic intervention specific for the pulmonary disease category.