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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3644_Библиотеки_им_академика_М_И_Перельмана
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463Associated Features
Fig. 8.5E–G. Legend see p. 462
8Venous Anomalies and Malformations464
Fig. 8.6A–C. A 5-year-old boy presenting with a vascular lesion located on the midline and associated orbital phlebolith. Diagnosis of sinus pericranii was easily made.
Note the association between transcranial drainage with a large bony defect (arrow)
as well as an associated developmental venous anomaly (DVA). Despite the cosmetic
problem, there was no neurocognitive problem.A Plain frontal skull X-ray.B,C Inter-
nal carotid angiogram in lateral early and late phases.Associated frontal DVA draining into a hemispheric vein (open arrow). Note the hyperemic aspect of the frontal
parenchyma.A large pouch (arrows) is progressively filled and bulges subcutaneously after producing a well-circumscribed bony defect
Two associations are encountered in children: maxillofacial vascular
malformations, in particular those of the venous (Boukobza et al. 1996)
and lymphatic type (Figs. 8.32, 8.36), dural sinus malformations (see
Chap. 7, this volume, and Scheme 8.1), multiple mucocutaneous venous
malformations (MMCVM, or blue rubber bleb nevi or Bean syndrome),
and cerebral cortical malformations (Barkovich 1988).
Associated features of DVA in children include the following:
Maxillofacial venous malformations
Maxillofacial lymphatic malformation
Sinus pericranii
Schizencephaly
Pachygyria
Microgyria
Cavernomas
Dural sinus malformation
Multiple mucocutaneous venous malformations
What might cause a DVA to develop occurs at the embryonic stage and
seems topographically unrelated to the type and segmental distribution
of maxillofacial malformations. The association seems totally fortuitous
and the DVA is rather considered as a time marker for the malformation
with which it is associated.Both DVA and a sinus pericranii can be simultaneously present (Figs. 8.6–8.10).The diagnosis is discussed in the presence of a midline frontal varix; pretherapeutic evaluation must determine whether the varix communicates with the sinus and whether it
drains any normal brain. The presence of a midline-located varix on the
face does justify the search for an anomalous cerebral drainage prior to
any removal or occlusion.The presence of a neurological symptom in the
clinical history of a facial vascular malformation, regardless of its type,
certainly requires intracranial screening; however,the discovery of a DVA
should not be considered as the appropriate explanation for the neurological manifestations noted.
465Associated Features
Scheme 8.1. Vascular diseases according to the arterio-veno-lymphatic tree
8Venous Anomalies and Malformations466
Fig. 8.7A–C. A 28-year-old female patient complaining of cosmetic problems related
to a subcutaneous midline varix of the forehead (A). The plastic surgeon prior to
surgery requested an angiogram. B, C Left and right venous phases of the respective
internal carotid angiograms demonstrate complex nonmalformative venous anomalies. The varix was identified as a sinus pericranii draining part of the right cerebral
hemisphere. Treatment of this varix was not and should not be undertaken.Note the
specific involvement of the striate system
467Associated Features
Fig. 8.8. A A 7-year-old girl presenting with a large frontal varix. Following repeated
attempts to correct the lesion surgically (without angiographic study), cheloid scar
and bone hypertrophy were observed, together with a parallel increase in the soft,
nonpulsatile expansile frontal varix. BMRI disclosed a small communication with the
superior sagittal sinus. C Following direct puncture into the sinus pericranii,catheterization of the small transosseous venous communication was achieved and occluded
with coils.Subsequent surgery allowed removal of the varix.At 15 years of follow-up,
the lesions at the nose persist (D)
The association with cortical migration anomalies was stressed by
Barkovich (1988). It is not rare but seldom proven, as angiographic
studies have only rarely been performed in such patients. The presence
of various types of focal cortical sulcation and cellular migration anomalies (nonlissencephalic dysplasias or those of the pachygyric (Figs. 8.11,
8.12) or polymicrogyric (Fig. 8.13) type, as well as schizencephalic clefts
(Fig. 8.14)), suggests the multiple opportunities for DVAs to develop during embryogenesis. It points to the role played by the transcerebral
venous system in the cortical migration process. This does not mean that
the venous anomaly is responsible for the cortical changes,but illustrates
the close relation in topography and time between the venous maturation
process (from the striatal veins and transhemispheric balance set-up)
and the cell migration from the germinal matrix.
8Venous Anomalies and Malformations468
Fig. 8.9A–C. Unusual type of
lateral sinus pericranii.Note
the converging pattern of
the deep venous system in the
inferior striate vein (A).
Secondary drainage runs on
the orbital roof and fills a
midline-located varix (B, C).
(Courtesy of P.Burrows)
469Associated Features
Fig. 8.10A–J. A 28-year-old female patient who presented, approximately 15years
ago,with a soft tissue mass in the right forehead and over the orbit. There were some
prominent “veins” in the forehead. There was no change during pregnancy and/or
during her menstrual cycles. The lesion has significantly increased in size in more recent months. The lesion is more prominent with Valsalva maneuvers. Physical examination demonstrates some pinkish discoloration in the skin. MRI (A,B) demonstrates
a lesion with an extension from the diplopic space into the dura. The MRI confirms
the intraorbital prominence of vascular structures (C–E). There are associated DVAs
in the RT basal ganglia and RT cerebellum.Angiography shows the complex venous
lesions involving the orbit, associated with several DVAs and a large frontal varix
(F–H). The external carotid artery opacifies the frontal venous varix via an inflammatory type of capillaries. (I, J)The lesion is considered to be typically venous despite
these minute AV shuntings.E–J see p.470
8Venous Anomalies and Malformations470
Fig. 8.10E–J. Legend see p.469
471Associated Features
Fig. 8.11A–C. A 7-year-old
child presenting with seizures
and a moderate, right-sided
deficit due to neonatal hemiparesis.A,B Angiography
demonstrated several cortical
venous anomalies.
C CT demonstrates pachygyria
at the level of one of the venous
dispositions
8Venous Anomalies and Malformations472
Fig. 8.12A–D. A 4-month-old child presenting with convulsions and neurocognitive
delay (actual age, 4 years; tested age, 2.5years). At the age of 4 months, the child fell
from a height of 3 m.Twenty days later,he had a partial convulsive crisis. He has been
receiving antiepileptic treatment since then. A, B Note the peculiar dysplastic aspect
of the frontoparietal veins on the right side. C, D MRI and CT also suggest some
degree of frontal pachygyria
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