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Biomedical subjects

K Vandenberghe

Publications and source records attributed to K Vandenberghe.

At least 55 records · Page 3Linked to original sources
PubMed1994

A model for fetal surgery through intrauterine endoscopy.

Video-endoscopic technology may offer new hope for the future of fetal surgery. Ten time-dated pregnant ewes (95 to 105 days; term, 145 days) were used to develop a model of fetal surgery through intrauterine endoscopy. Special 5-mm ports were designed with short, flexible, balloon-tipped shafts. The cannulas were inserted through purse-stringed hysterostomies. Amnioinfusion of warmed physiological fluid (Hartmann) was used as the work medium. Fetal heart rate and oxygen saturation (pulse oximetry), temperature, and amniotic pressure were monitored continuously through endoscopically placed sensors. Surgical procedures included subcutaneous dissection of the abdominal wall and ligation of the urethra in the male fetus (n = 7), ligation of the urachus within the umbilical cord (n = 8), and endoscopy of the fetal esophagus and trachea (n = 6). Bloodless surgery was achieved with a novel coagulation device that allows bipolar cutting and coagulation in an electrolyte-rich fluid medium. The mean fetal operative time was 147 minutes (range, 130 to 195). The intraamniotic temperature was maintained between 38.0 +/- 0.9 degrees C and 41.7 +/- 1.7 degrees C throughout the procedure. Oxygen saturation ranged from 51 +/- 20% to 72 +/- 14%, and fetal heart rate from 120 to 140 beats per minute. In conclusion, intrauterine endoscopy in a fluid medium allows fully monitored surgical procedures, lasting more than 2 hours, to be performed on the fetal lamb.

Animals↗
PubMed1994

Fetoscopy-guided fetal endoscopy in a sheep model.

Video-endoscopic surgical treatment of the fetus may, in the future, become an alternative to open fetal operation. Six 95-day-old fetal lambs were examined through intrauterine endoscopy using amnioinfusion and specially designed balloon-tipped cannulas. The fetuses were monitored endoscopically for oxygen saturation, heart rate and temperature throughout the procedure. With a rigid 5 millimeter telescope and a flexible 3 millimeter endoscope, the respiratory and upper gastrointestinal tracts could be examined. No mucosal or other trauma was noted upon completion of the endoscopy; all lambs survived the procedure. While the applications of fetal endosurgery are likely to be similar to those of open fetal operation, fetal endoscopy (looking in the fetus) is a new entity for which clinical relevance (rather than mere feasibility) remains to be demonstrated. This technique, however, could become a valuable research tool to study fetal gastrointestinal and pulmonary physiologic factors in situ.

Animals↗
PubMed1993

Congenital pulmonary lymphangiectasis with chylothorax: a heterogeneous lymphatic vessel abnormality.

We report on 7 perinatal autopsy cases of primary congenital pulmonary lymphangiectasis (CPL) with bilateral chylothorax. This study demonstrates that primary CPL is often complicated by chylous pleural effusions with ensuing pulmonary hypoplasia. Conversely, CPL appears to be a constant pathological finding in spontaneous congenital chylothorax. These observations indicate a common pathogenesis for both disorders. The basic defect is not an intrinsic lung abnormality, but a developmental error of the lymphatic system resulting in a pulmonary lymphatic obstruction sequence. The cause of CPL is heterogeneous. Apparently, most cases are sporadic occurrences. We report the second instance of CPL in sibs. This indicates that some cases are genetically determined with autosomal recessive inheritance. CPL may also be part of a multiple congenital anomalies (MCA) syndrome such as Noonan, Ullrich-Turner, and Down syndrome.

Abnormalities, Multiple↗
PubMed1993

Goldston syndrome reconsidered.

We describe two siblings with the combination of central nervous system malformations, renal dysplasia and hepatic ductal plate malformation. Based on the occurrence of a cerebellar Dandy-Walker malformation (variant), the diagnosis of Goldston syndrome was proposed in the first case. The second sibling however, showed a typical Meckel syndrome with occipital encephalocele. Both cases had a cranium bifidum with two defects in the squamous part of the occipital bone. This observation confirms that Goldston syndrome is not a distinct entity but merely a variant of Meckel syndrome, and that a cerebellar Dandy-Walker malformation can be a component of Meckel syndrome.

Abnormalities, Multiple↗
PubMed1992

Constrictive amniotic bands, amniotic adhesions, and limb-body wall complex: discrete disruption sequences with pathogenetic overlap.

In a fetopathologic evaluation of 18 cases with amniotic bands, we discerned 3 types of lesions: (1) constrictive tissue bands, (2) amniotic adhesions, and (3) more complex anomaly patterns, designated as limb-body wall complex (LBWC). Constrictive bands are caused by primary amnion rupture with subsequent entanglement of fetal parts (mostly limbs) by shriveled amniotic strands. Adhesive bands are the result of a broad fusion between disrupted fetal parts (mostly cephalic) and an intact amniotic membrane. Most of the craniofacial defects (encephaloceles and/or facial clefts) occurring in these fetuses are not caused by constrictive amniotic bands, but are the result of a vascular disruption sequence with or without cephalo-amniotic adhesion. Our observations confirm the fact that amnion rupture is not a conditio sine qua non for the development of LBWC. However, LBWC is often complicated by rupture of the unsupported amnion with ensuing formation of constrictive bands. We think that the concept that considers the 3 lesions in question as a single pathogenetic entity is erroneous and will inevitably lead to a never-ending debate between followers of the 2 prevailing theories. In our view, the theories of Streeter and Torpin are not mutually exclusive but rather apply to different types of lesions. The recognition of constrictive amniotic bands, amniotic adhesions, and LBWC as discrete but often combined disruption sequences with important pathogenetic overlap may resolve many dilemmas in interpretation when a fetus exhibits classical constrictive bands beside more severe defects.

Amnion↗
PubMed1992

Pathogenesis of congenital cystic adenomatoid malformation of the lung.

Congenital cystic adenomatoid malformation is a rare developmental abnormality of the lung. In most earlier reported cases, the anatomy of the bronchial tree was poorly documented. We describe four cases studied following autopsy. Post-mortem bronchography or serial microscopical examination showed segmental bronchial absence or atresia in each of them. Our observations provide further evidence pointing to bronchial atresia as being the primary defect leading to the development of congenital cystic adenomatoid malformation. The morphology of the lesion, i.e. the type of malformation, is determined by the extent of dysplastic lung growth beyond the atretic segment. The aetiology of the bronchial atresia is probably heterogeneous and may either represent a primary malformation, or be the result of vascular disruption.

Autopsy↗
PubMed1992

Laryngeal atresia sequence as part of the DiGeorge developmental field defect.

The subject of the present report is a male newborn with laryngeal atresia (LA) type I, giving rise to a malformation sequence consisting of overdistended polyalveolar lungs and nonimmune fetal hydrops with massive ascites. The infant was chromosomally normal and the first child of consanguineous parents. Retention of liquid secreted by the fetal lungs in utero was the pathogenetic mechanism, responsible for the LA sequence. The LA was part of a complex constellation of anomalies, compatible with the DiGeorge developmental field defect.

Asphyxia Neonatorum↗
PubMed1990

Pathogenesis of the lethal multiple pterygium syndrome.

We present autopsy studies in 4 unrelated fetuses with the lethal multiple pterygium syndrome (LMPS) with special emphasis on the neuromuscular system. The data suggest that LMPS combines the manifestations of a jugular lymphatic obstruction sequence with those of an early severe fetal akinesia sequence. The jugular lymphatic obstruction sequence with resultant edema and cystic hygroma colli causes fetal lethality usually in the second trimester of pregnancy. Generalized amyoplasia appears to be an important mechanism in the pathogenesis of fetal akinesia as part of LMPS and is not associated with dysgenesis or degeneration of the central nervous system (CNS) but is apparently the result of an early fetal muscular "dystrophy." We propose a genetically determined insult affecting the early embryonic development of both lymph vessels and muscles as the basic defect in LMPS. Placental structure, studied in all 4 cases, demonstrated that triploidy-like placental lesions are specific to LMPS. The present findings suggest that LMPS may be a less heterogeneous entity than previously proposed.

Abnormalities, Multiple↗
PubMed1990

Neonatal haemochromatosis.

Four cases of neonatal haemochromatosis presenting as fulminant hepatic failure in the newborn were diagnosed by autopsy. In all four cases the diagnosis was made by histochemical demonstration of excessive iron deposition in hepatocytes and extrahepatic parenchymal cells, particularly pancreatic acinar epithelium, thyroid follicular epithelium and distal renal tubules. No haemosiderin was detectable in the extrahepatic mononuclear-phagocytic cells of the spleen, lymph nodes and bone marrow. The liver was the most severely affected organ. The hepatic haemosiderosis was associated with massive hepatocellular necrosis of prenatal onset in three patients, one of whom showed formation of regenerative nodules, establishing true congenital cirrhosis. Other inconstant findings included giant cell transformation, diffuse sinusoidal fibrosis with segregation of small groups of hepatocytes and cholestasis with pseudoacinar change of liver cell plates. The fetal liver disease had its onset in the late second trimester of pregnancy and was reflected clinically by severe panhypoproteinaemia with non-immune hydrops; hyperbilirubinaemia and haemorrhagic diatheses were apparent in the newborn. Neonatal haemochromatosis is a metabolic disorder, probably of autosomal recessive inheritance. The site and nature of the basic defect remain uncertain. Pathologists should be aware of this condition and its potential recurrence in subsequent pregnancies.

Female↗
PubMed1988

Holzgreve-Wagner-Rehder syndrome: Potter sequence associated with persistent buccopharyngeal membrane. A second observation.

Here we present a second example of the syndrome first reported by Holzgreve et al. [1984] in this journal, i.e., Potter sequence with persistent buccopharyngeal membrane type II, postaxial polydactyly, cleft palate, cardiac anomalies, intestinal nonfixation, and intrauterine growth retardation. This specific complex MCA syndrome is not associated with a detectable chromosome abnormality.

Abnormalities, Multiple↗
PubMed1988

The syndrome of diaphragmatic hernia, abnormal face and distal limb anomalies (Fryns syndrome): report of two sibs with further delineation of this multiple congenital anomaly (MCA) syndrome.

We describe 2 sibs with the syndrome of diaphragmatic hernia, abnormal face, and distal limb anomalies. Both infants died shortly after birth with severe respiratory distress. Postmortem examination showed gross internal anomalies: Dandy-Walker malformation, ventricular septal defect, and renal cystic dysplasia. This combination of anomalies, also termed the Fryns syndrome, appears to be a distinct MCA syndrome with variable expression and probable autosomal recessive inheritance. Prenatal ultrasonographic diagnosis was successful in both patients.

Abnormalities, Multiple↗
PubMed1988

The fetal phenotype in 15q2 duplication.

In this report we summarize the findings in a prenatally diagnosed male fetus with 15q2 trisomy. The craniofacial findings were identical to those in liveborn patients with this type of partial autosomal trisomy. A short review of the 15q2 trisomy syndrome is presented.

Chromosome Aberrations↗