1/73. Diprosopus (partially duplicated head) associated with anencephaly: a case report.Craniofacial duplication (diprosopus) is a rare form of conjoined twin. A 16 year old mother with a twin pregnancy delivered one normally formed baby boy and one diprosopus male. The malformed baby was 33 weeks of gestation with a single trunk, normal limbs and various degrees of facial duplication. Of the following structures there were two of each: noses, eyes, ears (and one dimple), mouths, tongues and, with bilateral central cleft lips and cleft palates. This was associated with holoprosencephaly and craniorachischisis. Internal organs showed no duplication. There were multiple congenital anomalies including diaphragmatic hernia, small lungs, two lobes of the right lung, ventricular septal defect, small adrenal gland and small left kidney with short ureter. The body also had a short neck, small chest cavities and kyphosis. X-ray revealed duplication of the vertebral column. The case presented here represents a type II of diprosopia of Rating (1933) and is the least common type reported. We also reviewed 22 recently reported cases of diprosopus. In addition to facial duplication, anencephaly, neural tube defect and cardiac malformations represent the more common congenital abnormalities associated with diprosopus. The pathogenesis of diprosopus is not well understood. Factors that play a role in diprosopus are probably similar to those factors (genetic, environmental and abnormal placental circulation) which affect monozoygotic twins as observed in this case report. Early ultrasonography diagnosis of diprosopus permits one to consider a vaginal therapeutic abortion.- - - - - - - - - - ranking = 1keywords = mouth (Clic here for more details about this article) |
2/73. Disseminated intravascular meconium in a newborn with meconium peritonitis.A 3-day-old premature infant with meconium peritonitis, periventricular leukomalacia, and pulmonary hypertension died with respiratory insufficiency. An autopsy disclosed intravascular squamous cells in the lungs, brain, liver, pancreas, and kidneys. Numerous pulmonary capillaries and arterioles were occluded by squamous cells, accounting for pulmonary hypertension. brain parenchyma surrounding occluded cerebral vessels showed infarct and gliosis. A mediastinal lymph node filled with squamous cells alluded to the mechanism by which these cells from the peritoneal cavity likely entered the bloodstream--namely, via diaphragmatic pores connecting with lymphatics. Thus, disseminated intravascular meconium rarely may complicate meconium peritonitis and have devastating consequences.- - - - - - - - - - ranking = 1.5367688274398keywords = cavity (Clic here for more details about this article) |
3/73. Antenatal sonographic diagnosis of epignathus at 15 weeks of pregnancy.Epignathus is a rare, benign, congenital teratoma of the hard palate. Most of these teratomas are unidirectional and protrude through the mouth. Hence, the prognosis depends on the size of the tumor and the degree of face distortion and airway obstruction that it causes. However, some epignathi protrude bidirectionally, involving and destroying the brain tissue, resulting in a poor prognosis. This report presents a case of ultrasonographic detection of a bidirectional epignathus at 15 weeks of pregnancy.- - - - - - - - - - ranking = 1keywords = mouth (Clic here for more details about this article) |
4/73. prenatal diagnosis in a family with severe type I plasminogen deficiency, ligneous conjunctivitis and congenital hydrocephalus.Severe type I plasminogen deficiency may cause severe ligneous conjunctivitis, a rare and unusual form of chronic pseudo-membranous conjunctivitis that usually starts in early infancy, but also pseudo-membranous lesions of other mucous membranes in the mouth, nasopharynx, trachea and female genital tract, and in rare cases congenital occlusive hydrocephalus. The index patient, the daughter of a consanguineous marriage, had suffered from severe ligneous conjunctivitis and had died from decompensated congenital hydrocephalus despite numerous shunt revisions. She was found to be homozygous for a non-sense mutation in exon 15 of the plasminogen gene (Trp597->Stop). In her next pregnancy, the mother asked for prenatal diagnosis of the plasminogen deficiency. Chorionic villus biopsy was performed at 12 weeks of gestation. dna analysis of the plasminogen gene by PCR and single-strand conformation polymorphism (SSCP) revealed that the fetus exhibited an identical heterozygous band pattern as observed in the healthy mother. Therefore, the fetus was heterozygous for the Trp597->Stop mutation in plasminogen exon 15. In addition, the fetus was found to be male by cytogenetic analysis and by multiplex PCR analysis using two polymorphic X-chromosomal markers (DXS424, HPRT). These findings excluded the possibility of contamination by maternal dna. It was concluded that the fetus was not at risk for ligneous conjunctivitis and its associated complications. After the birth of a healthy boy, plasminogen functional activity was shown to be 38 per cent. dna analysis confirmed prenatal molecular genetic results.- - - - - - - - - - ranking = 1keywords = mouth (Clic here for more details about this article) |
5/73. Congenital nasal hemangiopericytoma: intrauterine, intraoperative, and histologic findings.hemangiopericytoma is a rare tumor of mesenchymal origin. To date, 91 cases of nasal or paranasal hemangiopericytoma and 59 congenital hemangiopericytomas have been reported in the literature. A congenital hemangiopericytoma arising from the nasal cavity and skull base has not yet been described. We report a case of a male newborn with a highly vascular nasal tumor diagnosed by in utero sonography with three-dimensional surface reconstruction. The tumor extended to the right anterior skull base, the right nasal cavity, and the right side of the nasal pyramid. A complete resection by neodymium:yttrium-aluminum-garnet-potassium titanyl phosphate ("Nd:YAG-KTP") laser was performed on the day of cesarean section at 33 weeks' gestation. The tumor was diagnosed as hemangiopericytoma by histologic and immunohistochemical findings. Postoperative nasal flow, feeding, and sight were unimpaired. At the 9-month follow-up, the infant remained free of disease.- - - - - - - - - - ranking = 3.0735376548797keywords = cavity (Clic here for more details about this article) |
6/73. Laparoscopic diagnosis and management of ovarian torsion in the newborn.BACKGROUND AND OBJECTIVES: The application of laparoscopic techniques in the surgical management of neonatal ovarian cysts is proving valuable both as a diagnostic tool and a potential therapeutic intervention. We report the successful management of a prenatally diagnosed ovarian cyst in a newborn female and provide operative evidence for the presumptive etiology of the cyst. methods AND RESULTS: A prenatally diagnosed ovarian cyst was managed using 5 mm laparoscopic instruments in a newborn female. The prenatal ultrasonographic and operative findings are consistent with in utero adnexal torsion with subsequent autoamputation and cystic degeneration of the ovary. The orphaned ovarian cyst was removed from the infant's abdominal cavity by enlarging the camera port incision. DISCUSSION: The application of laparoendoscopic procedures in infants and children continues to evolve with the availability, of microinstrumentation and increasing experience among pediatric surgeons. This approach may prove valuable in the diagnosis and management of prenatally diagnosed ovarian cysts. In addition, further insight into the etiology of congenital ovarian cysts may be obtained. The safety and efficacy of this approach in these infants remains to be fully evaluated.- - - - - - - - - - ranking = 1.5367688274398keywords = cavity (Clic here for more details about this article) |
7/73. Prenatal sonographic detection of nasopharyngeal teratoma.We present the case of a 34-year-old pregnant woman who had an elevated maternal serum alpha-fetoprotein level and sonographic findings of a semisolid mass protruding from the fetus's oral cavity. The large, heterogeneous mass filled the oropharynx and nasopharynx. Abnormal Doppler waveforms were detected in the umbilical artery of the fetus, who died in utero. Postmortem examination revealed a nasopharyngeal teratoma.- - - - - - - - - - ranking = 4.6690755594395keywords = oral cavity, cavity (Clic here for more details about this article) |
8/73. Isochromosome 1q as the sole chromosomal abnormality in two fetal teratomas. Possible trisomic or tetrasomic zygote rescue in fetal teratoma with an additional isochromosome 1q.An isochromosome of the long arm of chromosome 1 leading to tetrasomy 1q was detected as the sole chromosomal aberration in two cases of fetal teratoma arising from the oral cavity. This type of teratoma is extremely rare and has seldom been investigated cytogenetically. Studies of dna markers in the tumor, normal fetal skin, and parental cells demonstrated that in both cases the additional 1q material was of maternal origin. In one of the patients, the teratoma had maternal 1q marker alleles that were not found in the fetal body cells. This implies that the tumor was not derived in a direct way from the fetal body tissue; instead, the chromosomally-normal fetus might be the result of some trisomic or tetrasomic zygote rescue mechanism.- - - - - - - - - - ranking = 4.6690755594395keywords = oral cavity, cavity (Clic here for more details about this article) |
9/73. Ultrasonographic detection of intrauterine intussusception resulting in ileal atresia complicated by meconium peritonitis.A neonate with ileal atresia (IA) complicated by meconium peritonitis (MP) whose prenatal ultrasonography (US) detected an intrauterine intussusception (IUI) is reported. Fetal ascites, dilated bowel loops, and abdominal calcifications were identified on serial US from 25 weeks of gestation. Intestinal loops with high echogenecity and a "target-like" appearance suggestive of IUI were detected in the right lower quadrant. The 2,680-g male was delivered vaginally at term and underwent a laparotomy. Fibrous adhesions and small calcifications were scattered throughout the peritoneal cavity. IA (interrupted type) was confirmed 17.0 cm cranial to the ileocecal valve (ICV). An ileo-ileal intussusception was also found between 16.5 cm and 9.0 cm cranial to the ICV. Partial resection of the ileum and an ileo-ileal anastomosis was performed. The postoperative course was uneventful. In this case, the pathological process of IUI resulting in IA and MP was demonstrated sonographically by identifying the "target-like" appearance in the fetus.- - - - - - - - - - ranking = 1.5367688274398keywords = cavity (Clic here for more details about this article) |
10/73. prenatal diagnosis of epignathus in the first half of pregnancy: a case report and review of the literature.Congenital teratomas of the oral cavity commonly present as tissue masses projecting from the mouth. The important prognostic determinants are: the age of presentation, technical problems during surgical removal of the mass, associated anomalies, and the nature of the composing tissues. This study reports one case of epignathus (an extremely rare oropharyngeal teratoma) that was diagnosed by ultrasonography at 19 weeks of gestation and reviews the relevant literature.- - - - - - - - - - ranking = 5.6690755594395keywords = oral cavity, cavity, mouth (Clic here for more details about this article) |
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