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Jarcho-Levin Syndrome was first defined in 1938 by Saul Jarcho and Paul Levin. In the medical literature Jarcho-Levin Syndrome has a variety of synonyms such as Spondylocostal dysplasia/Dysostosis, Spondylocostal Dysostosis (SCD), Spondylothoracic dysplasia/Dysostosis and costovertebral dysplasia. For years the SCD and a similar disorder, spondylothoracic dysplasia, were considered the same disorder and referred as Jarcho-Levin Syndrome. Today we know that these two disorders are different clinical entities with different causes and that the term Jarcho-Levin Syndrome should be reserved for individuals with Spondylocostal dysplasia. Affected individuals with SCD have various abnormalities in the development of the spine and ribs. Due to these abnormalities they are more prone to develop thoracic insufficiency syndrome which may eventually lead to early neonatal death. In the current case report we describe two consecutive cases with SCD in the same family. In the case of a strong clinical suspicion from the findings of the ultrasound scan we should proceed to a molecular genetic diagnosis of a mutation in DLL3, MESP2, LFNG and HES7 gene by sequencing the entire coding area of the fetal Deoxyribonucleic acid (DNA).

Objectives: The aim of the study was to investigate the usefulness of fetal pulse oximetry in cases of severe variable decelerations in the second stage of labor. Methods: It is a prospective study including 58 patients. Thirty-eight patients (group A) had a normal uncomplicated labor and 20 patients (group B) developed severe variable decelerations during the second stage of labor. All patients were primiparous with normal pregnancies and had electronic fetal monitoring of labor in conjunction with fetal pulse oximetry. An estimation of fetal pH and base deficit was performed at delivery in all patients. Results: There was no statistically significant difference in relation to maternal age and gestational age between the two groups. Group A patients did not delivered neonates with metabolic acidosis. Six out of 20 (group B) patients delivered neonates with a pH <7.10 despite a fetal pulse oximetry reading of >30%. Conclusions: It appears that fetal pulse oximetry is not capable of detecting pre-acidotic or acidotic fetuses during the second stage of labor in patients with severe variable decelerations and the management of such patients should be supported by fetal scalp pH when indicated or otherwise the obstetrician should expedite delivery either with assisted operative delivery or cesarean section. Fetal heart rate monitoring was introduced into clinical practice over 30 years ago. It continues to be the predominant method of intrapartum fetal surveillance despite worries about its accuracy and efficacy. Copyright © 2005 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]