Absence Of Ribs Research Articles

Neues Therapiekonzept für Kinder mit Thoraxinsuffizienz-Syndrom aufgrund von Wirbelsäulenfehlbildungen

Children with congenital thoracic scoliosis associated with fused ribs and unilateral unsegmented bars adjacent to convex hemivertebrae will inevitably develop thoracic insufficiency syndrome and curve progression with hemithorax compression without treatment. It is assumed that the concave side of such curves and their unilateral unsegmented bars do not grow. In the past early spinal fusion was performed with consecutive short thoracic spines and loss of lung volume. Little attention has been paid to lung function. These patients often suffered from lung failure and early death due to a small thorax. A new surgical technique is based on an indirect deformity correction and enlargement of the thorax due to a longitudinal implant, the vertical expandable prosthetic titanium rib (VEPTR). The spine is not fused, thus promoting growth of the spine, the thorax and the lungs. Elongation of the implant is done every six months. Since 2002 this method has been performed on fifteen children in Basel as the first European center. Patients (mean age 6 years; 11 months to 12 years) were suffering from thoracic insufficiency syndrome due to unilateral unsegmented bars with fused ribs (n = 4), absent ribs (n = 2), bilaterally fused ribs (n = 2), hemivertebrae (n = 3) or neuromuscular scoliosis (n = 6). Doing fifteen primarily implantations and thirteen elongations there were three complications (two hook dislocations, one skin breakage). All patients improved cosmetically, functionally and radiologically which was shown on X-rays as a reduction of the Cobb angle from an average of 76 degrees (40-110 degrees ) to 55 degrees (30-67 degrees ). Expansion thoracoplasty and VEPTR implantation is a new treatment concept for children with thoracic insufficiency syndrome due to spinal deformities, which is based on distraction and expansion of the thorax thus allowing growth of the spine, the thorax and probably lungs. Presently it seems to be superior to any other method for the treatment of small children with progressive scoliosis and thoracic insufficiency syndrome.

Regionalization of axial skeleton in the lungfishNeoceratodus forsteri (Dipnoi)

Differentiation of the axial skeleton into distinct regions, once thought to be characteristic of the Tetrapoda, also occurs in the actinopterygian Danio rerio. In these taxa, the boundary between the cervical-thoracic regions correlates with Hoxc6 expression and morphological features such as position of the pectoral fin and associated nerves, and the absence of ribs. In the lungfish Neoceratodus, a member of the extant sister taxon to the Tetrapoda, the first vertebral element to chondrify is situated well posterior to the skull, developing from somites 6 and 7 (6/7) and associated with an enlarged cranial rib and nerves innervating the pectoral fin. Two vertebral elements develop later and more anteriorly, associated with somites 4/5 and 5/6. These three elements become incorporated into the occipital region of the skull during Neoceratodus ontogeny, until the cranial rib itself articulates to the rear of the skull. These features of early development indicate a regionalization of the Neoceratodus vertebral column: the cranial rib marks the boundary between the cervical and thoracic regions, the two more anterior vertebrae lacking ribs represent the cervical region, while somites 1-4 (cranial half), lacking any vertebral development, represent the occipital region. However, the cervical region of the vertebral column is effectively lost during ontogeny of Neoceratodus. A recognizable cervical region in the tetrapod vertebral column, as in zebrafish, suggests that cervical vertebrae are not incorporated into the skull but maintained as distinct elements of the column, representing an important shift in relative developmental timing and the influence of heterochrony in this region during the fish-tetrapod transition.

Lumbo-costo-vertebral syndrome with posterior spinal dysraphism

Lumbo-costo-vertebral syndrome, which includes abnormalities of the vertebral bodies, ribs and trunk musculature, is very rare and only few cases have been reported. We report a case of 18-month-old female child with absent ribs, hemivertebrae, superior lumbar hernia (features of lumbo-costo-vertebral syndrome) and posterior spinal dysraphism, which to our knowledge is the first case in the English literature with such a combination of defects. Embryology and management of the case is discussed.

Severe micrognathia, cleft palate, absent olfactory tract, and abnormal rib development: Cerebro-costo-mandibular syndrome or a new syndrome?

We report on a family in which two sibs had apparently absent ribs and severe micrognathia on prenatal ultrasonography. The pregnancies were terminated at 19 and 12 weeks of gestation, respectively. Autopsy findings in the first fetus (19 weeks of gestation) included severe micrognathia, a U-shaped defect of the soft palate, marked postnuchal edema, absent olfactory bulbs, and cribriform plate and rib abnormalities. The ribs consisted of cartilage anteriorly, with only a small amount of fibrous tissue present laterally and posteriorly. The second fetus (12 weeks gestation) had agnathia, with a large U-shaped defect in the soft palate. There was moderate postnuchal edema. The ribs were unossified and there were gaps in the cartilage where primitive mesenchyme was present posteriorly and laterally. These findings are consistent with a severe form of cerebro-costo-mandibular syndrome. The early fetal histopathology of both cases suggests a possible mechanism by which the characteristic "rib gaps" of cerebro-costo-mandibular syndrome may develop, with evidence for abnormal function of a gene or genes involved in regulation of rib chondrogenesis.

Anomalies of Cartilaginous and Ossified Axial Skeleton in Rat Fetuses Treated with Cyclophosphamide: Type, Frequency, and Stage Specificity

AbstractThe purpose of the present study was to assess the type, frequency and stage specificity of axial skeletal anomalies induced by cyclophosphamide (CP). Female Crj: Wistar rats were mated with male rats (vaginal sperm = gestation day 0, GDO). They were then injected intravenously with 5 mg/kg of CP in a saline solution on GD7, 8, 9, 10, 11, 12, 13, or 14, or with saline on GD8 for controls. Dams were sacrificed on GD20. Fetuses were stained with alcian blue and alizarin red S. Eighteen types of axial skeletal anomaly were detected, e. g., shifted ventral lamina, cervical rib, cleft arch of the atlas, axis fused to atlas, sternebral misalignment, bipartite sternum, fused ribs, change in pre‐sacral vertebral number, supernumerary rib, misdirected transverse process, anteriorly‐shifted spinous process, short spinous process, fused vertebral bodies, cartilaginous short rib at Th.7, short or absent rib at Th.12 or 13, dumbbell‐shaped vertebral body, wavy rib, and rib cartilage not attached to the sternal cartilage. The present experimental study indicates that: 1) observations of cartilaginous skeletons can provide more information than observations of ossified skeletons; 2) the pattern of CP‐induced anomalies along the anterior–posterior axis may depend on the sequence of development progression among structures, spinous process, vertebral arch, vertebral body, rib/transverse process; and 3) in more cranial vertebrae, anteriorly‐shifted anomalies, such as anteriorly‐shifted ventral lamina and anteriorly‐shifted spinous processes, rather than posteriorly‐shifted anomalies, may be the primary indicators for detecting developmental toxicity, since CP injection increased the frequencies of these anomalies.

New data about the evolution and phylogenetic relations of brachiopod families Basiliolidae and Cyclothyrididae on the basis of Late Cretaceous rhynchonellids from Bulgaria

On the basis of investigations of Bulgarian Late Cretaceous Rhynchonellida an attempt is made to outline some trends in the development and phylogenetic relations among them. The fam. Basiliolidae is represented by the genera Orbirhynchia and Parthirhynchia. It is suggested that Parthirhynchia derived from Orbirhynchia directly or by unknown till now link. Fam. Cyclothyrididae is represented by the genera Cyclothyris, Cretirhynchia and Septatoechia. The author maintains the view of Lobacheva and Smirnova that Lamellaerhynchia should be considered as synonym of Cyclothyris taking into consideration the established vast variability within the inner and outer shell morphology of Cyclothyris. Some evolutionary trends in the development of Cyclothyris are outlined. For Cretirhynchia it is suggested that the type of the ribs cannot have a high taxonomic value and cannot be used for subdividing the genus because transitions between "Cr. plicatilis" and "Cr. exculpta" series are found. But the presence or absence of ribs can be used as characteristic for subdividing. It is considered that Septatoechia derived from some of simple ribbed Cretirhynchia.

LEAF AND STEM ANATOMY OF SPECIES OF CYTISOPHYLLUM, CYTISUS, CHAMAECYTISUS, GENISTA, AND GENISTA SECT. TELINE (FABACEAE: GENISTEAE) AS AN AID FOR TAXONOMY

The leaf and stem anatomies of several shrubby species of theCytisus-Genistacomplex have been studied. The selected species have potential value for soil conservation and as feed for ruminants in the Mediterranean basin. The usefulness of anatomy for identification of such species and its value as an aid to clarify the taxonomy of the complex were examined. We conclude that leaf anatomy is of no taxonomic use at the generic and infrageneric level. On the other hand, stem anatomy presents systematic value. The presence or absence of ribs and the occurrence of vascular bundles within the ribs have proven useful to identify genera as well as to clarify the taxonomy of the complex at the generic level. The results support the generic arrangement proposed by Bisby (1981). Some species can be identified by the presence of peculiar stem anatomical traits.

Reflection of incident pressure waves by ribbed panels

The definitions of the reflection coefficients, in the absence of ribs and due to ribs, of an incident pressure wave on a plane at the surface of a ribbed fluid-loaded panel are defined, examined, and computed. The incident pressure wave is described by a plane wave and by a collimated beam. The results of representative computations are displayed. Through these displays, some aspects of the influence on the reflection coefficients caused by changes in the parameters that describe the ribbed panel, the fluid loading, and the incidence are investigated. These aspects include wave-number aliasings and symmetries in some of the terms and factors that compose the expressions for the reflection coefficients; the wave number of concern lies across the ribs. Aliasings are present, however, only when the separations between adjacent ribs are conditioned to be equal. Moreover, in some of these aliased terms and factors, symmetry is present only when the incidence obeys specific conditions. Disturbing these conditions tends to spoil the aliasing and/or the symmetries in these terms and factors. It is shown, however, that the aliasing and symmetry properties are invariant to changes in the parameters that describe the ribbed panel and/or the fluid loading to which it is subjected. The role that the phenomenon of pass and stop bands (bands in reference to the frequency domain) plays in the reflection properties of ribbed fluid-loaded panels is of particular interest here. It is illustrated that in the frequency ranges of pass bands, diffraction orders and aliasings tend to fade, and in the frequency ranges of stop bands they are enhanced. Fluid loading subdues the pass and stop bands; however, even substantial fluid loading does not eliminate these bands. Finally, the relationship between the reflection coefficients defined on the surface of the panel and on a control surface placed in the farfield is formulated and discussed.

Respirator dependency due to flail cheat from congenitally absent ribs: A method of repair

Respirator dependency due to flail cheat from congenitally absent ribs: A method of repair

Blunt thoracic trauma. Analysis of 515 patients.

A retrospective analysis of 515 cases of blunt chest trauma is presented. The overall thoracic morbidity rate was 36% and mortality rate was 15.5%. Atelectasis was the most common complication. Severe chest trauma can be present in the absence of rib or other thoracic bony fractures. Emergency thoracotomies for resuscitation of the patient with blunt chest trauma with absent vital signs proved unsuccessful in 39 of 39 patients. A high index of suspicion for blunt chest injury occurring in blunt trauma, coupled with an aggressive diagnostic and therapeutic approach, remains the cornerstone of treatment to minimize the morbidity and mortality of such injuries.

Pulmonary Actinomycosis

The diagnosis of thoracic actinomycosis is readily made when the patient presents with the constellation of poor oral hygiene, pulmonary infiltrates, empyema, sinus tracts, and osteomyelitis of the ribs. However, in the absence of rib and chest wall involvement, the disease may be overlooked. Three cases of primary pulmonary actinomycosis are presented showing three different pulmonary forms of the disease and comprising (a) subacute patchy pulmonary infiltrates, (b) a cavitary lesion simulating tuberculosis, and (c) a central mass lesion simulating bronchogenic carcinoma.

A radiological study of vertebral and rib malformations in children with myelomeningocele

Postmortem radiographs from 112 infants with thoracolumbosacral or lumbosacral myelomeningoceles were surveyed for associated vertebral and rib malformations. Sixty-four exhibited a variety of anomalies including hemivertebrae, fused vertebral bodies, fused vertebral arches, fused ribs, absence of ribs and absence of vertebral bodies. Anomalies were commonly associated with the cranial end of a dysraphic spina bifida region; in addition hemivertebrae and rib fusion exhibited peak incidences centred around the seventh cervical and fourth thoracic vertebra respectively. Fusion of vertebral arch elements increased in frequency throughout the thoracic region towards T10. The twelfth pair of ribs were absent in 20% of cases. Apparent absence of vertebral bodies from the caudal end of the vertebral column was seen in 24% of the radiographs.

Defects in the thoracic wall by absence of ribs or costal anomalies : M. Fèvre and D. Hannouche. Ann. Chir. Inf. 9:153–166, 1968

Defects in the thoracic wall by absence of ribs or costal anomalies : M. Fèvre and D. Hannouche. Ann. Chir. Inf. 9:153–166, 1968

III.—Contributions to Carboniferous Palæontology

In the July Number of the GeologicalMagazine, I described certain new species of bivalve Mollusca from the Carboniferous series of Scotland. These will now be found on Plate XII., accompanied by three other well-marked forms from the same formation, and which appear to me to be undescribed. In Messrs. Young and Armstrong's Catalogue of the Carboniferous Fossils of the West of Scotland is recorded a new species of Pteronites, under the specific name of P. fluctuosus (Etheridge, MS.), a shell so named by my father, but never yet described. P. fluctuosus is specifically remarkable for its small size, and the absence of ribs on the anterior side.

On the Batracholites, indicative of a small species of Frog ( Rana pusilla , Ow.). Addendum to the Communication from G. T. Clark, Esq., Bombay

The portions of shale transmitted by Mr. Clark contain delicate, but for the most part distinct, traces of the, generally, entire skeleton of small anourous Batrachia , the osseous substance is black, as if charred. The number of vertebræ, atlas and sacrum inclusive, is nine: the caudal vertebræ are fused into a long, slender cylindrical style, as in most anourous Batrachia . In the specimen (fig. 1) which lies on its back, the posterior convexity of the vertebral bodies is shown. The short, subcylindrical and very slightly expanded lateral or transverse processes of the sacrum, and the absence of ribs or their rudiments in the dorsal vertebræ, with the proportional expanse of the skull and length of the hind-legs, show the specimens to belong to the family of Frogs ( Ranidœ ). There are seven abdominal vertebræ, with long and subequal transverse processes, that of the second (third vertebra including the atlas) being the longest. The humerus is cylindrical, not expanded as in Cystignathus . The head is a little larger relatively than in Rana temporaria , R. esculenta , or Hyla viridis ; and still larger, therefore, than in the Toads and Natterjacks ( Bufonidœ ), or than in the Pipa : the expansion of the sacrum removes the genus Pipa and the Bombinatores from that of the present fossils. The following are admeasurements of the more perfect specimens:— All the specimens belong to individuals which had completed their metamorphosis, and they are similar to one another in size; they may have belonged either to a not quite full-grown brood