Application of Computer-Assisted Preoperative Planning in Treating Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion: A Case Report and Literature Review.

Congenital fusion of the jaws (syngnathia) is a rare facial malformation with an unknown etiology. This disease may vary in severity with adhesion of soft tissue and bony fusion. It can be anterior fusion, unilateral or bilateral fusion, and complete fusion. The main problem of these patients is the difficulty of airway maintenance and feeding, and the most common postoperative complication is the relapse of bony fusion. Here, we report a young male patient with bony syngnathia, involving bilateral fusion of the ascending ramus and body of the mandible with the maxillary complex. We performed bone isolation by computer-assisted preoperative planning and used an insertional temporalis flap to fix the wound surface to prevent refusion of bone.

Gynoecium structure in Sapindales and a case study of Trichilia pallens (Meliaceae).

Sapindales is a monophyletic order within the malvid clade of rosids. It represents an interesting group to address questions on floral structure and evolution due to a wide variation in reproductive traits. This review covers a detailed overview of gynoecium features, as well as a new structural study based on Trichilia pallens (Meliaceae), to provide characters to support systematic relationships and to recognize patterns of variations in gynoecium features in Sapindales. Several unique and shared characteristics are identified. Anacrostylous and basistylous carpels may have evolved multiple times in Sapindales, while ventrally bulging carpels are found in pseudomonomerous Anacardiaceae. Different from previous studies, similar gynoecium features, including degree of syncarpy, ontogenetic patterns, and PTTT structure, favors a closer phylogenetic proximity between Rutaceae and Simaroubaceae, or Rutaceae and Meliaceae. An apomorphic tendency for the order is that the floral apex is integrated in the syncarpous or apocarpous gynoecium, but with different length and shape among families. Nitrariaceae shares similar stigmatic features and PTTT structure with many Sapindaceae. As the current position of both families in Sapindales is uncertain, floral features should be investigated more extensively in future studies. Two different types of gynophore were identified in the order: either derived from intercalary growth below the gynoecium as a floral internode, or by extension of the base of the ovary locules as part of the gynoecium. Sapindales share a combination of gynoecial characters but variation is mostly caused by different degrees of development of the synascidiate part relative to the symplicate part of carpels, or the latter part is absent. Postgenital fusion of the upper part of the styles leads to a common stigma, while stylar lobes may be separate. Due to a wide variation in these features, a new terminology regarding fusion is proposed to describe the gynoecium of the order.

Development and evolution of extreme synorganization in angiosperm flowers and diversity: a comparison of Apocynaceae and Orchidaceae.

BACKGROUND AND AIMS: Apocynaceae and Orchidaceae are two angiosperm families with extreme flower synorganization. They are unrelated, the former in eudicots, the latter in monocots, but they converge in the formation of pollinia and pollinaria, which do not occur in any other angiosperm family, and for which extreme synorganization of floral organs is a precondition. In each family extensive studies on flower development and evolution have been performed; however, newer comparative studies focusing on flower synorganization and involving both families together are lacking. SCOPE: For this study an extensive search through the morphological literature has been conducted. Based on this and my own studies on flowers in various Apocynaceae and Orchidaceae and complex flowers in other angiosperms with scanning electron microscopy and with microtome section series, a review on convergent floral traits in flower development and architecture in the two families is presented. KEY FINDINGS: There is a tendency of protracted development of synorganized parts in Apocynaceae and Orchidaceae (development of synorganization of two or more organs begins earlier the more accentuated it is at anthesis). Synorganization (or complexity) also paves the way for novel structures. One of the most conspicuous such novel structures in Apocynaceae is the corona, which is not the product of synorganization of existing organs; however, it is probably enhanced by synorganization of other, existing, floral parts. In contrast to synorganized parts, the corona appears developmentally late. CONCLUSIONS: Synorganization of floral organs may lead to a large number of convergences in clades that are only very distantly related. The convergences that have been highlighted in this comparative study should be developmentally investigated directly in parallel in future studies.

Congenital fusion of jaw and ankyloblepharon filiforme adnatum: malformation and multiple systems anomaly.

Congenital fusion of jaw and its association with ankyloblepharon filiforme adnatum is reported but is a quite rare congenital benign anomaly. It may be unilateral or bilateral and can present with a single system or multiple systems involvement. This report concentrates on describing the clinical features of above disease, likely aetiological causes, and embryogenesis with classification, diagnostic, and, treatment modality, anesthesia problems and review of literature.

Refined Interpretation of the Pistillate Flower in Ceratophyllum Sheds Fresh Light on Gynoecium Evolution in Angiosperms.

Molecular phylogenetic analyses have revealed a superclade of mesangiosperms with five extant lineages: monocots, eudicots, magnoliids, Ceratophyllum and Chloranthaceae. Both Ceratophyllum and Chloranthaceae are ancient lineages with a long fossil record; their precise placement within mesangiosperms is uncertain. Morphological studies have suggested that they form a clade together with some Cretaceous fossils, including Canrightia, Montsechia and Pseudoasterophyllites. Apart from Canrightia, members of this clade share unilocular gynoecia commonly interpreted as monomerous with ascidiate carpels. Alternatively, the gynoecium of Ceratophyllum has also been interpreted as syncarpous with a single fertile carpel (pseudomonomerous). We investigate patterns of morphological, anatomical and developmental variation in gynoecia of three Ceratophyllum species to explore the controversial interpretation of its gynoecium as either monomerous or pseudomonomerous. We use an angiosperm-wide morphological data set and contrasting tree topologies to estimate the ancestral gynoecium type in both Ceratophyllum and mesangiosperms. Gynoecia of all three Ceratophyllum species possess a small (sometimes vestigial) glandular appendage on the abaxial side and an occasionally bifurcating apex. The ovary is usually unilocular with two procambium strands, but sometimes bilocular and/or with three strands in C. demersum. None of the possible phylogenetic placements strongly suggest apocarpy in the stem lineage of Ceratophyllum. Rescoring Ceratophyllum as having two united carpels affects broader-scale reconstructions of the ancestral gynoecium in mesangiosperms. Our interpretation of the glandular appendage as a tepal or staminode homologue makes the Ceratophyllum ovary inferior, thus resembling (semi)inferior ovaries of most Chloranthaceae and potentially related fossils Canrightia and Zlatkocarpus. The entire structure of the flower of Ceratophyllum suggests strong reduction following a long and complex evolutionary history. The widely accepted notion that apocarpy is ancestral in mesangiosperms (and angiosperms) lacks robust support, regardless of which modes of carpel fusion are considered. Our study highlights the crucial importance of incorporating fossils into large-scale analyses to understand character evolution.

Congenital Fusion of Dens to T3 Vertebra in Klippel-Feil Syndrome.

BACKGROUND: Patients with Klippel-Feil syndrome may present with neurologic complaints such as neck pain, radiculopathy and gait instability. Here we describe surgical management of a patient with congenital fusion of the occipital-cervical region and also block circumferential fusion of dens to T3 with spinal cord compression. This report is the first of its kind with such extensive fusion. CASE DESCRIPTION: Our patient was a 56 year-old female, who presented with neck pain and tingling in all extremities. On exam, she had a short neck, prominent jaw with extremely limited range of motion in neck and features of myelopathy. CT showed fusion of the dens to T3 vertebrae. Patient underwent sub-occipital craniectomy, C1 laminectomy and Occiput to T5 posterior fixation and fusion with neurologic improvement. CONCLUSION: This is the first reported case of Klippel-Feil syndrome with fusion of all cervical vertebrae down to T3. We recommend surgery for advanced cases of myelopathy or radiculopathy due to stenosis and spinal instability.

Surgical Treatment of Occipitocervical Dislocation with Atlas Assimilation and Klippel-Feil Syndrome Using Occipitalized C1 Lateral Mass and C2 Fixation and Reduction Technique.

OBJECTIVE: To introduce and assess a surgical treatment of occipitocervical (OC) dislocation with atlas assimilation and Klippel-Feil syndrome (KFS) using occipitalized C1 lateral mass and C2 fixation and reduction technique. METHODS: From January 2007 to August 2013, 58 symptomatic patients with OC dislocation and KFS of C2-3 congenital fusion and atlas assimilation were surgically treated in our institution via this technique. After opening the C1-2 facet joints via a posterior approach, OC reduction was conducted by intraoperative manipulation and C1 lateral mass and C2 pedicle screw and rod fixation. The instrument position, fusion status, and clinical outcome were analyzed. RESULTS: The average follow-up was 36 months (range, 18-52 months). Radiologically, effective reduction was achieved in 56 patients (96.6%) and <50% reduction in 2 (3.4%) who had additional transoral decompression. Neurologic improvement and solid bone fusion were achieved in all patients. The clinical symptoms improved for all patients, with the averaged Japanese Orthopedic Association myelopathy scores increasing from 11.5 to 15.6 (P < 0.01). CONCLUSIONS: In patients with OC dislocation and KFS of C2-3 fusion and atlas assimilation, posterior manipulative reduction combined with occipitalized C1 lateral mass and C2 fixation provides a reliable and effective treatment.

Organ homologies in orchid flowers re-interpreted using the Musk Orchid as a model.

Background and Aims. The presence of novel structures in orchid flowers, including auricles, rostellum and bursicles on the gynostemium and a lobed labellum, has prompted long-standing homology disputes, fuelled by conflicting evidence from a wide range of sources. Re-assessment of this debate using an improved model is timely, following recent phylogenetic insights and on the cusp of a revolution in developmental genetics. Methods. We use new data from floral development and anatomy in the small-flowered terrestrial orchid Herminium monorchis as a model to explore organ homologies in orchid flowers within the context of a review of recent literature on developmental genetics. Key Results. The apex of the median carpel of Herminium is trilobed, and the bursicles develop from its lateral lobes, relatively late in flower ontogeny. The bursicles enclose the viscidia, which adhere to the tapetal remnants to form a caudicle linking the viscidium with the pollinium. The auricles are initiated earlier than the bursicles, but they also remain unvascularized. The deeply trilobed labellum possesses three vascular traces, in contrast with the lateral petals, each of which contains a single vascular trace. The two lateral labellum traces diverge from the traces supplying the two adjacent lateral sepals. Data from flower ontogeny and anatomy conflict with respect to organ homologies. Conclusions. Much progress has recently been made in understanding the exceptional differentiation shown by orchids among perianth segments, focusing on multiple copies of the DEF/AP3 subclass of B-class MADS-box genes. In contrast, untangling homologies of profound congenital union of multiple floral organs forming the orchid gynostemium is hampered by their profound congenital union, which we ascribe to overlap in gene expression between organs. Thus, the functional morphology of the orchid flower could ultimately reflect extreme synorganization and associated genetic integration. Analogizing the deeply lobed orchid labellum with a compound leaf, we speculate that KNOX genes could be implicated not only in their demonstrated role in spur development but also in the development of both the characteristic lobed morphology of the orchid labellum and the lobing of the median carpel that differentiates the bursicles and rostellum.

Síndrome de Klippel-Feil: una enfermedad musculoesquelética, con malformaciones cardiovasculares asociadas / Klippel-feil syndrome, a skeletal muscle disease associated to cardiovascular anomalies

Introducción. El síndrome de Klippel-Feil (SKF) es un padecimiento de herencia autosómica dominante con penetrancia reducida y expresión variable, en el cual la tríada clínica inicial es de cuello corto, disminución en la movilidad del cuello e implantación baja del cabello posterior. Se asocia a otras malformaciones congénitas, entre ellas a cardiopatía (4-14%). El diagnóstico de la cardiopatía se debe realizar por el conocimiento de la asociación con este síndrome y con el apoyo del ecocardiograma. Material y métodos. Estudio retrospectivo, lineal, descriptivo y observacional de los niños con SKF atendidos durante los últimos 22 años en el Instituto Nacional de Pediatría, SS. Se analizaron las anomalías cardiovasculares, género, edad, tipo de herencia, datos clínicos y evolución. El diagnóstico clínico se realizó por el ortopedista y el cardiológico apoyado por ecocardiograma. Resultados. De los 46 pacientes, 24 correspondieron al género femenino y 22 al masculino. Las edades oscilaron entre un mes y 14 años.Todos los casos fueron de presentación esporádica. Las características clínicas más frecuentes fueron musculoesqueléticas, renales y cardiacas. Fueron valorados por el servicio de cardiología 19 pacientes, de los cuales 7 presentaron malformaciones cardiovasculares (7/46). Las cardiopatías que se presentaron fueron: comunicación interauricular (CIA), estenosis pulmonar (EP), comunicación interventricular (CIV) y dextrocardia, una paciente presentó además hipertensión arterial pulmonar primaria. Conclusiones. La proporción de cardiopatías en el SKF en nuestro hospital fue de 0.5. Las cardiopatías más frecuentes fueron la CIA, CIV y la EP. Hasta el momento ninguno de los pacientes ha fallecido.

Introduction. Klippel-Feil syndrome is a hereditary autosomal dominant disease with reduced penetrance and variable expression. It is characterized by multiple malformations but the 3 more constant are short neck, diminished mobility of the same and low implantation of posterior hair, associated to other congenital malformations as cardiopathy (4-14%).The diagnosis of cardiopathy must be done, first by knowledge of the association with this syndrome and after by clinical suspicion plus echocardiogram. Material and methods. In a study of 22 years in the National Institute of Pediatrics, we found 46 cases with Klippel-Feil syndrome of which 7 had cardiovascular anomalies.These were diagnosed with echocardiogram. Results. Of the 46 cases, 19 were evaluated by cardiologists; of these 7 (15%) had cardio-pulmonary malformations. Other malformations included: interauricular septal defect (ASD), interventricular septal defect (VSD), pulmonary stenosis (EP) and dextrocardia and one patient presented primary pulmonary hypertension.As of this communication, all patients are living. Conclusion. In this series, the proportion of cardiovascular anomalies was 15%. We conclude that all patients with Klippel-Feil syndrome should have a thorough cardiological evaluation including an echocardiogram.