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Results 201 - 250 of 471
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Yazici M - - 2001
Clinical and histopathological similarities and rare association of Blount's disease with various proximal femoral physeal affections (i.e. adolescent coxa vara and slipped capital femoral epiphysis) are well known. Association of tibia vara with another epiphyseal disease of the proximal femur has not been reported previously. In this paper, a monozygotic ...
Raybaud C - - 2001
The bipolar defects observed in schizencephalies-clefts in the hemispheric mantle on the one hand, absent septum pellucidum on the other--without any anatomic or functional continuity suggest that there is some sort of common specific vulnerability of both structures. A study of the correlation between lobar location of the clefts and ...
Alkan A - - 2001
Two cases of double lip malformation, an uncommon oral anomaly, are presented, and the factors involved in the development, pathogenesis, diagnosis, and treatment of these uncommon lesions are reviewed. Double lip is usually associated with Ascher's syndrome, which is a rare disease with three more or less consistently associated abnormalities: ...
Dalben G S - - 2001
Cleft lip and palate are relatively common congenital malformations, which may require specialist paedodontic treatment. In this article, the case of a 9-year-old boy with bilateral complete cleft lip and palate is presented. He attended the Hospital for Rehabilitation of Craniofacial Anomalies (HRAC) for routine examination, during which the presence ...
Hong R - - 2001
The DiGeorge anomaly, originally considered a clinical paradigm for isolated thymus deficiency, has now been redefined as a member of a group of disorders that share in common a chromosome deletion resulting in monosomy 22q11 (CATCH-22 or DGA/VCFS). In addition to the thymus defect, conotruncal heart anomalies, dysmorphism, hypoparathyroidism, and ...
de Die-Smulders C E - - 2001
A female new-born with bilateral lateral facial clefts, resulting in macrostomia, is reported. In addition she had a diminished palpebral fissure length. She died suddenly at the age of 6 months, presumably due to respiratory insufficiency. The unique combination of facial anomalies, present in this patient, has not been reported ...
Happle R R Department of Dermatology, University Hospital Marburg, Germany. - - 2001
Co-occurrence of aplasia cutis congenita and nevus sebaceus has sometimes been observed. We propose a genetic mechanism that may explain this coexistence of two different congenital skin disorders. We review 15 cases as reported in the literature and present an additional case. To explain the temporal and spatial proximity of ...
Woods S P - - 2000
This paper discusses the implications of Periventricular Leukomalacia (PVL) lesions for the development of Nonverbal Learning Disabilities (NLD) as illustrated through an identical twin case study. PVL lesions were identified in an 8-year-old child, but were not detected in his identical twin brother who served as a matched comparison. While ...
Cousley R R - - 2000
This study assessed the validity of the Yorkshire regional orofacial cleft database by comparing the computer-based records with locally collated records of primary surgical events for babies born over a 2-year period (1994-1995). One-hundred-and-thirty-two infants with clefts (excluding submucous cleft palate) were identified from the latter source with an equal ...
Bilkay U - - 2000
A case of facial cleft that fits the anatomic description of the rare bilateral Tessier no. 5 cleft accompanied by unilateral no. 1 and no. 6 clefts is presented, and brings the total number of reported cases to 21. This case and a review of the literature help to define ...
Sen S - - 2000
We report a rare case of upper sternal cleft in a 20-year-old adult with associated unusual symptoms such as dysphagia, restricted lingual movements and a voice problem. The strap muscles causing unusual symptoms were divided from their abnormal insertion site on the lower edge of the cleft. Direct complete closure ...
Ozcan C - - 2000
Parasitic or heterotopic conjoined twins are exceedingly rare, and these cases are referred to as "heteropagus" when there is a parasitic attachment in a nonduplicated fashion to any portion of the body. Epigastric heteropagus twinning refers to the attachment of the parasite to the epigastric region of the autosite. An ...
Juretschke L J - - 2000
Parasitic twins are a rare phenomenon and a challenge for caregivers. Occurring as a result of abnormal processes in the first several weeks of embryologic development, this condition is oftentimes diagnosed by prenatal ultrasound. This unusual condition may go undiagnosed until the time of delivery, however, despite the availability and ...
Jabor M A - - 2000
We report a case of a child born with bilateral complete cleft lip and palate, absence of the left lower leg, complete absence of the right arm, and absence of the second and third distal phalanges of the right foot. The child had no other associated abnormalities. Prenatal ultrasonography recognized ...
Aksoy F - - 2000
We describe a case of concordant body stalk anomaly in a monozygotic twin. Autopsy of the fetus showed abnormalities compatible with the maldevelopment of embryonic folding. Abdominal viscera were in a sac covered by the amnion and were attached directly to the placenta. The anus was not visible and no ...
Erkaya S - - 2000
A case of acardiac twinning where the pump twin dies in utero due to massive thrombotic occlusion of umbilical arteries at a site of umbilical cord stricture of the intact twin. In acardiac twinning the morbidity and mortality of the pump twin can be reduced by some invasive or medical ...
Samuels A H - - 2000
OBJECTIVE: To highlight issues of adoption and twinship in an unusual case of stalking. CLINICAL PICTURE: An ususual case of same gender, serial stalking which began at an early age in a young woman who was one of a set of twins adopted at birth. TREATMENT: Insight orientated psychotherapy. OUTCOME: ...
Suri M - - 2000
We report a female fetus with occipital encephalocele, dysraphism of the cervical spine, right renal agenesis and Mullerian agenesis. Additional findings included posterior cleft palate, absent left umbilical artery and Meckel's diverticulum. This fetus had the features of MURCS association with occipital encephalocele. This is the second report of encephalocele ...
Carter S R - - 2000
PURPOSE: A clinical observation showed that involutional entropion of the lower eyelid in Asians may occur more commonly than ectropion. A review of surgical cases was performed to examine this hypothesis. METHODS: A retrospective review of the number of Asian lower lid involutional ectropion and entropion repairs was performed in ...
Hatoko M - - 2000
We report corrected cases of posttraumatic nasal deformity using a hard palate mucosal graft as covering material of the inner-side defect. In all cases, the deformity was corrected satisfactorily. The nasal cavity was preserved, and because of the stiffness of the grafted mucosa, it did not collapse in the course ...
Hill C - - 1999
The 'unrepairable' cleft palate may be described as one in which the width of the cleft exceeds the sum of the widths of the palatal shelves. A report of such a case is presented, and the technique used to repair the cleft using bilateral buccinator musculomucosal flaps is described. It ...
Nandlal B - - 1999
Nasality is related to factors like velopharyngeal closure and acoustic factors pertaining to cavities. The present investigation is a retrospective study aimed at evaluating the effects of variation in the timing of palatal repair on nasality during speech development in complete cleft lip and palate cases. It has been observed ...
Libermanis O - - 1999
This paper reports the authors' experience with the use of microvascular flaps, elevated from severely injured fingers to cover soft-tissue defects of other digits. Two clinical cases are presented and pertinent literature is reviewed.
Karnak I - - 1999
Incomplete conjoined twinning or heteropagus attached at the autosite's epigastrium is an extremely rare form of conjoined twinnings. We report a case of epigastric parasitic twinning in which the parasite has a well developed lower trunk and pelvis with rudimentary lower limbs, and well developed upper extremities without shoulder girdles ...
Levy G - - 1999
Early-onset Huntington's disease (HD) occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, minimental state examination, blood laboratory exams, cerebrospinal fluid examination, skull computed tomography, and genetic examination for HD. Patients had the akinetic-rigid variety (Westphal variant) ...
Pijnenborg J M - - 1999
The monoamniotic twin is at risk for high perinatal mortality because of cord accidents. There is no consensus in literature for the obstetric management in these cases. Contradictory results are reported according to fatal complications by cord accidents after 32 weeks gestation and safety of vaginal delivery. Recently two patients ...
Hierl T - - 1999
OBJECTIVE: This report introduces the possibilities of callus distraction in the extremely atrophied, edentulous midface in a cleft lip and palate patient. INTERVENTION: After a subtotal Le Fort II osteotomy, tension wires were fixed to the zygomatic buttresses and frontal sinus walls by way of titanium miniplates and mesh and ...
Kirk E P - - 1999
We report a family in which four members of three generations are affected by median cleft palate. The proband and her mother have additional features including bilateral single transverse palmar creases, broad great toes and hypoplastic fifth toenails. Dominant isolated cleft palate has rarely been reported, and there are no ...
Nurko C - - 1999
Self mutilation of lips and tongue is considered a common type of Self-Injurious Behavior (SIB). Treatment of SIB in the form of Lip-Biting in developmentally disabled individuals has been the focus of several related reports using different oral appliances preventing or inhibiting the SIB. In this paper we report a ...
Seidman M D - - 1999
We describe 6 new cases of palatal myoclonus (PM), a rare disorder that is characterized by involuntary rhythmic contraction of the palatal musculature. Although it has been reported that PM is a lifelong condition, one of our patients experienced a complete resolution of PM, which casts doubt on the accepted ...
Breault L G - - 1999
In the oral cavity, masticatory mucosa covers the hard palate and gingiva. The epithelium of masticatory mucosa is relatively thick compared to other oral tissues. This results in firmness and immobility necessary to withstand the forces of mastication. In the anterior aspect of the hard palate, long papillae within the ...
Cooper T M - - 1999
Reported herein are 130 consecutive cases of free groin flap transfer performed by one surgeon over a 19-year period. Transplantation was performed for soft-tissue cover or augmentation of contour defects involving the head and neck (68 cases), trunk (4 cases), upper limb (14 cases), and lower limb (44 cases). Indications ...
Tsutsumi K - - 1999
BACKGROUND: Whereas the transcranial approach has been regarded as the therapy of choice for transethmoidal encephalocele, its feasibility for transsphenoidal encephalocele has remained controversial, particularly in neonates and infants. CASE REPORT: Two cases of transsphenoidal encephalocele operated transcranially are presented. In the first case, this 6-year-old boy underwent a transpalatal ...
Deprez M - - 1999
We describe 2 new cases of Alexander's disease, the first to be reported in Belgium. The first patient, a 4-year-old girl, presented with progressive megalencephaly, mental retardation, spastic tetraparesis, ataxia and epilepsy: post-mortem examination showed widespread myelin loss with Rosenthal fibers (RFs) accumulation throughout the neuraxis. She was the third ...
Daum R - - 1999
The authors discuss six cases of superior and total sternal clefts treated in their department in the last 34 years and discuss the problems of this congenital malformation. The inferior sternal clefts including Cantrell's pentalogy and the total ventral clefts were excluded because in these cases severe associated anomalies require ...
Natsume N - - 1999
Oblique facial clefts constitute approximately 0.20% of all facial malformation cases in Japan and approximately 0.22% in other countries. In the present study, the proportion in our institute was approximately 0.21%, which is almost equal to that reported by WILSON et al. The ratio of male and female patients did ...
Shah J - - 1999
Nasal gliomas are uncommon lesions, with approximately 100 cases reported in the literature. We present a case of intranasal glioma with a pedicle of glial tissue and defect in the cribriform plate of the ethmoid bone. Presence of such a pedicle is almost always present in the encephaloceles and is ...
Thomas S R - - 1999
Bilateral trigger thumbs in 4-year-old identical male twins are reported. To the authors' best knowledge, this is the first true description of this condition in identical twins. All four thumbs were treated by surgical release of the A1 pulley, with good results. The causes proposed for congenital and acquired trigger ...
Koltuksuz U - - 1998
In most omphalopagus twinnings, joined structures include the gastrointestinal tract, liver, biliary tree and bladder. In some instances of omphalopagus, joined attachments are limited to the intestine and bladder. Eight cases like these, that are also called minimally conjoined twinning, have been reported before. This article describes a set of ...
Filkins K A - - 1998
Studies published in the past year have attempted to model the pathophysiology of the twin-twin transfusion syndrome and also to establish the minimal diagnostic criteria for the condition. Many papers focused on treatment regimens and their sequelae. Aggressive treatment with serial amnioreduction continues to predominate, and appears to be more ...
Marques I L - - 1998
OBJECTIVE: To investigate the etiopathogenesis of isolated Robin sequence. DESIGN: A longitudinal and prospective study of children with isolated Robin sequence and no other associated syndromes or malformations. SETTING: The study was carried out at the Hospital de Reabilitação de Anomalias Craniofaciais (formerly the Hospital for Research and Rehabilitation of ...
Lees M M - - 1998
The association of optic disc abnormalities with basal encephaloceles, specifically of the sphenoethmoidal type, and midline facial clefts has rarely been reported, although the association of midline facial clefts with encephaloceles is well described. We now report six cases of children, three males and three females, presenting with a sphenoethmoidal ...
Arabin B - - 1998
OBJECTIVES: A combination of surgical and medical approaches may address both the underlying pathophysiological processes and the most threatening symptoms in twin-twin transfusion syndrome (TTTS). CASE: A primigravida with monochorionic twin pregnancy was referred with signs of TTTS at 16 weeks. One twin was severely hydropic with normal amniotic fluid, ...
Rapado F - - 1998
A case is described of an extensive acquired cholesteatoma of the middle ear cleft which had invaded the middle cranial fossa and produced a mass effect on the temporal lobe. It had also extended into the labyrinth without causing elevation in the bone conduction threshold. Furthermore, even after total bony ...
Antoniades D Z - - 1998
Exostoses, also known as hyperostoses, are localized bony protuberances that arise from the cortical plate. These developmental anomalies, or hamartomas, frequently affect the skeletal jaw. Different types of exostoses have been described. Torus palatinus and torus mandibularis are two of the most common intraoral exostoses. Other types of exostoses, affecting ...
Guion-Almeida M L - - 1998
We report on four Brazilian patients with, among other signs, cleft lip and palate, dental anomalies, ectropion of the lower eyelids, euryblepharon, and lagophthalmia. Two were sporadic cases and two were familial cases, a mother and her equally affected son. Recently, the reports with different combination of these signs were ...
Lipshutz G S - - 1998
Laryngotracheoesophageal cleft is a very rare congenital malformation characterized by a long midline communication between the esophagus and trachea extending from the larynx down the trachea for a variable distance. The most common repair strategy involves separation of the trachea and esophagus by a lateral approach via both a thoracotomy ...
Biswas J - - 1998
The occurrence of intermediate uveitis in monozygotic twins has rarely been reported in literature. We report the occurrence of this disease condition in identical twins. Twin sisters presented with typical features of intermediate uveitis, which appeared three years earlier in one of the sisters. They were treated with posterior subtenon ...
Sittel C - - 1998
We describe a 36-year-old patient with an aggressive, midline intranasal and naso- and oropharyngeal destructive process. For months the patient denied heavy abuse of nasal cocaine, but finally admitted it. Necrosis and atrophy of the inferior and middle nasal turbinates bilaterally, prominent naso and oropharyngeal ulcers, nasal septal as well ...
Doski J J - - 1997
The authors report the successful delivery, preoperative management, and postoperative courses of ischiopagus tripus twin girls successfully separated at 5 months of age. Surgical objectives were predicated on survival and optimum postseparation reconstructive potential for both girls. Each twin has subsequently undergone additional procedures, and both are doing well 2 ...
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