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Biomedical subjects

Sherard A Tatum

Publications and source records attributed to Sherard A Tatum.

16 recordsLinked to original sources

Floating the uvula: an intraoperative method for detecting bifidity.

A bifid uvula, midline diastasis of the palatal muscles, and notching of the posterior hard palate have classically formed a triad diagnosing submucosal clefts. The uvula has thus served as a tool for clinicians to detect the earliest signs of clefting. In this case report, we discuss how mucosal lining may be held together by mucous viscosity, making it difficult to detect notching or a grossly bifid uvula. We demonstrate a simple intraoperative technique to easily overcome this force. This paper involves a case report of an 8-year-old female undergoing an adenotonsillectomy. A previously undetected bifid uvula was found only after floating the uvula in normal saline solution. This changed our surgical approach from a complete to a partial adenoidectomy. A bifid uvula may be considered the earliest form of a cleft palate, and more importantly, it has been shown in the literature to be associated with other anomalies as submucosal cleft, hyoplastic eustachian tube orifice, and absence of the salpingopharyngeal folds. The intraoperative technique of floating the uvula helps to overcome mucous viscosity and identify an otherwise missed bifid uvula.

Adenoidectomy↗

Upper airway asymmetry in velo-cardio-facial syndrome.

OBJECTIVE: Various forms of asymmetry have been recognized as a feature of velo-cardio-facial syndrome (VCFS). This study was implemented to determine the frequency of anatomic and functional asymmetry of the velum, pharynx and larynx in children with VCFS. METHODS: Individuals with VCFS underwent prospective, blinded analysis by an expert panel who assessed the velum, pharynx and larynx with multi-view videofluoroscopy (MVF) and nasopharyngolaryngoscopy (NPL). The VCFS group was compared to an age-matched group of normal individuals. Eight different parameters were assessed in both groups for functional and anatomic symmetry including: velar elevation, adenoid size, posterior pharyngeal wall size, carotid pulsations, epiglottis size and shape, arytenoid size, true vocal cord size and true vocal cord motion. RESULTS: One hundred and twenty-one subjects with VCFS and 20 normal individuals underwent examination. Children with VCFS showed significantly more asymmetry compared to the normal group (69% versus 20%, P=0.01) with greatest differences seen with palatal motion, posterior pharyngeal wall size and epiglottis shape. On average, subjects with VCFS had three asymmetric parameters versus one parameter in the normal group. CONCLUSION: Asymmetric development of the pharynx and larynx in children with VCFS appears to be a distinct clinical feature of this syndrome. This finding may provide an important diagnostic clue for patients presenting with subtle features of the 22q11.2 microdeletion. These developmental abnormalities may increase the risk of speech impairment, aspiration and airway obstruction in affected individuals.

Adolescent↗

Cosmetic dentistry.

PURPOSE OF REVIEW: Patients seeking the expertise of facial plastic surgeons for facial aesthetic improvement may also desire or benefit from dental aesthetic procedures. This paper reviews current treatment options available in cosmetic dentistry. RECENT FINDINGS: Many techniques exist to improve dental aesthetics in color, position, shape, size, alignment and overall smile appearance. Although orthodontic therapy is still an important modality for smile aesthetics, some simpler procedures can provide acceptable aesthetic results. Comparison of external dental bleaching techniques reveals similar long-term results for in-office and at-home bleaching; in-office treatments, however, may provide the benefit of faster results. Internal dental bleaching is an effective method for correcting nonvital teeth coloration. Enamel shaping via either direct tooth contouring or the application of resins or veneers to tooth surfaces can correct defects, asymmetries and shape or rotation problems. Veneers or crowns are also options to correct intrinsic dental stains not amenable to bleaching techniques. Treatments to refine gingival margins and borders are another proven beneficial cosmetic procedure. SUMMARY: A myriad of techniques exist to correct a patient's particular concerns. Correction of discoloration is usually feasible as is the improvement of a patient's smile and overall dental aesthetics.

Crowns↗

Congenital unilateral multiple cranial neuropathy: an etiology shared with Mobius syndrome?

Mobius syndrome was originally described as bilateral 6th and 7th cranial nerve palsy and has since been expanded to include involvement of nearly all cranial nerves, limb deformities, orofacial anomalies and deficits in intellectual function. The etiology of Mobius syndrome remains elusive. Here we present a case with unilateral 5th, 7th, 9th, 10th and 12th cranial nerve palsy along with MRI evidence of ipsilateral brainstem hypoplasia. We propose the unilateral pathology supports the theory that vascular malformation in utero, which can be due to various etiologies, leads to brainstem ischemia and is the key factor in development of Mobius syndrome.

Brain Stem↗

Velo-cardio-facial syndrome: the pediatric otolaryngologist's perspective.

PURPOSE OF REVIEW: The understanding of velo-cardio-facial syndrome has grown markedly since the initial descriptions of this common genetic disorder nearly 30 years ago. Our knowledge of the syndrome has advanced in part from opportunities to monitor many patients into adulthood because of advances in the fields of cardiothoracic surgery and immunology. Longitudinal study has brought to light psychiatric and behavioral features of the syndrome that are often not apparent until late adolescence or the early adult years. Certain endocrine and immunologic features of the syndrome thought to be resolved in childhood are now witnessed in older patients. Variable expression and lack of disease awareness are two major factors that contribute to the delays in diagnosis in many cases. To address this, there has been a call to delineate screening parameters for patients at risk of carrying the deletion. RECENT FINDINGS: Several areas are highlighted in this review, reflecting the focus of scholarly work on velo-cardio-facial syndrome in the past year. Molecular genetics has shown smaller deletions in many families with the syndrome. The gene TBX1 has been found to be important to the phenotype. Surgical outcomes data reveal the greater challenges involved in correcting velopharyngeal insufficiency. SUMMARY: Defining the genetic basis of velo-cardio-facial syndrome will allow clinicians and basic scientists to make further inroads into understanding the variable expressivity of this syndrome. It is also important to be aware of the continued diagnostic challenges encountered by clinicians in attempts to improve the detection of patients with this syndrome.

Autoimmune Diseases↗

Craniometaphyseal dysplasia: a case report and review of medical and surgical management.

Craniometaphyseal dysplasia (CMD) is a genetic syndrome involving cranial and tubular bone anomalies that commonly present at a young age, often with otolaryngologic manifestations. In this paper, we report a rare case of a sporadic form of the disease resulting in an early state of hypocalcemia with secondary hyperparathyroidism. A conductive hearing loss is also documented prior to 12 months of age. The clinical aspects of CMD will be covered along with its pathogenesis. The current concepts surrounding medical and surgical treatments will be reviewed, and the management of our patient will be discussed.

Camurati-Engelmann Syndrome↗

Craniometaphyseal dysplasia: a case report and review of medical and surgical management.

Craniometaphyseal dysplasia (CMD) is a genetic syndrome involving cranial and tubular bone anomalies that commonly present at a young age, often with otolaryngologic manifestations. In this paper, we report a rare case of a sporadic form of the disease resulting in an early state of hypocalcemia with secondary hyperparathyroidism. A conductive hearing loss is also documented prior to 12 months of age. The clinical aspects of CMD will be covered along with its pathogenesis. The current concepts surrounding medical and surgical treatments will be reviewed, and the management of our patient will be discussed.

Camurati-Engelmann Syndrome↗

Update on craniofacial surgery.

Over the course of the past year, many interesting aspects of the multidisciplinary approach to the treatment of craniofacial disorders have been furthered. Several authors have shared their experiences, and new innovations have been described. Traditional ideas with regard to bone healing and growth have been expanded upon, and the role of alloplasts also is better understood now. Diagnosis and treatment planning have come a long way with the advent of new imaging techniques and applications. The ongoing discussion regarding absorbable fixation devices has continued, and the development of new technology has been presented. Some new techniques have also been brought to the forefront. Of note, distraction osteogenesis has been embraced as a viable alternative to traditional maxillomandibular surgery with bone grafting as well as other morbid procedures.

Bone Development↗

Pierre Robin sequence in a patient with ectrodactyly-ectodermal dysplasia-clefting syndrome: a case report and review of the literature.

Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is a rare condition first identified in 1970. The majority of cases are reported to be autosomal dominant with variable expression and penetrance. The most common clinical feature is ectodermal dysplasia. Ectrodactyly, cleft lip with or with out cleft palate and urogenital anomalies are also main clinical features of EEC syndrome. Pierre Robin (PR) sequence has the clinical findings of micro-retrognathia, retroposed tongue (glossoptosis), cleft of the secondary palate, and upper airway obstruction. Etiologically PR sequence is heterogeneous. It has been reported that less than 20% of cases are isolated, non-syndromic PR sequence. We present a case of a newborn male who was referred to our craniofacial clinic with EEC syndrome and PR sequence. This case represents a unique clinical association. We found no other reports in the literature of these two clinical entities occurring together. Upper airway obstruction of PR sequence, secondary to glossoptosis and micro-retrognathia, gives this association clinical relevance. In addition, this clinical association may represent a new gene locus associated with EEC syndrome.

Abnormalities, Multiple↗

Pharyngeal flap and the internal carotid in velocardiofacial syndrome.

Internal carotid artery anomalies have been documented as a common clinical feature in velocardiofacial syndrome. There has been some controversy over the need for preoperative imaging procedures, such as magnetic resonance angiography, when planning pharyngeal surgery for correcting velopharyngeal insufficiency. The purpose of this article is to describe 20 patients with velocardiofacial syndrome who received comprehensive evaluation and underwent pharyngeal flap surgery within a 2-year period and to report the technique used for dissecting the flap and the surgical outcomes. Anomalies of the major neck vessels were present in all cases, but 5 of these 20 cases had particularly severe anomalies of the internal carotid arteries that placed the vessels directly deep within the donor site for the pharyngeal flap. Surgery was carried out successfully in all 20 cases using a modified approach after radiographic imaging was performed to locate the arteries. In the 5 cases with severe malpositioning of the internal carotid arteries, it was clear that the vessels could have been injured had their location not been identified and the surgical approach modified to avoid them.

Adolescent↗

The coronal incision: sinusoidal, sawtooth, and postauricular techniques.

The coronal incision is a popular and versatile surgical approach for access to the cranial vault and the upper two thirds of the facial skeleton. It provides excellent exposure to allow neurosurgical access, craniofacial osteotomies, repair of facial fractures, calvarial bone grafting, and cosmetic procedures such as the forehead lift. Since the introduction and acceptance of the coronal approach, a variety of modifications of the incision have been used, including methods for camouflaging the incision in the hair. To quickly and easily produce a sinusoidal or sawtooth coronal incision line with or without postauricular extension, a tape measure is positioned at the level of the anteriormost point of the auricular helix. A mark is made every 2 cm on alternating sides of the tape measure. The tape measure is advanced coronally until the vertex of the skull is reached, and the procedure is repeated on the contralateral side. After the tape measure is removed, the resulting regularly spaced marks are connected in a sinusoidal or a sawtooth fashion. A postauricular incision can be easily extended from either the sinusoidal or sawtooth coronal template.

Facial Bones↗

Carbonated apatite and hydroxyapatite in craniofacial reconstruction.

Alloplastic biomaterials have revolutionalized craniofacial reconstruction. Commercially available bone substitute cements allow easy contouring and application, as well as avoidance of postoperative donor site complications. Hydroxyapatite and carbonated apatite are commercially available biomaterials currently being used as moldable bone substitutes in craniofacial reconstruction. This study presents 35 patients with 46 sites of reconstruction using hydroxyapatite or carbonated apatite. The characteristics, effectiveness, advantages, and limitations of each biomaterial are discussed.

Adolescent↗

Cerebro-costo-mandibular syndrome presenting as Pierre Robin sequence.

Cerebro-costo-mandibular syndrome is a rare disorder characterized by psychomotor retardation, posterior rib-gap defects, and the orofacial defects of Pierre Robin sequence. Most cases are sporadic, but several familial cases have been reported, many of which support autosomal recessive inheritance. We present a case of autosomal dominant inheritance from father to son; the seventh known case of dominant transmission. We also review the findings, inheritance pattern, and outcomes of Pierre Robin sequence that are useful in managing affected patients.

Abnormalities, Multiple↗