1/18. Salivary gland aplasia with cleft lip and palate: a case report and review of the literature.We report the case of a patient with lifelong symptoms of xerostomia and a repaired bilateral cleft lip and palate. The clinical evaluation demonstrated aplasia of the major salivary glands. A review of the literature pertaining to salivary gland aplasia is presented, along with a summary of the data regarding patient gender, defect sites, hereditary background, and combined manifestations. The diagnostic methods, possible pathogenesis, and management are also discussed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/18. Double tongue, intraoral anomalies, and cleft palate--case reports and a discussion of developmental pathology.OBJECTIVE: Isolated cleft palate is the most common presentation of the nonsyndromic cleft lip/palate combinations and is multifactorial in etiology. We report two cases of children with clefts of the secondary palate coexistent with double tongue and in either case mandibular epulis or superiorly displaced salivary gland. RESULTS AND DISCUSSION: In each case, the palatal cleft correlated anatomically with the intraoral space-occupying lesion. The ratio of tongue volume to intraoral volume during palatogenesis is discussed with reference to the pathogenesis of cleft palate. These clinical cases propose the model of a unifying sequence of developmental events whereby deformation of palatal shelf elevation results in secondary palatal clefting.- - - - - - - - - - ranking = 0.16666666666667keywords = gland (Clic here for more details about this article) |
3/18. Extreme fenestration of the basilar artery associated with cleft palate, nasopharyngeal mature teratoma, and hypophyseal duplication.The authors present the case of a newborn girl with extreme fenestration of the basilar artery. This anomaly was found incidentally during MR imaging study for cleft palate and nasopharyngeal teratoma. magnetic resonance angiography showed a totally duplicated basilar artery with connections at the proximal and distal ends of the artery, suggesting an extreme fenestration. Duplicated pituitary gland was also found on MR imaging.- - - - - - - - - - ranking = 0.16666666666667keywords = gland (Clic here for more details about this article) |
4/18. Increased tear evaporation in a patient with ectrodactyly-ectodermal dysplasia-clefting syndrome.PURPOSE: To describe the tear function and ocular surface disorders in a patient with ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome. methods: Routine ophthalmic examinations were performed, including slit-lamp biomicroscopy, anterior segment photography including transillumination photos of the lids, Schirmer tests I and II, tear film break-up time (BUT) assessment, corneal fluorescein staining, DR-1 tear film lipid layer interferometry, and tear evaporation rate measurements. RESULTS: Slit-lamp examination revealed conjunctival hyperemia, superficial punctate keratopathy, and corneal leucoma with neovascularization. Although the Schirmer test values were within normal limits, the BUT value was 0 s in both eyes. transillumination of the lids showed the absence of meibomian glandular structures. DR-1 tear film lipid layer interferometry results were dry eye grade 5 with an irregular tear film, areas of corneal surface exposure, and several dry spots. The tear evaporation rate was elevated and was measured as 6.98 x 10(-7) g/cm2 per second (normal, 4.1 /- 1.4 x 10(-7) g/cm2 per second). CONCLUSION: The ocular surface disorder and shortened BUT in EEC syndrome were attributed to the absence of meibomian glands, leading to lipid layer deficiency in the tear film with a concomitant increase in tear evaporation.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
5/18. Craniofacial defects of blastogenesis: duplication of pituitary with cleft palate and orophgaryngeal tumors.Duplications of organs and/or tissues are rare in morphogenesis and have frequently been attributed to incomplete twinning. To further elucidate the phenotypes associated with organ duplications, we present three infants with duplication of the pituitary gland (DPG). A review of previously reported cases with DPG showed that the commonest additional findings were hypothalamic enlargement, a broad or duplicated sella, cleft palate, hypertelorism, oropharyngeal tumors, agenesis or hypoplasia of the corpus callosum, and abnormalities of vertebrae. DPG and additional malformations constitute a distinct and recognizable pattern of anomalies, which may constitute a polytopic field defect due to splitting of the notochord. However, the precise inductive mechanism resulting in DPG remains unknown.- - - - - - - - - - ranking = 0.16666666666667keywords = gland (Clic here for more details about this article) |
6/18. Congenital lip pits and van der Woude syndrome.Van der Woude syndrome is an autosomal dominant disease characterized by lower lip pits with or without cleft lip and/or cleft palate. The lip pits commonly have salivary glands that drain into them, which leads to salivary flow from the lip pits. Lip pits may be associated with submucosal palatal cleft, velopharyngeal insufficiency, or genitourinary or cardiovascular anomalies. The pits are treated by surgical resection. The authors report a case of van der Woude syndrome with isolated lip pits and speech difficulties that had been unrecognized until the patient was 6 years old. The surgical technique is described to ensure that the often-bifurcating tracts are removed entirely.- - - - - - - - - - ranking = 0.16666666666667keywords = gland (Clic here for more details about this article) |
7/18. Poikiloderma, alopecia, retrognathism and cleft palate: the PARC syndrome. Is this an undescribed dominantly inherited syndrome?We report a clinicopathological observation concerning a man and his son affected by the same disease. The main cutaneous lesions included poikiloderma congenitale and generalized alopecia respecting the pubic area. These anomalies were associated with cleft palate and Robin sequence in the boy. The main histological changes consisted in a marked atrophy of hairs with persistence of sebaceous glands. In sun-exposed areas, we found colloid bodies, melanophages and hyperplasia of the elastic tissue network. Those features resembled those of the rothmund-thomson syndrome, but the phenotype and the apparent dominant mode of inheritance exclude this possibility. A new genodermatosis, related with, but distinct from the rothmund-thomson syndrome is suspected, for which the acronym PARC syndrome is proposed.- - - - - - - - - - ranking = 0.16666666666667keywords = gland (Clic here for more details about this article) |
8/18. Distichiasis and cleft palate.A patient with the unusual coexistence of distichiasis and cleft palate is described. Distichiasis is a rare congenital eyelid anomaly in which accessory eyelashes are present in the meibomian gland orifices. Its association with other systemic abnormalities is reviewed; the distinctions among distichiasis, trichiasis, entropion, and epiblepharon are outlined; and methods of treatment for distichiasis are described.- - - - - - - - - - ranking = 0.16666666666667keywords = gland (Clic here for more details about this article) |
9/18. Bilateral thymus found in association with unilateral cleft lip and palate.Ectopic localizations of the thymus have been reported in a variety of unusual sites, but not on the surface of the skin of the neck. As one can see in our case, the left neck mass (1.5 x 2 x 0.5 cm) and the right neck mass (4 x 4 x 0.5 cm), when added together in weight, also equal the total weight of the thymus at birth. Also, not being able to encounter the "sail sign" of the thymus gland on the chest x-ray proved to us that no other thymus gland was present. Because of these unusual findings, prior to excision of any congenital lesion in the neck, a biopsy should be done, and only after that should one proceed with the necessary surgical intervention. We are presenting this case because it is unique in this category.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
10/18. Absent meibomian glands in the ectrodactyly, ectodermal dysplasia, cleft lip-palate syndrome.A 27-year-old woman with the syndrome characterized by ectrodactyly, ectodermal dysplasia, and cleft lip-palate had an absent lacrimal punctum in each eye, with signs and symptoms of nasolacrimal obstruction during childhood. Examination disclosed bilateral corneal vascularization and opacification, with diffuse superficial punctate staining of the ocular surface epithelium by fluorescein, an instantaneous tear film break-up time, and normal Schirmer tear measurements. A full-thickness biopsy specimen of the eyelid confirmed the absence of meibomian glands that had been suspected because of absent meibomian gland orifices and secretions. The total absence of meibomian gland secretions in this patient may be a primary feature of this case and may contribute to a lipid-deficient and unstable tear film with resultant desiccation and destruction of the ocular surface epithelium. Breakdown of the corneal epithelium in association with obstruction and infection of the nasolacrimal system may be a particularly disastrous combination for the cornea that resulted in the recurrent, severe bacterial corneal ulcers found in our patient.- - - - - - - - - - ranking = 1.1666666666667keywords = gland (Clic here for more details about this article) |
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