1/147. Surgical management of macroglossia due to primary amyloidosis. A case of macroglossia due to primary amyloidosis is described, followed by a discussion of the various aspects of surgical intervention based on a literature review. ( info) |
| A case is reported of severe transient macroglossia after biopsy from the tongue in a 13-year-old boy who has had intermittent macroglossia since the age of 1 year as a result of extensive lymphangioma circumscriptum of the tongue. The acute lesions appear to result from haemorrhage into the lymphatic spaces following rupture of blood vessels in connective tissue septa and possibly secondary infection. ( info) |
3/147. Familial macroglossia-omphalocele syndrome. A kindred is reported in which 8 infants were affected with the macroglossia-omphalocele syndrome. Their characteristics varied from an almost complete clinical picture to nodular hyperplasia with cytomegaly of the adrenals as the only manifestation of the disease. Chromosome analysis was normal. This syndrome appears to be inherited as an autosomal recessive trait, with a high proportion of incomplete clinical forms. The index patient also has signs of the rubinstein-taybi syndrome. The simultaneous occurrence of these two syndromes is probably a fortuitous event. ( info) |
4/147. Vanished twin and fetal alcohol syndrome in the surviving twin. A case report. BACKGROUND: The diagnosis of twin pregnancy can be made early in pregnancy by ultrasonography (US). Follow-up examination occasionally demonstrates the disappearance of one of the twins. CASE: A twin disappeared on US examination; the surviving twin exhibited signs of fetal alcohol syndrome and other congenital anomalies, accompanied by a placental form of fetus papyraceus. CONCLUSION: Early US examination is useful for diagnosing multiple gestation. However, a follow-up examination is required to alert the clinician to the disappearance of one twin. Careful examination of the placenta may document fetal remnants. In this case a small, atretic nodule on the placental surface was evidence of the vanished twin. ( info) |
| Anesthetic considerations of 21-mo-old and 4-yr-old sisters with beckwith-wiedemann syndrome during surgical repair of cleft palate and reduction of macroglossia are presented and discussed. This syndrome is characterized by exomphalos, macroglossia, gigantism, hypoglycemia in infancy, and many other clinical features. This syndrome is also known as exomphalos, macroglossia, and gigantism (EMG) syndrome. Principal problems associated with anesthetic management in this syndrome are hypoglycemia and macroglossia. Careful intraoperative plasma glucose monitoring is particularly important to prevent the neurologic sequelae of unrecognized hypoglycemia. It is expected that airway management would be complicated by the macroglossia, which might cause difficult bag/mask ventilation and endotracheal intubation following the induction of anesthesia and muscle paralysis, so preparations for airway difficulty (e.g., awake vocal cord inspection) should be considered before induction. A nasopharyngeal airway is useful in relieving postoperative airway obstruction. ( info) |
| AL amyloidosis was diagnosed in a 56-year-old woman with spontaneous purpura, macroglossia and hepatomegaly, a serum IgGk monoclonal gammopathy and a 25% plasma cell bone marrow infiltration. She was started on high-dose treatment consisting of four monthly cycles of VID chemotherapy, then underwent a stem cell collection after priming with cyclophosphamide G-CSF. Myeloablative therapy was with melphalan and busulfan. Hematologic recovery was fast and uncomplicated. At follow-up 22 months from ASCT, the patient shows a complete remission of the clonal plasma cell disorder, normalization of liver size and alkaline phosphatase level and a significant improvement in the signs of vascular and soft tissue amyloid infiltration. ( info) |
7/147. Traumatic macroglossia: a life-threatening complication. OBJECTIVE: To describe the use of muscle relaxants and a bite raiser to avoid continued tongue trauma. DESIGN: Case report. SETTING: A tertiary general intensive care unit (ICU). INTERVENTIONS: muscle relaxation and bite raiser. MAIN RESULTS: muscle relaxation and a bite raiser were used in a 17-yr-old male with traumatic macroglossia, which allowed for rapid resolution of edema and prevented additional trauma to the tongue. CONCLUSION: Early use of a bite raiser together with muscle relaxants allows for more rapid solution of edema and prevention of additional trauma to the tongue in patients with traumatic macroglossia. ( info) |
8/147. Lymphangiomatous macroglossia. Lymphangiomatous macroglossia, or giant tongue, usually presents within the first two years of life. The tongue enlarges to the point of protrusion from the mouth with resultant ulceration and frank necrosis of the tip. There may be associated malocclusion and prognathia produced by the enlargement of the tongue. The pathology and clinical manifestations of lymphangioma of the tongue are discussed in this paper, and a case report is presented. ( info) |
9/147. adult orthodontic treatment: a challenging case report. The case of a 61-year-old woman with a primary diagnosis of spondyloepiphyseal dysplasia and severe maxillary and mandibular spacing as well as anterior and posterior crossbites is presented. Clinical findings led to a diagnosis of macroglossia which required partial glossectomy in addition to comprehensive orthodontic treatment to correct malocclusion. ( info) |
10/147. Extensive haemorrhagic-bullous skin manifestation of systemic AA-amyloidosis associated with IgGlambda-myeloma. In an 86-year-old woman with a multiple myeloma of the IgG lambda subtype a coinciding systemic amyloidosis manifested as a macroglossia, diffuse alopecia and generalized cutaneous involvement. The skin was affected by milium-like papules, petechial haemorrhages and an increased tissue fragility with subsequent blister formation. The typical histology and immunohistology pattern revealed large intradermal amyloid masses, reacting positively with anti-amyloid A antibodies, which surrounded cuff-like dilatated blood capillaries. The abundance of these amyloid deposits led to significant deflexibilization and fragility of the capillaries and the dermal matrix eventually resulting in the haemorrhagic-bullous eruptions. The peculiar feature of the present case is the intensity of bullous-haemorrhagic skin damage due to amyloid A deposition without any detection of cutaneous IgGl as the myeloma-derived paraprotein assumed to be causative for the development of systemic AA amyloidosis. ( info) |