| Macromastia is the massive enlargement of the breast, unilateral or bilateral, disproportional to growth in the remainder of the body. Most patients seen with macromastia have breasts that develop normally at puberty but simply reach excessive size. Virginal breast hypertrophy is a rare and distinct disorder with the rapid onset of macromastia at the onset of puberty. Although the disorder has been reported in the surgical, pediatric, and gynecologic literature, virginal breast hypertrophy has not been reported in dermatologic journals. We report two cases of virginal breast hypertrophy. Patient 1 is a 14-year-old girl who developed macromastia 6 months after a liver transplant for alpha 1-antitrypsin deficiency. She had intense erythema as well as verrucous hyperplasia overlying the breasts. Patient 2 was a 12-year-old girl who experienced severe bilateral breast enlargement 4 months after beginning treatment for thyrotoxicosis. She had extreme tenderness, erythema, and edema of the breasts and was treated with tamoxifen citrate, with improvement of her symptoms. Although virginal breast hypertrophy is a rare disorder, the dermatologist may be asked to consult on the associated skin changes in these cases and should be aware of its existence. The definitive therapy is surgical, but until breast growth is stabilized, cutaneous manifestations need to be managed. ( info) |
62/1102. Adenotonsillar hypertrophy: a precipitating factor of cerebrovascular accident in a child with sickle cell anemia. Cerebrovascular accident is one of the most serious complications of sickle cell anemia. The specific factors that predispose patients with sickle cell anemia to stroke are increased disease severity, higher baseline white blood cell count and lower baseline hematocrits. Likewise the presence of a co-existent alpha thalassemia trait and/or high fetal hemoglobin (HbF%) may reduce the risk. We report a child with sickle cell anemia and marked adenotonsillar hypertrophy resulting in obstructive sleep apnea syndrome. There was no other known risk factor for developing cerebrovascular accident in this child during her hospitalization for adenotonsillectomy. ( info) |
63/1102. Manifold manifestations of ergotism. Vasospastic side effects leading to organic manifestations are rare in ergotamine therapy. To our knowledge, combinations of more than two signs of ergotism have rarely been described in the literature so far. We present a 65-year-old male patient who as a consequence of severe migraine had developed ergotamine abuse. He was admitted to our hospital after one week of increasing abdominal pain. During laparotomy, necrotic areas of the small intestine and the sigmoid colon were resected, which on histopathologic examination revealed severe hypertrophy of the smooth musculature of mesenteric arteries, resulting from chronic vasospasms. Postoperatively, the patient developed ischaemia of the limbs which was confirmed by angiography. Before death, the patient also showed ischaemic signs in the acrae and necrosis of the tongue. ( info) |
64/1102. Reversible cardio-pulmonary changes due to adeno-tonsilar hypertrophy. Adeno-tonsillar hypertrophy, with signs of upper airway obstruction is a common presentation in ENT clinics. Recently it is identified as a major cause of sleep apnea syndrome. Several isolated case reports of pulmonary hypertension and corpulmonale appeared in the literature. The authors report two such children aged less than 2 years with cardio-pulmonary changes occurring secondary to chronic adeno-tonsillar hypertrophy that were successfully treated with the surgical removal. ( info) |
| OBJECTIVE: To present cases of coexisting sarcoidosis and sjogren's syndrome (SS). methods: We analyzed the clinical data of our 464 patients with SS with a clinical picture suggesting coexisting SS and sarcoidosis. We followed them for several years. RESULTS: We found 5 patients with coexisting SS and sarcoidosis. In 3 patients, pure sarcoidosis could mimic SS. CONCLUSION: In our experience, the frequency of sarcoidosis in SS is much higher than in the general population. One of the European criteria is that before the diagnosis of SS is made, the presence of sarcoidosis must be excluded. From our experience of 5 cases we determined that the 2 diseases can coexist, which indicates the value of these criteria. ( info) |
| A case of unilateral virginal breast hypertrophy with a review of the etiological factors and treatment modalities is presented. A 16-year-old girl presented with progressive enlargement of the left breast of 5 months' duration. The result of the mammographic examination was consistent with cystosarcoma phyllodes. Fine-needle aspiration biopsy revealed giant fibroadenoma. Although some of the characteristics of the fine-needle aspiration biopsy specimen were suspicious for cystosarcoma phyllodes, there were no adequate epithelial structures, which are obligatory for the diagnosis. The patient was treated with subcutaneous mastectomy and subpectoral insertion of a silicone gel implant. The histopathological examination was consistent with virginal hypertrophy. The breast maintained its volume with no further growth in the affected or in the normal breast after 4 years of follow-up. ( info) |
67/1102. A child with adrenocortical adenoma accompanied by congenital hemihypertrophy: report of a case. We report herein the findings of a 7-year-old male child with a ruptured adrenocortical adenoma and congenital hemihypertrophy which was incidentally detected after suffering a trauma. A review of 21 pediatric cases of adrenocortical neoplasms in the literature was made. The patient showed precocious puberty such as pubis and advanced bone age, but an endocrinological examination revealed no definite abnormalities. The right adrenal tumor with hematoma was resected after these evaluations. adrenocortical adenoma is considered to occur more frequently in female children. However, the incidence of adrenocortical tumors accompanied by congenital hemihypertrophy does not differ between males and females. The outcomes were relatively good, although the observation periods were short in some patients. A large number of patients presented with a tumor and hemihypertrophy on the same side. This finding is of interest when considering the possible association between hemihypertrophy of the organs and tumor proliferation. However, their association in terms of development was unclear. It is necessary for patients with hemihypertrophy to have regular examinations for the possible development of malignant tumors, especially in the kidney, adrenal gland, and liver. ( info) |
68/1102. Hydrops of placental stem villi complicated with fetal congenital adrenal hyperplasia. The authors present a case report of hydrops of placental stem villi. Numerous small aechoic spaces were demonstrated by prenatal ultrasonography. The patient spontaneously delivered a female newborn at 26 weeks' gestation. The infant showed hypertrophied clitoris and urogenital sius, and had a normal 46, XX karyotype. Endocrinological examination revealed that 3beta-hydroxysteroid dehydrogenase deficiency caused the anomaly. To our knowledge, this is the first report that congenital genital malformation complicated the placental mesenchymal dysplasia. ( info) |
69/1102. Congenital muscular dystrophy associated with calf hypertrophy, microcephaly and severe mental retardation in three Italian families: evidence for a novel CMD syndrome. We describe four Italian patients (aged 3, 4, 12, and 13 years ) affected by a novel autosomal form of recessive congenital muscular dystrophy. These patients were from three non-consanguineous families and presented an almost identical phenotype. This was characterized by hypotonia at birth, joint contractures associated with severe psychomotor retardation, absent speech, inability to walk and almost no interest in their surroundings. In addition, all patients had a striking enlargement of the calf and quadriceps muscles. Ophthalmologic examination revealed no structural ocular abnormalities in any of the children; one patient had severe myopia. In all cases a magnetic resonance imaging of the brain showed an abnormal posterior cranial fossa with enlargement of the cisterna magna and variable hypoplasia of the vermis of the cerebellum. Abnormality of the white matter was also present in all patients, in the form of patchy signal most evident in the periventricular areas. serum CK was grossly elevated in all. The muscle biopsy from all cases showed dystrophic changes compatible with congenital muscular dystrophy. Immunofluorescence studies showed mild to moderate partial deficiency of laminin alpha 2 chain. Linkage analysis in the only informative family excluded the known loci for congenital muscular dystrophy, including laminin alpha 2 chain on chromosome 6q2, the Fukuyama congenital muscular dystrophy locus on 9q3 and the muscle-eye-brain disease on chromosome 1p3. We propose that this represent a novel severe variant of congenital muscular dystrophy, with associated central nervous system involvement. ( info) |
70/1102. Biliary stricture due to hypertrophied liver rotation after right hepatic lobectomy. BACKGROUND: After partial hepatectomy, liver regeneration occurs with the hepatocyte mass to its almost previous size. Biliary stricture due to hypertrophied liver rotation following partial hepatectomy is uncommon and its management still remains controversial. We encountered extrahepatic biliary stricture due to hypertrophied left lobe rotation in 2 patients who had right hepatic lobectomy for liver trauma. Herein, we present our experience with the modified Longmire procedure for the management of this complication. RESULTS: There were no complications during the early postoperative period in either of the patients. After the Longmire procedure, the first patient was followed for 5 months with some elevation in liver enzymes and the other was followed for 14 months with normal liver function tests. Both of the patients are completely symptom free postoperatively. CONCLUSION: The present experience suggests that the modified Longmire procedure is the most promising surgical approach to the management of biliary stricture due to hypertrophied left lobe rotation after right hepatic lobectomy. ( info) |