Umbilical cord hemangioma associated with polyhydramnios, congenital abnormalities and perinatal death in a twin pregnancy.

A twin pregnancy is described in which an umbilical cord hemangioma, polyhydramnios, developmental abnormalities and perinatal death were restricted to one twin, while the other twin was unaffected. Cord hemangiomas are rare and their association with fetal abnormalities is controversial. This case study supports a direct association between the cord hemangioma and the adverse pregnancy outcome, since congenital abnormalities and a cord hemangioma were present in only one of the twins.

Bacillary angiomatosis of the spleen.

Bacillary angiomatosis is a recently described vasoproliferative lesion associated with infection by a newly characterized rickettsial organism, Rochalimaea henselae. Most previous reports have described skin lesions in immunocompromised patients infected with human immunodeficiency virus. This is the first case report detailing the features of bacillary angiomatosis of the spleen occurring in a patient undergoing cytotoxic chemotherapy for disseminated ovarian carcinoma.

Oral teratoma (epignathus) with intracranial extension: a report of two cases.

Epignathi are unusual congenital tumours presenting as oropharyngeal masses, often resulting in rapid asphyxia following birth. Occasionally, intracranial extension of the tumour is present, and two such cases are described. The presence of this complication, diagnosable by ultrasound examination, indicates that aggressive surgical treatment is inappropriate.

Fetal tissue involvement in the late infantile type of neuronal ceroid lipofuscinosis.

Prenatal diagnosis in a pregnancy at risk for late infantile neuronal ceroid lipofuscinosis (Batten's disease) was undertaken at 17 weeks' gestation by ultrastructural examination of amniotic fluid cells. The presence of curvilinear profiles indicated an affected fetus and the diagnosis was confirmed, after the pregnancy was terminated, by the finding of many typical curvilinear profiles in multiple tissues which included skin, amnion, umbilical vessels, blood, liver, and brain. Comparison between the involved cells in the amniotic fluid and fetal tissues suggests that these cells are probably derived from the periderm, and possibly also from the amnion. The prominent presence of cytosomes in the periderm and intermediate cells of the fetal epidermis and occasionally also in the endothelial cells of the dermis suggests that fetal skin may be a useful alternative site for assessing fetal involvement. Control specimens of the amniotic fluid, fetal skin, amnion, and liver showed no similar cytosomes. However, some control amniotic fluid samples did contain cells with large collections of irregular trilaminar membranes, and these could be open to misinterpretation. It is important that only typical curvilinear profiles are considered as an indication of an affected pregnancy.

Nodular breast lesions during pregnancy and lactation.

A review of the clinical and pathological features of 30 lactating nodules which were retrieved over the period 1975-1991 was undertaken. Clinically, the nodules occurred during any pregnancy and in subsequent pregnancies no further nodules developed. There were no favoured sites or lateralization of nodules, nor was there any correlation between nodule occurrence and ethnic background. Morphologically, a pre-existing underlying lesion was readily recognized in 18 cases. Seven fibroadenomas, five tubular adenomas, two of which were infarcted, and six breast hamartomas were identified. The other 12 cases were well circumscribed nodules with a variable degree of inflammation and lobular hyperplasia. It is best to recognize these lesions as a heterogeneous group and designate them as lactating nodules, noting any underlying pathological process rather than using the older term lactating adenoma which implies a single neoplastic entity.

Eccrine syringofibroadenoma: a report of 2 cases.

Eccrine syringofibroadenoma is a rare benign tumor of the skin arising from the acrosyringeal portion of the eccrine duct. The tumor was first described by Mascaro in 1963, and since then only 8 case reports in 6 publications have been documented. This lesion is likely to be misdiagnosed both clinically and histologically due both to its rarity and its similarity to other more aggressive skin tumors. This report of 2 cases is presented to highlight the salient pathologic and clinical features of the tumor and to describe the long term follow up of 2 patients in whom the lesions have been electively reviewed rather than excised.

A new treatment for acquired reactive perforating collagenosis.

Acquired reactive perforating collagenosis is reported in an insulin dependent diabetic patient with renal impairment, managed successfully with surgical debridement and split skin grafting. The literature on treatment of reactive perforating collagenosis is reviewed.

A clinical-histological study of individuals with diabetes mellitus and proteinuria.

Coexistent renal pathology with diabetic glomerulosclerosis was found in 38 of 136 (28%) consecutive renal biopsies performed primarily for proteinuria in individuals with diabetes mellitus. The histological lesions found were glomerulonephritis (14), focal tubulointerstitial disease (23), and amyloidosis (1). Significant microscopic haematuria was present in 66% of all patients and did not help to distinguish non-diabetic disease. The severity of diffuse diabetic glomerular disease was independently associated with duration of diabetes, raised plasma creatinine, the presence of hypertension, clinical retinopathy and neuropathy, but not with type of diabetes, degree of proteinuria or glycosylated haemoglobin at the time of biopsy. Diffuse interstitial fibrosis was related to the severity of glomerular disease and, if severe, also with a significantly (p less than 0.01) higher plasma creatinine. Coexisting renal disease was found to be associated with a significantly higher plasma creatinine (p less than 0.01) independent of the severity of diabetic glomerulopathy. Coexistent pathology is a not uncommon finding in renal biopsies from diabetic patients with proteinuria. These lesions and their underlying causes may not only influence the renal function and natural history of renal disease in diabetic individuals, but may also determine the response of proteinuria to therapy.

An unusual case of Sturge-Weber syndrome.

This report describes a case of cranio-facial angiomatosis with rapid growth of the angiomas leading indirectly to death at 3 mth of age. Extensive involvement of the cranial nerves, pituitary stalk, brain-stem meninges and liver and the presence of a Dandy-Walker malformation cyst have not been previously described in this condition.