Renovascular hypertension in childhood: successful treatment by renal autotransplantation.

Five cases of renal autotransplantation for renovascular hypertension in children are described. Hypertension in these cases was managed temporarily with medications until the patients grew to a size that made an operative procedure feasible. Following operation the blood pressure returned to normal in three patients without any antihypertensive medication during the period of follow-up (8 months to 6 years). In the other two patients, small doses of antihypertensive medications still were needed, despite correction of the stenosis. The results of this series demonstrate that autotransplantation is a safe and effective procedure in childhood renovascular hypertension in contrast to other forms of vascular reconstruction, which carry a high rate of failure in this age group.     

Benign symptomatic lesions of the pineal gland. Report of seven cases treated surgically

Summary Seven patients with non-neoplastic symptomatic lesions of the pineal gland have been operated on in our department since 1988. Clinical symptoms were slight and there were intermittent signs of a space occupying lesion in the quadrigeminal area, i.e. visual and gait disturbances. In three patients, obstructive hydrocephalus with related symptoms was found. Diagnosis was confirmed by use of MRI in sagittal planes, revealing a cystic lesion in five and a solid tumour in two patients. All patients were operated on without complications using an infratentorial supracerebellar approach. Histological examination showed glial cysts of the pineal gland in five patients. In the two solid specimens, normal pineal tissue was found. These lesions seem to be of special interest, as the only pathological property are their size: Both lesions — too large pineal glands — caused obstruction of the outlet of the third ventricle with subsequent hydrocephalus. Surgical treatment was curative in all cases with prompt relief of the symptoms. Clinical symptoms and signs, diagnostic and pathological findings, as well as the surgical results of these cases will be reported.     

Renovascular hypertension in children.

OBJECTIVE: To study the etiology, clinical spectrum. image findings, management and outcome of children with renovascular hypertension (RVH). MATERIAL AND METHODS: Twenty children (aged 5 days to 15 years) were studied and treated for RVH during 1977-1998. In 14 cases hypertension was found during a routine examination. Six cases had heart failure and/or hypertensive encephalopathy. Diagnosis was made with aortography. Post-captopril renography and Doppler ultrasonography were obtained in 8 patients and spiral computed tomography angiography in 2. Treatment consisted of surgery (8 patients), percutaneous transluminal angioplasty (PTA) (5) or antihypertensive drugs only (8). RESULTS: Initial blood pressure was 62 +/- 31 mmHg > 95th percentile for systolic and 44 +/- 22 mmHg for diastolic blood pressure. Twelve children had unilateral and 8 had bilateral arterial stenosis. In 3 cases lesions were intrarenal. RVH was due to fibromuscular dysplasia (7 patients) and associated to middle aortic syndrome (5). neurofibromatosis (3), William's syndrome (2). Takayasu's arteritis (1) and pheochromocytoma (1). Treatment of choice was decided depending on the size of the child and location and severity of the stenosis. At the end of the follow-up (78 +/- 49 months), 9 patients are normotensive without medication and 7 are normotensive with drugs. Three patients have died, 2 for unrelated causes and I for cardiac failure; 1 child was lost to the follow-up. CONCLUSIONS: Although symptoms are relatively uncommon. renovascular disease is a frequent cause of severe hypertension in childhood. Non-invasive diagnostic techniques appear useful as screening methods. Treatment by surgery or PTA is successful if patients are carefully selected.     

Vaginal hydrocele. Report of 55 surgically treated cases

The authors analyze retrospectively the files of patients who have been operated on for hydrocele between 6th January 2000 and 27th November 2001 (23 months) in Talanga? hospital at Brazzaville. The overall operation rate for that pathology was 4.44%. Prevalence according to age group is as follows: 14 infants (25.45%), three adolescents (5.45%); 14 adults (25.45%); 24 old persons (43.64%). The localization of hydrocele was right in 54.55% of cases, left in 27.27% and bilateral in 18.18% of cases. In all patients, there was a case of idiopathic hydrocele. Patients got the surgical cure more frequently through the homolateral scrotal route than inguinal and the post-therapeutic healing occurs in all cases without recurrence. The authors make comments on those results and they specify certain current data about the disease.     

The acromegaly-gigantism syndrome. Report of four cases treated surgically

Four cases of growth-hormone-secreting pituitary adenoma, with associated aspects of acromegaly and gigantism, are reported in patients aged 12–26. All of the patients had macroadenomas and were treated surgically, three by the transsphenoidal approach and one with a transfrontal craniotomy. Histologic examination revealed eosinophilic adenomas in three of the cases and a mixed eosiophilic-chromophobe adenoma in one, all with cellular irregularities (mitosis and cellular and nuclear polymorphism), local invasivity, or both. Because surgical treatment did not produce complete normalization of growth hormone levels, radiotherapy followed the operations in all four cases. In our opinion, the treatment of acromegalic gigantism poses more therapeutic problems than that of simple acromegaly, with combined treatment (surgical, radiation, and medical) often being necessary.     

Surgical treatment of renal artery stenosis in children 60 surgically treated cases]

Sixty children with renal artery stenosis (unilateral in 44, bilateral in 16) underwent surgical treatment. Ten nephrectomies and 69 vascular repair procedures were performed. Postoperative follow-up ranged from one to 14 years. After surgery, blood pressure returned to normal and subsequently remained so in 54 patients without any antihypertensive medication. This represents a 90% cure rate. In six patients blood pressure remained high due to lesions of the renal parenchyma. Fibrous dysplasia of the renal artery was the prevailing pathologic finding. In children, associated lesions are especially frequent: coarctation of the abdominal aorta, stenosis, occlusion, aneurysms of the splanchnic arteries, pheochromocytoma. Postoperative prognosis of renovascular hypertension in children is good since atheroma and visceral or renal lesions are usually lacking.     

Gastric metastasis from melanoma. Report of 2 surgically treated cases

Metastatic disease involving the gastrointestinal tract is a rare pathology and melanoma is the extra-intestinal neoplasm more frequently concerned. Two cases of gastric metastases, revealed by symptoms of upper gastrointestinal bleeding, are reported. In the first case the disease-free interval, after excision of the primary lesion located in the right lower limb, was 13 years; in the second case the primary lesion remained unknown, although it probably originated from a giant congenital nevus of the left foot. Both patients had been affected before by inguinal nodes metastases, treated by radical groin lymphadenectomy; the concomitant multiple metastases to other sites (adrenal glands, retro-peritoneum, liver, lung, small bowel, brain, ovaries) limited surgery to a likely prospect of palliation, conditioning an unfavourable prognosis. Resection of gastrointestinal metastases is justified for the relief of intestinal hemorrhage (as in these reported cases), perforation and obstruction, even if treatment of single non-complicated lesions can have a curative intent. The conclusion is drawn that more aggressive diagnostic and staging procedures are indicated for the early detection of gastrointestinal metastases whenever non-specific abdominal symptoms and a history of melanoma are present.     

Microscopic bone pathology in two cases of surgically treated secondary hyperparathyroidism. Report of a distinct skeletal lesion

We report bone biopsy findings in two hemodialysis patients with a history of secondary hyperparathyroidism treated by parathyroidectomy. Discrete, punched-out lesions in trabecular bone, which show little evidence of resorption or osteoblast activity, probably correspond to previous zones of osteoclastic tunneling resorption. Because we have seen these lesions in no other clinical setting, we suggest that they are residua of healed secondary hyperparathyroidism in dialysis patients. Their persistence may be due in part to aluminum intoxication.     

Idiopathic portal hypertension in renal transplant recipients: Report of two cases

We present herein the cases of two patients who developed idiopathic portal hypertension (IPH) following renal transplantation. Both patients had been treated with azathioprine and prednisolone for 6 years and 4 months and for 4 years and 7 months, respectively, and presented with splenomegaly and thrombocytopenia suggesting hypersplenism. Celiac angiography showed a dilated splenic artery and vein in both patients. When the splenic artery was obliterated with a balloon catheter in case 1, the portal venous pressure decreased from 51 cmH₂O to 36 cmH₂O and the direction of the superiomesenteric venous blood flow became hepatopetal rather than hepatofugal. These results suggested that the spleen might have played an important role in the development of IPH in these two patients. A splenectomy was therefore performed, immediately following which the portal venous pressure decreased remarkably, and the esophageal varices disappeared during the postoperative follow-up period. Microscopic examination of liver biopsies taken at the operation revealed lymphoplasmacytic infiltration with bile duct hyperplasia but no evidence of periportal fibrosis, and electron microscopy demonstrated very mild perisinusoidal fibrosis. Thus, the histological changes seen in the livers of these patients seemed not to have caused the portal hypertension. In conclusion, although few patients develop IPH after renal transplantation, we should be aware of its possibility and consider splenectomy as the treatment of choice.     

Congenital mitral incompetence and coarctation of aorta: Report of two cases treated surgically 1

Two patients with congenital mitral incompetence and coarctation of the aorta are presented. One patient had associated patent ductus arteriosus, bicuspid aortic valve, and endocardial fibroelastosis. The diagnosis in the two patients presented is well established by clinical, laboratory, and surgical findings and also by necropsy examination in one case. It is proposed that the rarity of reported cases in the literature may have resulted from the frequent diagnosis of left ventricular failure in infancy secondary to coarctation, leading to the assumption that a mitral insufficiency murmur, when present, is due to functional regurgitation. Likewise, the murmur may be mistakenly thought to originate from a ventricular septal defect.     

CNS involvement of Langerhans cell histiocytosis. Report of 23 surgically treated cases

We present 23 cases of Langerhans cell histiocytosis with central nervous system (CNS) involvement. The major complaints were a mass on the cranial vault in fifteen (65 %), visual disturbance in four (16 %), polyurea-polydipsia in three (13 %), and progressive weakness in all extremities in one patient (4 %). Neurological examination revealed no abnormality in sixteen patients (70 %), cranial nerve palsy, visual field defect and optic atrophy in six (26 %) and paraparesis in one (4 %). Tumoral mass was found to be located on the cranial vault (65 %), in the suprasellar region (21 %) and in the spinal column (8 %). The cranium and spinal column were both involved in one patient. All patients underwent surgery; craniectomy with grossly total tumor excision plus cranioplasty (65 %), craniotomy with subtotal tumor excision (26 %), and vertebrectomy with grafting (13 %) were performed. The clinical radiological and histopathological features, as well as therapeutical considerations are discussed and the pertinent literature is reviewed.     

Clinical evaluation of renal autotransplantation in renal vascular hypertension

From 1976 to 1984, 22 cases of renal vascular hypertension were treated with renal autotransplantation. 20 cases have been followed up for 3-11 years, 17 cases of them over 5 years. The results showed that 14 cases were cured, 5 cases relieved, 1 cases ineffective. 19 cases of these patients suffered from large arteritis, 3 cases from atherosclerosis. The clinical efficacy of the former was better than the later. Our results suggest that the clinical efficacy has nothing to do with the course of disease. For bilateral renal arteriostenosis, as long as the extent of contralateral arteriostenosis do not exceed by 50%, good result can be achieved by means of renal autotransplantation for more severe side arteriostenosis.