The antenatal diagnosis of sacrococcygeal teratoma

The teratomas are rarely met with the newborn and the sacrococcygeal teratomas are the most often among them. A private case is detailedly described, correctly diagnosed antenatally by the ultrasound method, the clinical session and the outcome of the pregnancy. In the discussion the theories of their origin are shown, the anomalies connected with them and the delivery. The opinions of other authors on this matter have been quoted.     

Prenatal diagnosis and perinatal management of fetal sacrococcygeal teratoma

OBJECTIVE: To perform an exploratory analysis of the relative contribution of single MHC genes to the pathogenesis of systemic lupus erythematosus (SLE) in a homogenous white population. METHODS: MHC class II alleles and C4 allotypes were determined in 64 SLE patients and in ethnically matched controls. HLA-DR and DQ typing was performed by polymerase chain reaction amplification with sequence specific primers. C4 allotypes were determined by agarose gel electrophoresis. RESULTS: The frequency of C4A*Q0 was significantly higher in patients than in controls (46.9% v 25.3%, p = 0.002). HLA-DRB1, DQA1, and DQB1 alleles in the whole group of SLE patients were not significantly different from those of controls. On the other hand increase in DRB1*03 was observed in the group of patients with C4A*Q0, as compared with patients with other C4A allotypes (p = 0.047). There was no significant correlation between severe and mild disease, as judged by the SLEDAI, and HLADR, DQ alleles and comparing the patients with C4A*Q0 with those with other C4A allotypes there was no significant difference regarding clinical manifestations. CONCLUSION: The results are consistent with the argument that C4A deficiency contributes independently to susceptibility and the pathogenesis of SLE. C4A*Q0 in SLE patients in Iceland shows weaker linkage disequilibrium with DR3 genes than reported in most other white populations and emphasises the role of ethnicity.     

Perinatal and perianesthetic management of the sacrococcygeal teratoma in a neonate

We report perinatal and perianesthetic management of a female infant with sacrococcygeal teratoma who underwent fetal bladder puncture and postnatal tumor resection. At 33 weeks' gestation, fetal ultrasonography revealed an intrapelvic mass, oligohydramnios and the dilatation of the bladder. At 34 weeks' gestation, bladder puncture was performed in utero to relieve urinary obstruction by the mass. And it served to reserve the renal function but caused remarkable ascites at birth due to urine leakage to the peritoneum through the puncture site. After the delivery by cesarean section, the patient underwent the tumor extirpation at 2 days of life. The operation and anesthesia proceeded uneventfully. In previous reports, several mortalities due to exsanguinating hemorrhage during surgery have been reported. In addition, sacrococcygeal teratoma is occasionally accompanied by coagulopathy and high output cardiac failure caused by arteriovenous fistulae. Therefore it is important for good patient outcomes to evaluate preoperatively the risks mentioned above.     

Testosterone-producing benign cystic teratoma with virilism. A case report

BACKGROUND: Mature cystic teratomas are common tumors in reproductive-age women. Biologic activity in a small percentage of these neoplasms is well recognized. Thyroid hormone is the most commonly secreted substance. CASE: A 25-year-old woman presented with a large abdominal mass and virilism. Preoperative evaluation revealed elevated creatinine and testosterone levels and anemia. Computed tomography (CT) showed fat-fluid levels and multiple calcifications within the mass and obstruction of the right kidney. At surgery a 30-cm left ovarian mass was removed. Left ovarian vein testosterone levels were markedly elevated. Pathology reported a mature cystic teratoma. Postoperatively the testosterone levels returned to normal. CONCLUSION: In rare instances androgen production and virilization may occur with mature cystic teratoma.     

Malignant teratoma of the sacrococcygeal area in a fetus unrecognized in pregnancy

We report two infants with eosinophilic gastroenteritis (EG). This rare disease can mimic the clinical symptoms and US appearance of idiopathic hypertrophic pyloric stenosis (IHPS). US examination of the antropyloric region with a high-frequency linear transducer can assist in the differentiation of EG from IHPS, which is important because the therapeutic approaches are completely different. Eosinophilic gastroenteritis should be considered in the differential diagnosis of IHPS, especially when there has been an ineffective pyloromyotomy.     

A compound nevus in a benign cystic teratoma of the ovary

The occurrence of a compound nevus in an ovarian benign cystic teratoma is presented. This appears to be the first report of this lesion in the ovary without coincident melanoma. The case illustrates a probable origin for ovarian melanoma and raises a question concerning the origin of the melanocyte.     

Adenocarcinoma arising from the gastrointestinal epithelium in benign cystic teratoma of the ovary

Malignant transformation of benign cystic teratoma of the ovary is rare, with an incidence of 1.8%. The commonest malignant neoplasm to develop is squamous carcinoma (80%). Adenocarcinoma occurs with less frequency, and only one of which has ever been cited to be of gastrointestinal origin. A 38-year-old female underwent TAH-BSO due to a large right ovarian tumor. Microscopically and immunohistochemically, the tumor was defined as mucinous adenocarcinoma originating from gastrointestinal epithelium in benign cystic teratoma.     

A case of fibrin sealant application for closing benign trachea-esophageal fistula (TEF)

Surgical treatment of upper airway-esophageal communications are frequently lead to failure. Having previous experience of thoracic administration of Tissucol fibrin glue authors attempted the local application in a case of TEF. A young lady suffering from myasthenia gravis required longstanding artificial ventilation. Not surprisingly a TEP developed in the area of the tracheostomy. There was no room for surgical repair of the TEF. Two administrations of rapid acting form of Tissucol was needed following local astringent therapy and enzymatic debridement to achieve a complete and permanent closure of a tracheobronchial sinus in a diameter of 5 mm. In addition of meticulous technique and of general supporting therapy special attention was paid to the followings: 1/healthy wound edges 2/ local infection control 3/dry environment 4/patient building up strategy.     

Neonatal intracranial tumor highly suspected of teratoma in the lateral ventricle consisting of multiple cysts: case report]

The role of maternal diet in the development of the fetal brain has not been adequately explored. Marine n-3 fatty acids have, however, been proposed to be important for brain development. The present case-control study aimed to investigate the relationship between dietary intake during pregnancy and the occurrence of cerebral palsy (CP) in the offspring. Children with CP (n 109), born between 1984 and 1988 to mothers residing in the Greater Athens area, were identified at any time in 1991 or 1992 through institutions delivering care and rehabilitation. Successful nutritional interviews were conducted with ninety-one of these children. Controls were chosen among the neighbours of the CP cases or were healthy siblings of children with neurological diseases other than CP, seen by the same neurologists as the children with CP. A total of 278 control children were chosen, and 246 of them were included in the nutritional study. Guardians of all children were interviewed in person on the basis of a questionnaire covering obstetric, perinatal socioeconomic and environmental variables. A validated semiquantitative food-frequency questionnaire of 111 food items was used to estimate maternal dietary intake during pregnancy. Statistical analysis was done by modelling the data through logistic regression. Food groups controlling for energy intake were alternatively and simultaneously introduced in a core model containing non-nutritional confounding variables. Consumption of cereals (mostly bread) and fish intake were inversely associated with CP (P < 0.05 and P < 0.09 respectively) whereas consumption of meat was associated with increased risk (P < 0.02). A protective effect of fish consumption and a detrimental effect of meat intake have been suggested on the basis of earlier work and appear to be biologically plausible. If corroborated by other studies, these results could contribute to our understanding of the nutritional influences on fetal brain development.     

Aorto-esophageal fistula secondary to benign Barrett's ulcer: a rare cause of massive gastrointestinal hemorrhage

Aorto-intestinal fistula is a rare cause of massive upper gastrointestinal bleeding. The authors report on an 82-year-old man who had an aorto-esophageal fistula due to a benign Barrett's ulcer. The patient presented with a sentinel hemorrhage followed by fatal exsanguination despite vigorous attempts at resuscitation. Management of the entity requires awareness, appropriate radiologic and endoscopic investigation and early surgery. Even with appropriate management, the mortality remains high.     

Immature teratoma of the ovary with a neural component ("solid" teratoma). A clinicopathologic study of 20 cases

Twenty cases of immature teratoma of the ovary with a neural component are analyzed. A plea is made for use of the nomenclature adopted from the new World Health Organization classification of ovarian tumors, the past confusion over terminology and histogenesis of this rare tumor is discussed. All the primary tumors in the present series contained at least some immature tissues (predominantly of neural origin) and were thus graded from 1 to 3 according to the criteria of Thurlbeck and Scully. No grade 0 tumors ("benign solid teratomas") were identified. We believe that thorough sectioning almost always insures the identification of immature elements. The prognosis was closely related to the histologic grade, but correlated poorly withthe clinical stage, the latter being influenced by the common finding (25 per cent of the cases in this series) of peritoneal implants composed exclusively of mature glial tissue, which is associated with a benign clinical evolution. This phenomenon of maturation or differentiation appears to be the rule rather than the exception in this tumor, since implants are usually of better or equal differentiation when compared with their primary tumors and older patients tend to have lower grade tumors than younger patients. Since the majority of patients with this tumor are young, primary surgical therapy should be conservative, unilateral salpingooophorectomy often being sufficient. Spontaneous or operative rupture of the tumor capsule carries an increased risk of subsequent dissemination. We have noted impressive clinical responses in patients with disseminated tumors of a high histologic grade after treatment with triple chemotherapy (vincristine, actinomycin D, and cyclophosphamide) but do not recommend adjuvant therapy in patients with only grade 0 implants.     

Neonatal hypocalcaemia associated with rotavirus diarrhoea

We observed an association between rotavirus diarrhoea and hypocalcaemia in several patients and therefore started a prospective evaluation with measurement of calcium levels in all patients with rotavirus infection during a period of 8 months. We report on 54 infants with rotavirus gastro-enteritis. Serum concentrations of sodium, potassium, and total and ionized calcium were measured on admission. If hypocalcaemia was detected, total and ionized calcium were measured every day until recovery. Calcium was supplemented as calcium gluconate which was added to milk. Out of 54 newborns with rotavirus gastro-enteritis, 20 developed hypocalcaemia. All these newborns had severe diarrhoea. Seven infants were admitted because of convulsions, but EEG and ultrasonographic examination of the brain revealed no abnormalities. Once the infants' clinical condition and the consistency and frequency of the stool had improved, calcium concentrations increased and remained within the reference range without supplementation. CONCLUSION: Rotavirus gastro-enteritis seems to be a cause of neonatal hypocalcaemia.     

Neonatal encephalopathy with neuronal vacuolar degeneration

Spontaneous augmented breaths (active sighs) reduced the tidal volume and inspiratory time of succeeding breaths; manual lung inflations (passive sighs) reduced the tidal volume but had little effect on inspiratory time. Sighs in air, whether active or passive, reduced tidal volume more than sighs in hyperoxia (100% oxygen or 33% oxygen in nitrous oxide) after both active and passive sighs (overall difference about 10%); the reduction in inspiratory time after a sigh was less affected by gas mixture. Calculated mean inspiratory flow was reduced after passive sighs, but active sighs were more likely to cause arousal, which complicated the analysis. Tidal volume was reduced after a sigh partly because of reduced peripheral chemoreceptor input, the main effect of which was to reduce inspiratory flow, and partly because inspiratory time was shorter. Although the chemoreceptors may affect inspiratory time after a sigh, the greater effect of active sighs compared with passive sighs makes it likely that the shortening was either part of the neural output that causes spontaneous sighs, or was caused by mechanoreceptor input not mimicked by manual lung inflation.     

MRI in the assessment of a newborn with cervical teratoma

Routine anaerobic culture of urine identified the urinary tract as the primary focus of sepsis in a postoperative patient with Bacteroides fragilis septicaemia. Specimens of urine from six other symptomatic patients grew > 10(8) cfu/litre of a Bacteroides species in pure growth. The significance of these isolates is discussed. Multipoint technology and the availability of anaerobic work stations have facilitated anaerobic culture and reduced its cost. The incorporation of anaerobic culture of urine into routine laboratory practice may be clinically valuable and should be considered.     

Aortic root abscess with fistula formation

Aortic root abscess is a common complication of aortic valve endocarditis. However, aortic root abscess and formation of a fistula from the aortic root to the right ventricular outflow tract in the setting of a native aortic valve and previous repair of an aortic dissection with a Dacron graft is an uncommon event. Transesophageal echocardiography is superior to transthoracic echocardiography for the diagnosis of aortic root abscess. To our knowledge, no studies have compared the diagnostic value of cardiac MRI with transesophageal echocardiography for this condition.     

Testicular teratoma with a unique mode of spread and spontaneous maturation

A 52-year-old woman who developed a pale sclerotic second left toe with loss of the nail plate is described. Biopsy showed changes of lichen sclerosus. There were no other skin or genital lesions present.     

Neonatal encephalopathy with a pungent body odour

A neonate had transient unexplained bleeding into the gut, severe encephalopathy, and an abnormal pungent body odour. An inherited metabolic defect was excluded. The malodour was due to methanethiol and hydrogen sulphide, identified in urine. These sulphur compounds may have contributed to encephalopathy. Colonic bacteria were the probable source.     

Managing patients with benign prostatic hyperplasia

Benign prostatic hyperplasia is the usual cause of prostatism--irritative and obstructive urinary symptoms. Steps in making the diagnosis include a focused neurologic examination, urinalysis, serum creatinine determination and, possibly, catheterization to detect urinary retention. It is difficult to predict the likelihood of future complications, such as complete urinary obstruction, even for patients with severe symptoms. The natural course of prostatism is a waxing and waning of symptoms. Treatment options are watchful waiting, medication, transurethral prostatectomy, and newer surgical treatments such as microwave thermopathy and laser ablation. The family physician can counsel patients about the potential side effects of these treatments as well as the problems incurred by simply adjusting to the disabilities associated with benign prostatic hyperplasia.