Ultrasound screening strategies for the diagnosis of congenital anomalies of the kidney and urinary tract.

PURPOSE: Congenital anomalies of the kidneys and urinary tract (CAKUT) are among the most common anomalies in newborn infants, and may cause chronic renal disease in newborns. We investigated the sensitivity and specificity of different ultrasound-based screening strategies for CAKUT. MATERIALS AND METHODS: Newborns (n = 4331) were analyzed for CAKUT in at least one ultrasound examination as a part of the Survey of Neonates in Pomerania (SNiP), a 7-year population-based study on neonates in Western Pomerania (Germany). Intrauterine ultrasound examinations were compared with early postnatal ultrasound findings (from days 3 - 7 of life) and pathological findings within the first 6 months of postnatal life. RESULTS: Cases of CAKUT were detected in 309 (3.7 %) kidneys in one ultrasound examination at the following points of time at least: (i) prenatally in 56 newborns (18.2 %), (ii) 3 - 7 days postnatally in 201 newborns (65.2 %) and (iii) in 52 newborns (17 %) during the 6-month follow-up. The prevalence was significantly higher in male infants, and hydronephrosis was found to be the most frequent obstructive nephropathy (83.3 %). Significant co-morbidity was observed with CNS malformations. The diagnostic sensitivity was significantly higher in postnatal ultrasound screening (79.6 vs. 18.2 % prenatally), while the specificity was above 99 % at all time points. CONCLUSION: This study demonstrates a high prevalence of CAKUT and demonstrates the importance of combined prenatal and postnatal ultrasound examinations for early CAKUT diagnosis.

[Cavernoma of the medulla oblongata mimicking "Anorexia nervosa" - a case report]. / Kavernom der Medulla oblongata unter dem Bild einer "Anorexia nervosa".

An 11-year-old girl presented with recurrent vomiting, reduced food and fluid intake, weight loss and dizziness. In an external hospital she was diagnosed as having habitual vomiting and a beginning eating disorder. The physical examination revealed a very thin, malnourished child with abdominal pain on palpation but without neurologic deficits. Laboratory findings, X-rays, endoscopy and ultrasound revealed no pathological results either. Since the EEG and the cranial computed tomography (CT) were normal, we also suspected the beginning of an eating disorder especially due to some psychical peculiarities. The MRI which was done for ultimate exclusion of an infratentorial tumor showed a well defined displacing structure in the dorsal medulla oblongata and in the upper cervical spinal cord. A corresponding prolongation of the central conduction time was found in the median nerve SSEP whereas the BAEPs were normal. The tumor was excised in toto and the histological examination confirmed the suspected diagnosis of cavernous hemangioma (cavernoma). The post operative phase was without any complications and the intiated nutrition was well tolerated. Neurological deficits were not observed.

[Prepontine epidural hemorrhage]. / Präpontine epidurale Blutung.

Extradural haematomas of the clivus represent a rare event and occur almost only in infancy. The case of a haematoma promptly operated upon via a transdural cerebellopontine angle approach is described for the first time. The mechanism of injury is discussed.

Maffucci's syndrome as cause of chronic liquorrhea.

We present the case of a 14-year-old girl suffering from Maffucci's syndrome, a rare ailment belonging to the group of osteochondrodysplasias. At the age of 6 months, a diffuse swelling first appeared in the girl's right cheekbone region and the periauricular area. Because of recurrent meningitis with massive otoliquorrhea, several surgical revisions were performed, beginning at the age of 4 years. The histological and immunohistochemical diagnosis showed hemangioma and lymphangioma with enchondroma. As a peculiarity of our patient's diagnosis, we found multiple bone defects apparently caused by venous and lymphomatous angiomatosis. There was also enchondromatosis of the skull base and the upper cervical vertebrae, which caused the recurrent otoliquorrhea and rhinoliquorrhea. A fistula closure was undertaken through a retromastoidal, suboccipital approach and fascial graft of the posterior cranial fossa.

The diagnostic importance of eosinophil granulocytes in the CSF of children with ventricular-peritoneal shunt systems.

We investigated the occurrence of cerebrospinal fluid (CSF) eosinophilia in patients with ventriculo-abdominal shunt systems with regard to possible infection. For this purpose, we examined the CSF of 83 children at the time of shunt obstruction or malfunction. In 32 children (38.6%) we found more than 4% of eosinophil granulocytes in the CSF with a maximum of 76%. In 15 of these 32 children the CSF was sterile, whereas in 17 patients bacterial infection was present. In the CSF of 16 of those 17 children, Staph. epidermidis was cultivated. There was statistically significant correlation between positive Staph. epidermidis culture and the occurrence of CSF eosinophilia (P<0.05). The occurrence of CSF eosinophilia in patients with ventriculo-peritoneal shunts is well known and was mostly attributed to an allergic reaction. Our findings support the theory of an infectious etiology of the eosinophilia and are thus in line with new American and French studies. We believe that CSF eosinophilia indicates a persistent infection of the central nervous system by the contaminated shunt system. As the organism which is the most common cause we cultured Staph. epidermidis.

[Use of the Glasgow Coma Scale in pediatric craniocerebral trauma]. / Die Anwendung der Glasgow-Coma-Skala beim kindlichen Schädel-Hirn-Trauma.

Over five years the applicability of a modified Glasgow Coma Scale was analysed in 38 children (mean age 7.2 +/- 3.8 years) with head and associated injuries (47.4%). The score was estimated after the accident and in the course of intensive therapy. At the beginning of the treatment on the intensive care unit, the cases were staged according to the severity of the head injuries (Glasgow Coma Scale: 4-8, 9-12 and 13-19 points). At the stage evaluated as between 4 and 8 points, 50% of the patients died and the survivors were ventilated (11.7 +/- 10.7 days) and intensively treated (45.7 +/- 31.5 days). All patients had had neurological damage. Additional injuries worsened the prognosis in the acute phase. 42.9% of the patients received intracranial pressure monitoring. In the patients between 9 and 12 points, the time of ventilation (3.7 +/- 2.9 days) and of intensive therapy (19.5 +/- 13.3 days) decreased. Over 13 points, all patients had a shorter duration of treatment (10.8 +/- 8.8 days) and a quick and good recovery. The Glasgow Coma Scale has the advantage of an examination with a quantitative analysis and resulting effective diagnostic and therapeutic measures. Even the inexperienced physician can use the Glasgow Coma Scale with success at the site of the accident.

[Case contribution on intracranial dermoid in early childhood]. / Kasuistischer Beitrag zum intrakraniellen Dermoid im Kleinkindesalter.

Case-report on an intracranial dermoid with reference to origin, symptoms, diagnostics, therapy and prognosis of that during the childhood rarely diagnosed tumor.