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The presence of a circumscribed area of alopecia on the scalp raises several differential diagnostic considerations. A ring of hypertrichosis around such a lesion is called "hair collar sign" and raises suspicion of ectopic neural tissue and an underlying defect of the skull. We report two cases of male newborns with a hair collar surrounding a small localized area of scalp alopecia. In one child a cephalocele was diagnosed radiologically. In the other child an osseous defect was ruled out, and the histological examination confirmed the diagnosis of rudimentary meningocele after complete excision. The importance of this clinical sign has not been addressed in the German dermatological literature., (© The Authors • Journal compilation © Blackwell Verlag GmbH, Berlin.)

Two black and white female German Holstein calves showed malformations of the cerebrum. The first calf exhibited a cystencephaly and the second calf a meningoencephalocele. The animals originated from two different dairy farms. Both calves were sired by two unrelated sires used in artificial insemination. The calf affected by cystencephaly was lacking the corpus callosum which may had been caused the cystencephaly. Exept for a pressure atrophy, the remaining parts of the brain were macroscopically and histologically inconspicious. Histological examination of the cerebrum, brain stem and cerebellum in the second calf did not reveal specific changes. A further finding in the second calf was a unilateral anophthalmia. Both animals were affected by additional defects in the spinal column including brachyuria, duplications and fusions of vertebral bodies and rips as well as malformations of the heart such as ventricular-septal defects. Only mild clinical symptoms could be observed in both calves. The calves were not inbred and further calves affected by the identical anomalies could not be ascertained at the farms where the calves were born. Chromosomal anomalies could not be detected after examination of metaphase spreads using light microscopy.

Background: Occult malformations of the lateral skull base are rare anomalies, but can cause severe complications such as recurrent meningitis. Therefore, they need to be precisely delineated and sufficient surgical closure is mandatory., Patients and Methods: Between 1986 and 2004 twenty patients (10 children and 10 adults) with occult malformations at the lateral skull base were treated surgically at the ENT-Department of the Hospital Fulda gAG. Of these 3 Mondini-malformations, 11 defects of the tegmen tympani or the mastoidal roof, 2 dural lesions to the posterior fossa and 4 malformations within the pyramidal apex have been found. Four patients have had multiple anomalies. Routing symptom was in all cases at least one previous meningitis. Radiological diagnostics included high-resolution computed tomography (CT) and magnetic resonance imaging (MRI) as well as CT- or MR-cisternography. Depending on type and localisation of the defect the following surgical algorithm was carried out: The trans-mastoidal approach was used in all cases of Mondini-malformation (including obliteration of the ear), in case of lesions to the posterior fossa as well as partly in anomalies at the tegmen tympani and mastoidal roof, respectively. Defects of the pyramidal apex should be explored via the trans-mastoidal way if the lesion is located caudally to the inner auditory canal (IAC), whereas the trans-temporal approach should be used if the lesion is situated ventral to the IAC and dorso-medially to the internal carotid artery (ICA). The trans-temporal approach was also performed in large defects of the tegmen tympani and mastoidal roof as well as in recurrences., Conclusions: In all cases of recurrent meningitis caused by agents of the upper airway tract the basic principle should be to search for occult skull base malformations radiologically as well as by sodium fluorescein endoscopy as long as the anomaly is detected.

Background: There are transmastoid, transtemporal and combined approaches for surgical management of endaural encephaloceles. The advantages of a transmastoid approach are the exploration and option of surgical treatment of the middle ear cavity without craniotomy. Nevertheless there are recent studies preferring the transtemporal or combined approach because of the better survey., Patient: We present a 27-year old male patient, who developed hearing loss and otorrhea of the right side. He underwent middle ear surgery associated with cholesteatoma three times before. On examination there was a smooth pulsatile mass taking origin in the roof and almost filling the external auditory canal. High-resolution CT and MRI scans of the petrous bone demonstrated a spherical soft tissue mass communicating with the middle cranial fossa. The encephalocele was exposed and resected through a transmastiodal approach. Behind the cele a relapsing cholesteatoma was found and resected in the same session. The laterobasilar defect was sealed with ear cartilage and a perichondrium flap., Conclusion: There are three different surgical approaches to manage laterobasilar encephaloceles. Due to the history of a previous cholesteatoma we performed a transmastoidal approach. A transtemporal approach alone would have failed to notice the relapsing cholesteatoma and would have led to a greater trauma.

The most frequent complication of an acute cerebral lesion is brain edema. With increasing size of brain edema an increase in intracranial pressure is observed. This leads to decreased cerebral perfusion and brain death. Cerebral edema can be subdivided into three groups: vasogenic, cytotoxic and interstitial brain edema. This classification is of relative importance because only the extracellular edema can be influenced by drugs. Clinical symptoms of brain edema are related to the increased intracranial pressure, associated with decreased cerebral microperfusion. Symptoms are headache, vomiting, vision disturbances and, with increased intracranial pressure, bradicardy, respiration disturbances and at the end brain death by compression of the cerebrum. Therapy is directed to the underlying disease. Unfortunately, even today specific therapeutical approaches are limited.

The role of high-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in the diagnosis of preoperative and postoperative complications caused by acquired cholesteatomas will be described in this paper. The pre- and postoperative imaging of the temporal bone was performed with HRCT and MRI.HRCT and MRI were performed in the axial and coronal plane. MRI was done with T2 weighted and T1 weighted sequences both before and after the intravenous application of contrast material. All imaging findings were confirmed clinically or surgically. The preoperative cholesteatoma-caused complications depicted by HRCT included bony erosions of the ossicles, scutum, facial canal in the middle ear, tympanic walls including the tegmen tympani, and of the labyrinth. The preoperative cholesteatoma-caused complications depicted by MRI included signs indicative for labyrinthitis, and brain abscess. Postoperative HRCT depicted bony erosions caused by recurrent cholesteatoma,bony defects of the facial nerve and of the labyrinth, and a defect of the tegmen tympani with a soft tissue mass in the middle ear. Postoperative MRI delineated neuritis of the facial nerve, labyrinthitis, and a meningo-encephalocele protruding into the middle ear. HRCT and MRI are excellent imaging tools to depict either bony or soft tissue complications or both if caused by acquired cholesteatomas. According to our findings and to the literature HRCT and MRI are complementary imaging methods to depict pre- or postoperative complications of acquired cholesteatomas if these are suspected by clinical examination.

The VEP was investigated in 172 patients with infratentorial and in 100 patients with supratentorial space occupying lesions. In each group 65% of the patients showed marked or even extensive changes in VEP. An immediate relation of the lesion to the optochiasmatic system or a hydrocephalus was noted in a few cases only. In contrast the VEP of patients with a clear transtentorial herniation showed severely pathological changes, occasionally up to complete loss of the potential. The VEP changes were reversible on removal of the space occupying lesions in all patients. The constellation of VEP changes in subgroups of patients with different types of the space occupying lesions, as well as the observed symmetry of the VEP changes, allow the assumption that the functional inhibition of the optic pathway occurs in the perimesencephalic part via their compression or stretching. The assumption is supported by pathoanatomical MRT findings in some cases. It seems, therefore, that VEP investigations enable the registration of a compression or torsion of the cranial mesencephalon (mesencephalo-diencephalic mechanical irritation) or the presence of a clinical silent transtentorial herniation in space occupying lesions. The loss of the VEP appears to yield an early and certain signal of the progressing mid-brain syndrome.

Within 27 months 122 patients with severe head injury were treated at our clinic. Of these patients twelve (9.8%) were categorized as having a primary brain stem lesion (9 male and 3 female, mean age 28.3 years (17 to 73 years). Their injuries were caused primarily by traffic accidents. Initial and follow-up CT ruled out mass lesions or other causes for transtentorial herniation, supporting the diagnosis of primary brain stem lesion. Respiratory insufficiency and control of vegetative function demanded artificial ventilation and analog-sedation for up to 32 days (mean 18 days) on our Intensive Care Unit. In all patients we performed initial and follow-up CT scans, ICP monitoring, evoked potentials (AEP, SSEP) and TCD. MRI was carried out in four patients. One patient died during the acute hospital phase, 7 were transferred in poor and four in good condition. During rehabilitation one patient died, two, one in a vegetative state and one in poor condition were transferred to a caring facility. Eight patients with a good or moderate recovery were dismissed home, subsequently regaining their prior social function. The primary traumatic brain stem lesion presents as a dramatic clinical picture. As shown in our series the prognosis is good independent of the duration of coma. The important prognostic factors were the primary neurological state according to the Gerstenbrand and Luecking classification, the degree of the brain stem lesion in CT scan and MRI, and normal evoked potentials, indicating a favourable outcome.

Eleven patients (6 female, 5 male) with unilateral (6) or bilateral (5) (peri)orbital encephaloceles (nasofrontal in 4 cases, naso-orbital in 3, spheno-orbital in 4), were ophthalmologically examined. All had normal anterior segments, but ipsilateral or bilateral anomalies of the optic nerve head such as coloboma, pits, morning glory syndrome, dysplasia, tilted disk syndrome, hypoplasia, or a persistent hyaloid artery. In regard to the pathogenesis of (peri)orbital encephaloceles associated with optic disk malformations a common clefting defect in the neuroectodermal and neurocristal midline structures of the head is postulated, taking place in the fourth to sixth week of development.

Reference is made to a case history of prenatal use of ultrasonography, in 34th week of pregnancy by which formation of a ventral cleft in conjunction with cephalocele was diagnosed. Additional methods to secure diagnosis are described and compared, including alpha-foetal protein evaluation and amniofoetography. Also discussed are obstetric consequences likely to result from possible prepartum diagnosis of such malformations.

We report the case of a 4.9-year-old boy with congenital hydrocephalus and obstruction of his shunt who just after the induction of anaesthesia suddenly developed generalized muscle rigidity that made intubation impossible. Because of temporary mydriasis the situation could hardly be differentiated from acute cerebral herniation. The lack of any decrease in muscle tone after emergency trephination and drainage of the right lateral ventricle and the immediate improvement following intravenous dantrolene left no doubt about the diagnosis of malignant hyperthermia. The diagnosis was confirmed by the increase in serum creatine phosphokinase and the documentation of massive myoglobinuria. Reconstruction of the course of the first anaesthetic necessary for shunt implantation at the age of 62 days revealed that the same symptoms already had occurred. However, they then were not attributed to malignant hyperthermia but interpreted as symptoms of acute herniation. A detailed description of this first anaesthetic is given which again elucidates the problems associated with the abrupt onset of muscular hypertonus in a patient with neurologic disorder; moreover this may well be the first published case report of malignant hyperthermia at the age of just 2 months.

We describe a patient with a pulsatile nasopharyngeal tumour. The diagnostic methods such as endoscopy, computed tomography and NMR are illustrated as well as the anatomical details of encephaloceles. Correct diagnosis prevents complications which can occur as a result of mistaking this lesion for a cyst or adenoids.

The article reports on the ultrasonographic diagnosis of a Meckel-Gruber's syndrome in the 17th week of pregnancy. This malformation syndrome is characterized by the triad: encephalocele, polycystic kidneys, and polydactyly. This congenital disease is compared with malformation syndromes which are distinguishable by differential diagnosis, such as Potter's renofacial syndrome. Thorough ultrasonic examination will be able to detect the severe renal changes as well as the neural tube defects associated with Meckel-Gruber's syndrome. Further diagnostic aids for detecting neural tube defects are, in the first place, the alpha-fetoprotein values obtained from the serum of the mother and/or the amniotic fluid, as well as the determination of acetyl cholinesterase.

With reference to a case of occipital encephalocele associated with iniencephaly diagnosed by ultrasonography in the 20th week of pregnancy, the diagnostic problems of craniospinal defects, factors influencing prognosis and clinical consequences are described. Even when results of biochemical tests are negative, minute neural canal defects can be diagnosed early by ultrasonography. In the authors' opinion an ultrasonographic diagnosis of encephalocele represents an indication for termination of pregnancy, in view of the poor prognosis.

In three pregnant women, sonography and amniocentesis suggested cranial abnormalities of the foetuses. In view of the far-reaching consequences of such a diagnosis, CT was carried out to confirm the diagnosis. It was possible to show the intra-uterine abnormalities and the type of malformation in considerable detail. In one case an encephalocele was demonstrated, in the two others, an anencephalic foetus was shown.

The differential diagnosis of Meckel's syndrome and Ellis-van-Creveld's syndrome with encephalocele is summarized utilizing two own observations. Radiographic criteria are of special importance. In EvCS typical signs of chondrodysplasia are present whereas in MS we found pelvic changes similar to Down's syndrome and striking rhomboid deformities of the tibiae. Clinically relevant is ectodermal dyplasia in EvCS, especially, when cystic kidneys in MS are not palpable.

The Meckel syndrome is caused by an autosomal recessive gene. The three main features are encephalocele, polycystic kidneys and polydactyly. At least two of these should be present to establish the diagnosis. Several minor symptoms are observed in various combinations. The possibility of prenatal diagnosis is explained by a case report: A neural tube defect was diagnosed by ultrasonography and AFP-assessment in amniotic fluid whereas unfortunately the presence of polycystic kidneys was not recognized sonographically. The methods of today are able to detect the polydactyly but failed in this case. One can suggest that the complete prenatal diagnosis of the Meckel syndrome is not essential for clinical management since the presence of one major symptom will lead to legal abortion. The search for Meckel syndrome is important in cases of delivery of a child with multiple malformations, indicating a genetic counseling for each subsequent pregnancy.

If, in twin pregnancy, one twin shows severe malformation, the question arises as to how the other twin can remain unaffected. For the purpose of ensuring the diagnosis in case of suspected neural tube defect, the main diagnostic tools are sonography, biochemical methods of alpha-fetoprotein determination and acetyl cholinesterase activity, as well as cell-morphological identification of so-called "rapidly adhering cells". Parents and obstetricians face a difficult decision when confronted by the possibilities of maintaining pregnancy or of induced abortion after diagnosis of a malformed and a healthy twin. The case of a severe discordant cranial malformation is described on the basis of a case report. The diagnostic and therapeutic problems are discussed.

Today, echography can be considered as routine diagnosis in every pregnancy. Besides the detection of growth retardation very often in early stages of the fetal life malformations can be diagnosed incompatible with postnatal life. Reviewing the cases of the II. Women's Department of Obstetrics and Gynaecology in Vienna within the last 2 years, 13 cases of serious malformations could be detected by regular routine ultrasonic examinations. The echographic appearance of these partially very rare cases is discussed and documented by pictures. The advantage of ultrasonic diagnosis in comparison with other diagnostic procedures in the detection of malformation is discussed. The necessity and importance of regular ultrasonic examinations of all pregnancies as well in respect in the detection of malformations are stressed.

Two typical cases of nasoethmoidal encephaloceles with liquorrhoea, which were operated on by neurosurgeons, are demonstrated. In one case, polyposis of the nose had been operated on twice in childhood, due to the ignorance of the cause with the result of a liquorrhoea. The second patient showed recurrent meningitis with nasal liquorrhoea and turricephaly. The tentative diagnosis of a nasoethmoidal enceplaocele which was suspected by x-ray-tomography and radio-isotopetest could be certified by the aid of detail-angiography. The neurosurgical technique of intracranial, intradural resection of the encephalocele and closure of the liquor fistual with an acrylic flap is shown.