Treatment of papuloerythroderma of Ofuji with Re-PUVA: a case report and review of the therapy.

Papuloerythroderma of Ofuji (PEO) is a disease of elderly men, characterized by intensely pruritic and widespread, red, flat-topped papules with sparing of the body folds and creases (the so-called 'deck-chair' sign). The etiopathogenesis of the disease remains unknown. Psoralen plus UVA (PUVA), topical and systemic corticosteroids, etretinate, cyclosporin and interferon are the main approaches in the treatment of this rare disease. A case of PEO in a 60-year-old man who responded to retinoid plus PUVA (Re-PUVA) treatment is reported here and a review of the therapy with other relevant cases is presented.

Neuroendoscopic biopsy for intraventricular tumors.

Neuroendoscopy has been shown to be an effective and minimally invasive method in the management of intraventricular tumors. Endoscopic tumor biopsy with or without additional endoscopic procedures such as third ventriculostomy and septostomy can be performed at the same session. Neuroendoscopic tumor biopsy was performed in 18 patients in our department. Their ages ranged from 2 to 65 years (median 12 years); only two of them were adult. Location of the tumors were as follows: pineal region in 7, hypothalamus and 3rd ventricle in 4, lateral ventricle in 4, thalamus in 2, and tectal in 1 patient. All procedures were performed under general anesthesia using rigid neuroendoscopes. Cerebrospinal fluid (CSF) was collected at the beginning of the procedure for cytological analysis and for pineal tumor markers. Biopsy forceps were used to obtain tissue from the lesion. The third ventriculostomy was performed in all patients with a pineal tumor, in addition to the tumor biopsy. The pathological examinations revealed a low-grade astrocytoma in 6 patients, anaplastic astrocytoma in 3, germinoma in 3, pineocytoma in 1, pineoblastoma in 2, glioblastoma multiforme in 1 and granulomatous lesion in 1. Subsequent mode of treatment such as radiation therapy, chemotherapy or radical surgery was determined on the basis of pathological diagnosis. Neuroendoscopic tumor biopsy is a less invasive method than open surgery and has some advantages such as treating the hydrocephalus at the same surgical session and the availability of CSF cytology. Neuroendoscopic techniques should be considered in selected patients.

Is meningocele really an isolated lesion?

OBJECT: We designed this study to elucidate the associated occult spinal lesions in patients with simple dorsal meningocele. METHODS: The study population was comprised of two groups. Group I comprised newly diagnosed patients with dorsal spinal meningocele, and group II comprised patients who had had surgery for meningocele and presented with progressive neurological deficits. Magnetic resonance imaging (MRI) scans of the whole spinal column were done. The associated spinal cord malformations were also treated at the same operation. There were 14 boys and 8 girls, with an age range from birth to 4 years (mean 3.9 months), in group I. Of 20 patients (90%) with associated spinal lesions, 6 had more than one lesion, excluding hydromyelia. Group II was made up of 6 patients who had been previously operated on for a meningocele and who presented with tethered cord syndrome. These were 4 boys and 2 girls, who ranged in age from 4 to 10 years (mean 6 years). RESULTS: The level of the conus terminalis was lower than L3 in all patients. The other findings on MRI, besides low conus, were as follows: tight filum, split cord malformation, epidermoid, dorsal lipoma and hydromyelia. CONCLUSIONS: Meningocele frequently camouflages a second, occult, spinal lesion. MRI of the whole spinal column should be performed. An intradural exploration performed with a microneurosurgical technique is needed to detect the fibrous bands that may lead to spinal cord tethering and to release the entrapped nerve roots. The other associated spinal anomalies should be operated on during the same operation.

Growing skull fractures (craniocerebral erosion).

The incidence of growing skull fractures ranges from less than 0.05% to 1.6%. We reviewed 22 growing skull fracture patients retrospectively. There were 15 boys and seven girls ranging in age from newborn to 6 years (mean: 12.4 months) at the time of injury. Falling was the most frequent cause of injury. In total, 17 patients presented with a scalp mass. The scalp was sunken over the bone defect in three patients. Other symptoms and signs were seizure in five patients, hemiparesis in four, recurrent meningitis in one, and pulsatile exophthalmus in one. The most common location was the parietal region. The extent of dural defect was always greater than that of bony defect, except in one case that had been previously shunted for hydrocephalus. In another patient with a growing fracture in the posterior cranial fossa, the dural edges could not be exposed, although a wide craniotomy was performed. Therefore, a cystoperitoneal shunt was inserted. Gliotic tissue was present in all the patients. Cyst or cystic lesions were observed in only nine patients, duraplasty was performed in 21, 16 were neurologically intact, and six had minor deficits. All patients under the age of 3 years with a diastatic skull fracture should be closely followed up. A sustaining diastatic fracture and brain herniation through the skull defect shown on CT or MRI imply a growing skull fracture.

Complications of subduroperitoneal shunting.

OBJECTS: Subduroperitoneal (SP) shunts have been widely used in the management of pediatric subdural fluid collections. METHODS: We retrospectively reviewed the complications of SP shunting in 73 boys and 24 girls, who ranged in age from 1 to 180 months (median 7 months). Subdural fluid collection was bilateral in 75 and unilateral in 22 patients. The most common complication was shunt obstruction (13 patients). Shunt migration was seen in 8 patients. Migration occurred only with unishunts without a reservoir and with peritoneal catheters. However, the shunts with a reservoir or flushing valve led to skin necrosis in 4 patients (P=0.003). Unilateral drainage though bilateral collections were present, infection, bowel perforation, and ileus occurred in 5, 4, 1 and 1 patients respectively. CONCLUSIONS: These SP shunt complications, some of which are avoidable, should be kept in mind.

Intraosseous neurinoma of the parietal bone.

Intraosseous neurinoma is very uncommon tumor. Its location in the skull is extremely rare. A 4-year-old boy presented with a lump in the right occipital region. Computed tomography revealed a soft tissue mass with bony erosion in the right occipital bone. The solid, nontender, and immobile mass was totally removed. The pathological examination showed that it was an intraosseous neurinoma. Total resection of intraosseous neurinomas is sufficient treatment and is not followed by recurrence.

Intracranial meningeal tumours in childhood: a clinicopathologic study including MIB-1 immunohistochemistry.

Primary tumours of the meninges with a relatively high tendency for malignant behaviour are uncommon in childhood. This study concerns 18 cases of meningeal tumours in children under the age of 16, of which 13 were meningiomas and five were other tumours arising in the meninges. Meningiomas showed a preponderance in females as in adult series, and the majority were supratentorial in localisation. The percentage of meningeal tumours and meningiomas among all brain tumours in our centre were 3.72% and 2.69%, respectively. Four out of 13 meningiomas were fibroblastic, four were transitional, one was meningothelial, two were psammomatous and two were papillary meningiomas. Seven (38.8%) out of 18 tumours showed anaplastic features, including two papillary meningiomas, two hemangiopericytomas, one mesenchymal chondrosarcoma, one pleomorphic sarcoma and one anaplastic meningeal tumour. Papillary meningiomas with hemangiopericytoma-like solid areas were seen frequently in our cases (15.3%). Meningoangiomatosis was associated with two meningeal tumours. MIB1 (Ki-67) labelling indices (LIs) ranged between 0% and 13.6% (mean 1.83%) in benign, and between 1% and 20% (mean 7.2%) in malignant tumour, including papillary meningiomas. Mean MIB-1 LIs were 5.61% and 1.14% in non-recurrent and recurrent cases, respectively. MIB-1 LIs showed significant differences between benign and malignant meningeal tumours but no significant correlation either with prognosis or recurrence. Despite the fact that brain tumours are among the most common neoplasms of childhood, meningeal tumours are rare lesions, accounting for less than 2% of published series of intracranial neoplasms in childhood [5, 8, 18, 24, 30, 32]. It has been suggested that the clinical and pathological characteristics of meningiomas in this age group differ from those of adults [14, 18, 24, 45]. Besides meningiomas, there are a few reports of other meningeal tumours in childhood and difficulties in differential diagnosis may arise within this group, especially in anaplastic tumours [11, 13, 32, 44, 46]. One of the major problems in meningiomas and some tumours arising in the meninges is the discordance that arises between the histologic appearance of the tumour and behaviour [4]. Several studies have attempted to determine the proliferation potential of meningiomas, including immunohistochemical labelling with monoclonal antibodies to Ki-67, proliferating cell nuclear antigen (PCNA), and bromodeoxyuridine (BUdR); flow cytometric DNA analysis; or argyrophilic nucleolar organizer regions (AgNORs) counting [9, 10, 15, 19, 22, 26, 31, 35, 53]. The studies concerning proliferation markers have contradictory results [9, 10, 15, 26, 31, 42, 53]. MIB-1 detects the same or a similar epitope as the original antibody Ki-67 and reacts with a proliferation associated antigen expressed in all active parts of the cell cycle, G1, S, G2 and M (mitosis), but not in the G0 or quiescent phases [7]. In this study we examined the clinicopathological characteristics and MIB1 values of 18 meningeal tumours in children under the age of 16 years within the last 25 years (from 1970 to 1995).

Complications in craniofacial surgery: the Turkish experience.

The records of 71 patients who underwent 75 consecutive craniofacial procedures involving a transcranial component have been analyzed retrospectively to assess the incidence and type of complications encountered during the 14-year period of 1985 to 1998 at Ege University Hospital, Department of Plastic and Reconstructive Surgery. The results were compared with those of similar reports from other craniofacial centers. Operations were classified into six groups according to their type to evaluate effectively their complication rates. Complications were also graded in terms of their severity. Serious complications were seen in 12 patients (16.8%), whereas life-threatening complications were seen in 6 patients (8, 4). The mortality rate was 2.7%. It is determined that complication incidence was notably higher in the group that underwent monobloc frontofacial advancement. A decrease in the rate of serious complications occurred with increased experience with the surgery. The results of our study indicate that although craniofacial surgery carries an inherent risk for significant complications, the risk can be minimized and the rate of mortality and major complications kept to an acceptable level by a careful and experienced craniofacial team.

A modified fixation method in supraorbital bar advancement.

In almost all congenital craniofacial deformity reconstructions there is a need to advance the supraorbital bar. This bar, which is fixed by several techniques, should be firm enough to minimize a relapse. In this paper a new modification during osteotomy of the supraorbital bar is presented that provides firmness and prevents relapse even without grafts. The last 15 patients with craniofacial anomalies were operated with this modification. At the stage of the supraorbital bar osteotomy, bilateral small triangles are created at the end of the bar. Then, on the lateral orbital rim, two small notches are created in which to place these triangles. By fixating these triangles to the notches, sliding of the bar and subsequent relapse is prevented, and also the fixation provided is much more rigid.

Management of frontoethmoidal (sincipital) encephalocele.

Frontoethmoidal encephaloceles are congenital malformations that cause complex deformities in the frontal, orbital, and nasal regions. As the term implies, with frontoethmoidal encephaloceles, intracranial material has herniated through the dural and skull defect. In this report, 21 patients with frontoethmoidal encephalocele operated by a craniofacial team are presented, and accompanying anomalies, results, and complications are discussed.

Cerebral hydatid cysts in children.

A parasitic tapeworm, called Taneia Echinococcus, causes hydatid disease. Hydatid disease is endemic in sheep and cattle-raising areas of the world. Hydatid disease of the central nervous system constitutes 2%-3% of all reported cases of hydatid cysts. In our institution, 23 children underwent surgery for intracranial hydatid cysts between 1979 and 1995. There were 14 boys and 9 girls, aged between 3 to 16 years (mean 8.8 years). Signs and symptoms were related to the site and size of the cyst. Headache and vomiting due to increased intracranial pressure were the most common presenting symptoms. A round cystic lesion without perifocal edema and rim enhancement is the characteristic appearance on a computed tomography (CT) scan. A magnetic resonance image visualizes cyst location better than CT. Associated systemic hydatidosis in four of our patients involved kidney, liver, lung, and liver and lung, respectively. Intact cyst removal was achieved in 14 patients. In three patients with infected or inflamed hydatid disease, the ruptured cyst capsule was totally resected. Aspiration and extirpation were performed in only one patient. Eleven patients were treated with chemotherapeutic agents such as albendazole or mebendazole due to cyst rupture during surgery or associated systemic hydatid disease. Hydatid disease can also be seen in Western countries because of travel and migration. Cerebral hydatid cyst should be kept in mind for the differential diagnosis of cystic lesions.

Intraorbital osteoma and surgical strategy.

The most common tumoral lesion of the bony orbital region is osteoma. It is an infrequent and benign tumor, and generally attacks the craniofacial skeleton, but intraorbital involvement is extremely rare. After necessary radiologic examinations (radiographs and computed tomography scanning), surgery should be planned according to the tumor's localization. In the case presented here, osteoma originated mainly from the medial orbital wall. Therefore, for better surgical exposure, extra- and intracranial approaches were planned and carried out. The mass was removed successfully. At the 3-year follow-up, no recurrence was shown.

Outcome of patients with meningomyelocele: the Ege University experience.

The medical records of 190 patients with meningomyelocele operated on between 1979 and 1993 were reviewed. In 65 patients, psychometric tests were performed. The Denver Developmental Screening Test and the Wechsler Intelligence Scale for Children, Revised (WISC-R) were used in children under and over 6 years old, respectively. There were 82 boys (43.2%) and 108 girls (56.8%). The lumbar region was the site of the meningomyelocele in 113 patients (59.5%). Patients with cervical and sacral meningomyelocele had a higher rate of almost normal motor function than those with meningomyelocele at other levels (P=0.000). Only 36 (21.7%) of 166 patients followed up by us did not have hydrocephalus. We also noted that the higher the location of the meningomyelocele, the greater the control of both sphincters (P=0.014). Fifty-four percent of the hydrocephalic patients had a normal development or IQ. Psychometric tests were normal in 76% of those without hydrocephalus. Twenty-four patients were lost to follow-up. The follow-up of the remaining 166 patients ranged from 1 month to 180 months (mean 60.2 months). Fifty-three patients (32%) died, central nervous system infection being the most common cause of death (44%). The management of children with meningomyelocele needs a team approach.

Split spinal cord malformations in children.

OBJECT: The authors reviewed and analyzed information on 74 patients with split spinal cord malformations (SSCMs) treated between January 1, 1980 and December 31, 1996 at their institution with the aim of defining and classifying the malformations according to the method of Pang, et al. METHODS: Computerized tomography myelography was superior to other radiological tools in defining the type of SSCM. There were 46 girls (62%) and 28 boys (38%) ranging in age from less than 1 day to 12 years (mean 33.08 months). The mean age (43.2 months) of the patients who exhibited neurological deficits and orthopedic deformities was significantly older than those (8.2 months) without deficits (p = 0.003). Fifty-two patients had a single Type I and 18 patients a single Type II SSCM; four patients had composite SSCMs. Sixty-two patients had at least one associated spinal lesion that could lead to spinal cord tethering. After surgery, the majority of the patients remained stable and clinical improvement was observed in 18 patients. CONCLUSIONS: The classification of SSCMs proposed by Pang, et al., will eliminate the current chaos in terminology. In all SSCMs, either a rigid or a fibrous septum was found to transfix the spinal cord. There was at least one unrelated lesion that caused tethering of the spinal cord in 85% of the patients. The risk of neurological deficits resulting from SSCMs increases with the age of the patient; therefore, all patients should be surgically treated when diagnosed, especially before the development of orthopedic and neurological manifestations.

Continuous external subdural drainage in the management of infantile subdural collections: a prospective study.

Continuous external subdural drainage (CESD) was suggested as a treatment step to be inserted prior to SP shunting, primarily because it makes it possible to avoid shunt placement in a significant number of patients. Thirty-three patients with symptomatic chronic subdural collection confirmed by computed tomography were included in this study. Unilateral CESD was performed in all cases, using a lumbar drainage set. The drains were left in place for no more than 10 days. A subduroperitoneal (SP) shunt was inserted in those patients in whom re-accumulation of the subdural collection had occurred. Of 33 patients, 17 were definitively treated by CESD and 16 subsequently needed an SP shunt. The cost of treatment with CESD was just less than half the cost of treatment with SP shunting. CESD can be used as a step before SP shunting in the management of chronic infantile subdural collections, since it is effective without further treatment in half the patients and safer than subdural tapping.

Immunoglobulin prophylaxis in shunt infections: a prospective randomized study.

Cerebrospinal fluid shunt infection is serious and one of the most frequent complications of shunt implantation. Age has been one of the most significant host factors for the development of shunt infections. A relative deficiency of the immune response against bacteria in infants could partly explain the higher infection rate in the very young patients. This prospective-randomized study was conducted in two groups: group A (immunoglobulin group) and group B (control group). There were 30 patients in each group. The patients in group A received intravenous immunoglobulin (Sandoglobulin) at a dose of 1 g/kg in the night before surgery. Each patient was followed up to 6 months. No infection was seen in group A. In group B, infection rate per procedure were 5.1% (P = 0.494) and 6.6% (P = 0.492), respectively. Intravenous immunoglobulin prophylaxis in infants seems to reduce the shunt infections.

Cerebrospinal fluid shunt complications.

We report our experience with cerebrospinal fluid shunt procedures performed on 306 patients between 1983 and 1993. Patients were between the ages of one day and 15 years (average 14.9 months) on admission. Three hundred and thirty-six shunt placements and 274 revisions were done. The first complication occurred in the first postoperative month in 52 patients and within the first six months following surgery in 97 patients. Age was determined as a statistically significant factor in only infection and the slit ventricle syndrome (SVS). Shunt types and systems were not significant factors causing complications. The level of consciousness of the patients at the time of surgery influenced the rate of complications; patients with impaired consciousness at the time of surgery had higher complication rates than those operated on in a normal state of consciousness (41% and 8.5%, respectively).

Cerebellar mutism: report of two unusual cases and review of the literature.

Mutism is not a common condition following cerebellar damage. Mutism following posterior cranial fossa surgery was first reported by Rekate et al. and Yonemasu in 1985. Since then, many case reports of mutism have appeared in the English literature. Very few cases developed mutism following brain stem surgery. Although mutism has been described in patients with head injury, only one case of mutism caused by a cerebellar injury has been reported, to our knowledge. We report on two patients in which the cerebellar mutism following a radical excision of an exophytic brain stem glioma and cerebellar injury developed. We reviewed the relevant literature and discussed the mechanism of cerebellar mutism.

Late results of hypertelorism correction.

The late results of patients who were operated on for the correction of hypertelorism were evaluated. Follow-up ranged between 1 and 11 years (mean, 4.3 years). The mean interorbital distance was 32.9 mm preoperatively and 26.7 mm postoperatively. The significant decrease in the interorbital distance and the absence of serious postoperative complications revealed that craniofacial surgery for the correction of hypertelorism was an effective and reliable procedure.