A rare case of congenital choroid plexus carcinoma.

We report a 1-year-old child with the prenatal (week 29 + 5) diagnosis of a brain tumor. The parents were informed about all aspects of prognosis and options concerning termination of pregnancy. The parents opted for an aggressive therapeutic approach after extensive and informative case conferences and counseling. The histopathological diagnosis after partial tumor resection was choroid plexus carcinoma, two weeks thereafter tumor size was progressive. The parents opted for palliative treatment at this stage. The patient died at the age of one year. Ethical aspects have to be actively considered and addressed when caring for children with choroid plexus carcinoma.

Single-incision multiport laparoscopy does not cause more pain than conventional laparoscopy: a prospective evaluation in children undergoing appendectomy.

BACKGROUND: The benefit of single-incision multiport laparoscopy (SIMPL) remains a matter of vivid discussion. For good reason it has been speculated that SIMPL causes more postoperative pain, because a minilaparotomy is required to place the multiport system. We prospectively evaluated postoperative pain scores and requirement of analgesic medication following conventional laparoscopic (CL) versus SIMPL appendectomy in children. METHODS: The access for laparoscopic appendectomy was decided upon the surgeon's preference. Between April and October 2010, individual abdominal pain scores at 8, 16, 24, 48, and 72 hours postoperatively as well as the incidence of umbilical or shoulder pain and the total amount of peri- and postoperative analgesics, operative time, length of hospital stay, and demographics were assessed. Analgesics (paracetamol and/or metamizole, 15 mg/kg body weight) were administered regularly or on inquiry of the patient. Data are presented as means±standard deviation tested at a significance level of P<.05. RESULTS: All operations were laparoscopically completed without conversion or addition of extra ports. Thirty-nine patients (8 SIMPL appendectomy) at a mean age of 12.3±2.4 years and a mean body mass index of 19.16±3.2 kg/m(2) were included. Equal operation times were observed (SIMPL: 68.5±19.9 minutes versus CL: 66.2±19.5 minutes). There were no significant differences for the individual pain scores or the incidence of umbilical and shoulder pain between study groups. The total amount of required analgesic medication was significantly lower after SIMPL appendectomy (SIMPL: 65.73±43.8 mg/kg versus CL: 106.39±46.4 mg/kg, P=.04). CONCLUSION: In summary, the present study substantiates the evidence that SIMPL appendectomy in children and adolescents is not only feasible but also beneficial for the patient without translation into increased postoperative pain. Presently, we are conducting a randomized, blinded study to validate these findings.

Embryology of the distal urethra and external genitals.

Faulty ventral openings of the urethra constitute a broad spectrum of malformations that are subsumed under the term "hypospadia." The normal development of the urethra and the genitals critically depends on the following events: (a) formation of the external genitalia, (b) fate of the cloacal membrane, and (c) formation of the distal urethra. The purpose of this study was to demonstrate these events using microsurgical techniques and scanning electron microscopy in staged rat embryos.

Visual field constriction in children with shunt-treated hydrocephalus.

OBJECT: Many ophthalmological abnormalities are described in conjunction with hydrocephalus. The results of visual field diagnosis remain a matter of further discussion. The aim of this study was to investigate visual field deficits in children with shunt-treated hydrocephalus. METHODS: All children over 6 years of age treated for hydrocephalus at the authors' institute between December 2007 and December 2008 were included in the study. The children underwent an ophthalmological investigation for strabismus and binocular function, ophthalmoscopy, visual acuity, and refraction. The special focus was the visual field diagnosis, which the authors established in all children with cognitive conditions. The investigation was made by using the Goldmann visual field examination (kinetic perimetry). Children with and without visual field defects were compared concerning age at the time of ophthalmological examination, genesis of hydrocephalus, and fronto-occipital horn ratio measured on current CT or MR images. RESULTS: Complete investigations were undertaken in 56 children (24 girls and 32 boys, mean age 15.1 years). The following orthoptic pathological entities were diagnosed: 29 children have a strabismus in 29 cases, 17 of these have an exotropia, 12 an esotropia, 4 children a hypotropia, 2 a hypertropia and 3 children a heterophoria. A nystagmus was found in 10 children. The ocular fundus investigation showed 13 children with an optic nerve atrophy. A visual field diagnosis was possible in 44 of the 56 patients and was incomplete in 12 patients with cognitive deficits or inadequate compliance. In 24 of 42 children there was a concentric visual field constriction between 10° and 50° out of the center. Children with visual field deficits were older than those with a normal visual field (p = 0.051). Nine of 10 children with postmenigitic hydrocephalus had a visual field defect (p = 0.025). In children with visual field defects the fronto-occipital horn ratio was significantly higher (p = 0.013). CONCLUSIONS: The results suggest that children with shunt-treated hydrocephalus have a higher risk of having ophthalmological abnormalities. Visual field deficits are often a problem in these patients. A diagnostic visual field examination can complete the ophthalmological monitoring in patients with hydrocephalus, especially in patients with large ventricles. Children with postmeningitic hydrocephalus should be ophthalmologically monitored more frequently and intensively.

Congenital myelomeningocele - do we have to change our management?

BACKGROUND: Eagerly awaiting the results of the Management of Myelomeningocele Study (MOMS) and with an increasing interest in setting up intrauterine myelomeningocele repair (IUMR), the optimal management of patients suffering from congenital myelomeningocele (MMC) has become a matter of debate again. We performed a cross-sectional study at our referral-center for MMC to determine the outcome for our expectantly managed patients. MATERIALS AND METHODS: A computed chart review at our institution revealed 70 patients suffering from MMC. Forty-three patients were eligible for the study and analyzed further. A retrospective analysis was performed only in patients that underwent MMC repair within the first two days of life and were seen at our outpatient clinic between 2008 and 2009 for a regular multidisciplinary follow-up. Data were collected on: gestational age (GA) and weight at birth, age at shunt placement and shunt status after the first year of life, radiological evidence for Arnold-Chiari malformation (ACM) and tethered cord (TC), need for surgery for TC, bladder function, lower leg function and educational level. Data were compared to published results for IUMR and to studies of historical controls. RESULTS: Patients were born with MMC between 1979 and 2009 and are now 13.3 ± 8.9 (mean ± SD) years of age. At birth, mean GA was 37.8 ± 2.3 weeks and mean weight was 2921.3 ± 760.3 g, both significantly higher than in IUMR patients. Shunt placement in our cohort was required in 69.8% at a mean age of 16.0 ± 10.7 days, which was less frequent than for historical controls. Amongst our cohort, radiological observations showed 57.1% had ACM II and 41.9% had TC. Only two of our patients underwent a surgical correction for TC. Clean intermittent catheterization was performed in 69.7% of our patients, 56.4% were (assisted) walkers and 64.1% attended regular classes, both comparable to historical controls. CONCLUSIONS: With a close and interdisciplinary management by pediatric surgeons, neurologists and urologists, the long-term outcome of patients suffering from MMC can currently be considered satisfactory. With respect to the known drawbacks of fetal interventions for mother and child, especially preterm delivery, the results of the MOMS trial should be awaited with caution before proceeding with a complex intervention like IUMR.

The testicular descent in the rat: a scanning electron microscopic study.

PURPOSE: Numerous researchers studied the morphology of testicular descent including the possible function of gubernaculum. However, a clear illustration of this process is still missing. The aim of this study was to illustrate testicular descent using scanning electron microscopy (SEM) in a rat model. METHODS: The abdomen of rat fetuses between gestational day (E) 15 and E 22 and newborns at postnatal day (D) 0 and D 1.5 was opened by microsurgery. Standard preparation for SEM was carried out. The position of the testis and gubernaculum testis was documented. RESULTS: The gubernaculum was obvious in male rat embryos at E 17.5. In a first phase (E 16-E 21) the testis moved from cranio-lateral and dorsal to caudo-medial and ventral, while clear signs of an active role of the gubernaculum were missing. In a second phase (E 22-D 1.5) the processus vaginalis peritonei (PVP) developed, while the conus of the gubernaculum disappeared, after which, the testis moved out of the abdominal cavity and entered the PVP. CONCLUSION: In our study, we could not specify the role of gubernaculum for testicular descent. However, our data showed that the testis lay intraperitoneal throughout the descensus testis.

Diagnostic value of S100B and neuron-specific enolase in mild pediatric traumatic brain injury.

OBJECT: During recent years, several biomarkers have been introduced for use in the diagnosis of traumatic brain injury (TBI). The primary objective of this investigation was to determine if S100B (or S100 calcium-binding protein B) and neuron-specific enolase (NSE) serum concentrations can effectively be used to discriminate between symptomatic and asymptomatic children with minor head trauma. METHODS: The authors conducted a prospective clinical study that involved patients age 6 months to 15 years who had sustained minor head trauma. Children with concomitant extracranial injuries were excluded. Blood samples were obtained within 6 hours of injury to measure S100B and NSE levels in serum. The authors defined 2 diagnostic groups: a mild TBI group (patients with Glasgow Coma Scale [GCS] scores of 13-15) in whom there were clinical signs of concussion (short loss of consciousness, amnesia, nausea, vomiting, somnolence, headache, dizziness, or impaired vision) and a head contusion group (patients with a GCS score of 15) in whom symptoms were absent. Both S100B and NSE concentrations were compared between the 2 groups. Secondary end points were defined as follows: correlation of S100B/NSE and a) the presence of scalp lacerations, b) GCS score, c) age, and d) correlation between S100B and NSE. RESULTS: One hundred forty-eight patients were enrolled (53 in the contusion group, 95 in the mild TBI group). After adjusting for differences in age and time of injury to blood sample withdrawal, there was no significant difference in S100B or NSE between patients in the 2 groups. Scalp lacerations and GCS score had no affect on posttraumatic S100B or NSE concentrations. The correlation between S100B and NSE was significant. Both markers showed a significant negative correlation with age. CONCLUSIONS: The authors demonstrated that S100B and NSE do not discriminate between symptomatic and asymptomatic children with minor head injury. There seem to be limitations in marker sensitivity when investigating pediatric patients with mild TBI.

Experiences with a gravity-assisted valve in hydrocephalic children. Clinical article.

OBJECT: Over the past decade, a gravity-assisted valve (GAV) has become a standard device in many European pediatric hydrocephalus centers. Because past comparative clinical outcome studies on valve design have not included any GAV, the authors in this trial evaluated the early results of GAV applications in a pediatric population. METHODS: For a minimum of 2 years the authors monitored 169 of 182 hydrocephalic children who received a pediatric GAV at their first CSF shunt insertion (61.5%) or as a substitute for any differential pressure valve (38.5%) at 1 of 7 European pediatric hydrocephalus centers. Outcomes were categorized as valve survival (primary outcome) or shunt survival (secondary outcome). The end point was defined as valve explantation. RESULTS: Within a follow-up period of 2 years, the valve remained functional in 130 (76.9%) of 169 patients. One hundred eight of these patients (63.9%) had an uncomplicated clinical course without any subsequent surgery, and 22 (13%) were submitted to a valve-preserving catheter revision without any further complications during the follow-up period. Thirty-nine patients (23.1%) reached an end point of valve explantation: 13 valve failures from infection (7.7%), 8 (4.7%) from overdrainage, and 18 (10.6%) from underdrainage. CONCLUSIONS: Compared with nongravitational shunt designs, a GAV does not substantially affect the early complication rate. Valve-preserving shunt revisions do not increase the risk of subsequent valve failure and therefore should not be defined as an end point in studies on valve design. A significant impact of any well-established valve design on the early complication rate in shunt surgery is not supported by any current data; therefore, this correlation should be dismissed. As overdrainage-related complications have been shown to occur late, the presumed advantages of a pediatric GAV remain to be shown in a long-term study.

Surgical anatomy of large retroperitoneal teratomas in infants: report of two cases.

We report on two infants of 11 and 12 months of age, respectively, with large solid-cystic retroperitoneal tumors. Complete resection was achieved, and both children are doing well over a follow-up of more than 17 months. The presented paper focuses on surgical anatomy of this very rare type of tumor. Teratomas were located mainly within the upper abdominal cavity, and both tumors displaced the pancreas in an anterior position. The following anatomical features were observed (1) displacement of surrounding organs, (2) deformation and elongation of large retroperitoneal vessels, (3) fibrous incorporation of large vessels by the tumor pseudocapsule, (4) wrapping of anterior aortic branches by lobes and fingers of the tumor.