Pediatric diseases in Juan Carreño de Miranda's paintings.

Pediatric obesity has become a widespread problem of health in developed countries. Overweight in the pediatric population obeys to a variety of causes. A few of Carreño de Miranda's paintings show pathological conditions occurring in children of his epoch. We briefly illustrate the significance of Carreño's paintings that portray some of these diseases. Two of his best-known works constitute an artistic representation of childhood obesity.

Craniosynostosis in Kabuki syndrome.

Niikawa-Kuroki, or Kabuki syndrome (KS), is characterized by distinctive facial features, skeletal anomalies, persisting fingertip pads with dermatoglyphic abnormalities, short stature, and mental retardation. Neurological manifestations and CNS anomalies have been described in some patients with this condition. However, craniosynostosis has been documented in only 4 patients with KS who did not undergo operations. The authors report a case of KS with unicoronal synostosis that constitutes the first documented instance of a patient with this syndrome submitted to surgery. Previous reported instances of craniosynostosis occurring in KS are briefly reviewed. Although rarely documented, craniosynostosis might represent a relatively frequent feature of this syndrome. Kabuki syndrome should be considered at the time of evaluating children with craniosynostosis. The diagnosis of KS can be suspected from the patients' characteristic facial features. Kabuki syndrome appears to be an underdiagnosed condition in the craniosynostosis population. Given that most patients with this syndrome suffer from only mild to moderate mental retardation, surgical correction can be considered in instances of KS with craniosynostosis.

Foreign body reactions causing spinal cord tethering: a case-based update.

BACKGROUND: Patients operated on for myelomeningocele (MMC) and lipomeningocele (LMC) can suffer from late functional worsening that, in many cases, is due to spinal cord tethering by the post-repair scarring process. ILLUSTRATIVE CASES: In this case-based update, we report two patients operated on for MMC and LMC, respectively, who presented the clinical manifestations of spinal cord tethering, which we attributed to severe foreign body reactions to the materials used at their primary back surgery. In the first case, the cause of the tethering was an intense fibrotic scar around the silk suture used at the initial MMC repair, while in the second one, it was due to a fibrotic mesh containing the lyophilized dural graft implanted for LMC surgery. After a search of the current literature, we were unable to find cases of spinal cord tethering related to excessive scarring after dural repair with foreign materials used for the surgical correction of spinal dysraphism. DISCUSSION AND CONCLUSIONS: The pathogenesis of spinal cord tethering in our instances closely relate with the histopathologically observed intense foreign body reaction around the materials used at the primary back surgery. We recommend avoiding as much as possible the use of foreign materials during the initial repair of spinal dysraphism.

Management of neonatal hydrocephalus: feasibility of use and safety of two programmable (Sophy and Polaris) valves.

BACKGROUND: Neonates represent a unique group of pediatric patients with special peculiarities. Hydrocephalus valves have not always been designed to meet the requirements of these small children. Few series have addressed the problem of cerebrospinal fluid shunting in newborn babies. OBJECTIVES: We aimed (1) to evaluate the feasibility of the use of two programmable valves (Sophy and Polaris) in hydrocephalic neonates and (2) to ascertain complications and safety issues arising from their use. MATERIALS AND METHODS: We performed a prospective study of 100 consecutive preterm and term babies (<2 months of age) given a programmable valve. Valves' settings were readjusted at different pressure levels as required. Outcomes were obtained from the records of our Outpatient Clinic. RESULTS: The study group was formed by 60 term and 40 preterm infants (average weight 2,440 g, mean age of 25 days). Mean follow-up was 55 months. Only one fifth deaths was shunt-related. In 70 babies, no complications occurred, and hydrocephalus was successfully controlled. Proximal catheter obstruction presented in 20% and infection in 5% of cases. Several external adjustments of the valves apparently avoided several surgical shunt revisions. CONCLUSIONS: (1) Both programmable valves (Sophy and Polaris) can be safely used for treatment of neonatal hydrocephalus, introducing some technical modifications. (2) Both valves are comparable to other shunts with regard to indications, performance, and safety. (3) The possibility of modifying their working pressure seems to constitute their main advantage. Prevention of late overdrainage syndromes with these valves needs a longer follow-up.

Shunt-related craniocerebral disproportion: treatment with cranial vault expanding procedures.

Two patients with intracranial arachnoid cysts, one with myelomeningocele-hydrocephalus and the other with a subdural fluid collection, were given a cerebrospinal (CSF) extracranial shunt. All four patients developed features of CSF overdrainage following shunting and were treated by cranial vault expanding procedures. Before undergoing decompressive craniotomy, the patients were treated by a variety of procedures, including changing of obstructed ventricular catheters (n=4), insertion or upgrading of programmable valves (n=3), and foramen magnum decompression (n=1). Clinical manifestations of these four patients were attributed to craniocerebral disproportion caused by chronic and progressive skull changes due to dampening of the CSF pulse pressure, which is necessary for maintaining normal cranial growth. On the basis of our previous experience with expanding craniotomies in cases of minimal forms of craniosynostosis, we treated these patients with bilateral parietal craniotomies, with satisfactory results. In conclusion, biparietal decompressive craniotomy constitutes a useful and safe procedure for relieving the clinical manifestations of some CSF overdrainage syndromes, especially in cases with slit-ventricle syndrome and craniocerebral disproportion that prove to be refractory to simpler management procedures.

Midline cutaneous lumbosacral lesions: not always a sign of occult spinal dysraphism.

OBJECTIVE: The authors report two unusual cases of extraspinal midline lumbosacral lesions that resembled the usual skin markers of occult spina bifida. PATIENTS AND METHODS: The pathological diagnosis of the masses was plexiform neurofibroma and mastocytoma, respectively. RESULTS: Some lesions, as those we are reporting, although occurring at the lumbosacral midline, do not necessarily belong to occult spinal dysraphism, as was initially suspected. Plexiform neurofibroma and skin mastocytoma are very rare indeed in this spinal location. CONCLUSION: The two cases represent an important addition to the differential diagnosis of a congenital dorsal midline mass.

Hydrocephalus in intraspinal dermoids and dermal sinuses: the spectrum of an uncommon association in children.

BACKGROUND: Hydrocephalus associated with intraspinal tumors constitutes a well-documented occurrence. The accepted mechanisms for this association seem to be well established. On the contrary, hydrocephalus in the context of intraspinal dermoids has been rarely recognized and its pathogenetic mechanism appears to be different. PATIENTS AND METHODS: The authors report four pediatric cases of spinal dermoid tumors and dermal sinuses that developed hydrocephalus during the evolution of these congenital lesions of ectodermal origin. DISCUSSION: In two children, the mechanism leading to the development of hydrocephalus consisted of leptomeningeal inflammation due to bacterial meningitis or to spillage of dermoid cyst contents in the cerebrospinal fluid spaces. We hypothesize that ventricular dilatation in the other two might be the result of chemical meningitis occurring during intrauterine life. Two cases developed ventriculomegaly prior to the diagnosis of their spinal cord disease. CONCLUSIONS: The unusual evolution of these cases suggests that neuroimaging studies that include the spine should be performed in cases of childhood "unexplained hydrocephalus".

Rhabdoid meningioma: a new subtype of malignant meningioma also apt to occur in children.

CASE REPORT: The case of a 14-year-old girl who presented with a 2-week history of raised intracranial pressure is reported. A left frontal extra-axial tumor was totally removed, whose histopathologic diagnosis was rhabdoid meningioma (RM). DISCUSSION: Rhabdoid meningiomas constitute a special malignant phenotype of meningioma that has been recently included in the WHO classification of tumors of the nervous system. Usually, RMs affect middle-aged and elderly individuals. We report the fourth case of a RM occurring in a child to illustrate that the diagnosis of this tumor subtype, given its prognostic implications, must also be considered in pediatric patients.

Autologous tissues for dural grafting in children: a report of 56 cases.

BACKGROUND: Diverse materials have been used for dural closure, including grafts of cadaveric origin. Some 168 cases of Creutzfeldt-Jakob disease have been reported following the implant of human dura mater. A few publications have addressed the use of dural grafts in children, but none has analyzed the feasibility of autologous tissues for this purpose. AIM: Since 1994, the authors have utilized autologous tissues for dural grafting in children undergoing neurosurgical procedures, aimed at studying the feasibility of its use in this age group. MATERIAL AND METHODS: We studied 56 children submitted to neurosurgical procedures who underwent an implant of a patch of autologous tissue for dural closure. Epidemiological, clinical, and neuroimaging data were processed using statistical methods. RESULTS: The study group was composed of 29 boys and 27 girls, with ages ranging from 1 month to 17 years (mean 7.6 years). The procedures corresponded to lesions at the posterior cranial fossa (n = 41), supratentorial cranial compartment (n = 12), and spine (n = 3). Seventy-five percent of the children had no complication. Four patients had adverse effects unrelated to dural grafting, while ten children had complications that might be associated with it. The most frequent complication was pseudomeningocele (n = 6), followed by infection (n = 3) and CSF leak (n = 1). Although hydrocephalus seemed to have an influence on the occurrence of complications, their incidence in patients without hydrocephalus did not differ statistically. CONCLUSIONS: Autologous grafts of fascia or pericranium can be safely used for dural reconstruction in children. Given the reported complications associated with the use of foreign materials for duraplasty, the authors recommend the use of autologous tissues for dural repair, reserving other types of dural grafts for cases in which its usage is unavoidable.

Positional skull deformities in children: skull deformation without synostosis.

BACKGROUND: Patients with craniosynostosis are readily diagnosed by clinical and neuroimaging findings. Surgical treatment is indicated for preventing neurological deficits and for correcting esthetically unacceptable head deformities. In recent years, we have witnessed a progressive number of neurosurgical consultations for abnormal head shapes unrelated to premature fusion of the cranial sutures, especially of positional plagiocephaly. There have been descriptions of abnormalities in the cerebrospinal fluid (CSF) spaces in children with craniosynostosis. OBJECTIVES: The aim of the present study was to investigate the role of the changes of the CSF spaces in the development of positional skull deformities in children. PATIENTS AND METHODS: The authors reviewed demographic, clinical, and neuroimaging data of 23 patients assessed for some form of nonsynostotic skull deformity (group A). The results were compared with those of a simultaneous group of nine infants diagnosed with benign extracerebral collections of fluid (group B). RESULTS: The study group was composed of 11 boys and 12 girls, aged 3 years or younger. Seventeen children had plagiocephaly, four scaphocephaly, and two brachycephaly. Sixteen children (15 with plagiocephaly and 1 with brachycephaly) exhibited enlarged subarachnoid CSF spaces. In group B, the boy/girl ratio was of 7:2. Infants in group A presented at an older age (mean 12.7 months) than group B (mean age at presentation of 7.17 months). Children with benign extracerebral fluid collections were born with a head circumference (HC) greater than infants with positional skull deformities (p=0.005). The percentile of the children's HC at consultation was also larger for children of group B (p=0.03). The form of clinical presentation differed between the two groups. Most infants of group A were seen because of a type of head deformity, and children in group B were studied for macrocephaly. Long-term follow-up assessment showed better outcomes for patients in group B than for children of group A in regard to regression of initial symptoms (p=0.03). CONCLUSIONS: Most positional head deformities appear to be related with the children's positioning for sleeping. We have not confirmed macrocephaly as a contributing factor for positional deformities. The distribution of extracerebral CSF and the presence of abnormal collections of fluid in children with positional head deformities do not seem to be related with the findings of pericerebral CSF encountered in children with benign extracerebral collections of fluid. In our view, brain pulsations, transmitted to these accumulations of CSF, play an important part in the development of the infants' skull deformities (p=0.02). The findings of enlarged CSF spaces in children with nonsynostotic skull deformation constituted an age-related event, as these collections tended to disappear as the children grew older (p=0.04).

Thalamic tumors: clinical presentation.

INTRODUCTION: Thalamic tumors (TTs) account for approximately 1% of all intracranial neoplasms. TTs are seen predominantly in children and young adults. Most childhood neoplasms in this location are of glial lineage, a large proportion being low-grade tumors. The evolution of symptoms before diagnosis is characteristically shorter in children than in adults. CLINICAL PRESENTATION: Clinical features of TTs reflect the pressure of the mass on the cerebrospinal fluid pathways, the pyramidal tracts, the thalamic nuclei and the optic radiations. In this paper, we review the clinical data of 20 children with TTs seen at our hospital and compare the findings with those in the current literature. An acute presentation is not rare and was encountered in 20% of our patients, although most individuals had a subacute or slow evolution. Symptoms and signs of raised intracranial pressure (65%), motor deficits (40%), and seizures (35%), alone or in combination, were the most frequent manifestations of TTs. Behavioral and mental changes are not uncommon in TTs and were found in 25% of our patients. Involuntary movements (10%) and the classic thalamic syndrome (5%) were quite exceptional. Infants and young children with TTs may present with macrocephaly, psychomotor delay, and failing vision or disorders of ocular movements.