Elena Caresta

Improving ischaemic skin revascularisation by nerve growth factor in a child with crush syndrome

Nerve growth factor (NGF) is the first described neurotrophin that stimulates the growth and differentiation of nerve cells and promotes skin and peripheral tissue regeneration. Recent studies suggest that NGF influences endothelial cell proliferation and angiogenic activity. In view of these proposed regenerative effects, we evaluated the efficacy of subcutaneous administration of highly purified murine NGF in a child with severe crush syndrome of the lower left limb. NGF 10 μg was administered subcutaneously every eight hours for seven days to the extensive ischaemic skin lesion of the calcaneal area. After treatment we observed gradual improvement of the ischaemic area; no side effects were noted. The child was discharged in good clinical condition to await a limited calcaneal escharotomy.

Hemispherectomy procedures in children: haematological issues.

In literature, excessive perioperative haemorrhage and related haemodynamic instability have been described as major risk factors in hemispherectomy. In this report we analyse the impact of neurosurgical operation on both the haematological and coagulative patterns of these children, especially focusing on younger patients. From 1993 to 2003, 18 consecutive children suffering from intractable epilepsia and treated by hemispherectomy were admitted to the Pediatric Intensive Care Unit (PICU) of Catholic University Medical School, Policlinico Gemelli, Rome. Eight children had an entire hemisphere removed (anatomical hemispherectomy), whereas the remaining 10 underwent disconnective procedures (functional hemispherectomy) or cerebral cortex ablations (e.g. hemicorticectomy). Eleven out of these 18 children underwent hemispherectomy because of hemimegalencephaly (HME): their mean age was 14.5 months (range 3-56 months); non-HME patients underwent surgery for epileptogenic lesions involving the cerebral hemisphere to a great extent or diffusely. Data have been compared with an historical cohort of 13 children operated on before 1992 at the same institution comparable for age, aetiology of epilepsy and the modalities of surgical operation.Blood losses and haemotransfusions showed a profound influence on the haematologic/coagulative status of the children operated upon. A strict correlation was demonstrated between estimated red cell volume (ERCV) loss and haemostatic impairment in this series. Recent surgical techniques appear to reduce blood losses and related haemocoagulative risks even in younger patients. ObjectsMethodsConclusions

Sevoflurane–remifentanil vs isoflurane–remifentanil for the surgical correction of craniosynostosis in infants

Background : The aim of the present study was to compare the efficacy of isoflurane–remifentanil and sevoflurane–remifentanil combinations during neurosurgical correction craniosynostosis.

Noninvasive ventilation options in pediatric myasthenia gravis

A 10‐month‐old female infant with congenital myasthenic syndrome suffering from acute respiratory failure was supported using facemask positive pressure ventilation until definitive diagnosis and specific treatment was achieved. A 12‐year‐old girl suffering from seronegative myasthenia gravis was treated by helmet‐delivered noninvasive ventilation during recurrent myasthenic episodes. Noninvasive support was really beneficial in the myasthenic crisis with respiratory muscle weakness, whereas a shift to tracheal intubation was necessary when pulmonary infection and multiple atelectasis occurred. The new helmet interface for noninvasive positive pressure ventilation can represent a valuable means of respiratory support in the early phase of respiratory failure in older children.

Cerebral hemorrhage in Henoch-Schoenlein syndrome

HeadingAbstractBackground. Henoch-Schoenlein syndrome (HSS) is the most common form of vasculitis seen in childhood. The clinical diagnosis is based on the association of nonthrombocytopenic purpura, arthritis and abdominal pain. Nephropathy is the most common complication. Hemorrhages can occur in the respiratory, gastrointestinal and urinary tracts. Neurological complications are rare, though they may be particularly severe.Case report and discussion. Intracranial hemorrhage is an extremely rare complication of the disease; we report the case of a child with cerebral hemorrhage in HSS and review the literature.

Critical presentation of pleuropulmonary blastoma

We report two cases of critical presentation of a quite rare lung neoplasm of childhood. Presentation findings were at the extremes of the clinical pattern of this polymorphous neoplasm, ranging from an enormous solid mass causing airway compression and dislocation to an apparently benign cystic lesion discovered because of a tension pneumothorax. Both children were discharged from the pediatric intensive care unit and underwent appropriate surgical removal and oncological management. Congenital lung cysts, even asymptomatic, should not be underestimated and need elective surgical excision and histologic examination.

Oxidation of intravenous lipid in infants and children with systemic inflammatory response syndrome and sepsis.

During sepsis in adults, fat becomes a preferred fuel; however, oxidation may be impaired relative to the circulating fatty acid levels. Little is known about the ability of infants and children to oxidize lipids during systemic inflammation (SIRS) and sepsis. The aim of this study was to examine the oxidation of exogenous lipid in these patients. Sixteen patients with SIRS/sepsis and eight controls with no evidence of sepsis were studied by indirect calorimetry during an i.v. lipid utilization test (1 h of 0.3 g/kg/h glucose followed by 3 h of 0.1 g/kg/h glucose plus 0.15 g/kg/h lipid). The respiratory quotient (RQ) (1.0 for carbohydrate utilization and 0.7 for fat utilization) was measured. Results were compared by repeated-measures analysis of variance (ANOVA), paired or unpaired t tests. There was no difference in baseline RQ between controls and patients with SIRS/sepsis (mean ± SD; 0.82 ± 0.08 versus 0.82 ± 0.04). The RQ of controls dropped significantly to 0.78 ± 0.08 at 240 min (p < 0.001). The RQ of patients with SIRS/sepsis also fell to 0.78 ± 0.06 (p < 0.01). Infants and children with SIRS/sepsis are able to oxidize i.v. lipid.

Massive congenital intracranial teratoma: perioperative coagulation impairment.

Massive congenital intracranial teratoma is a rare neoplasm with a poor prognosis. Surgery may be curative only if complete resection can ben obtained. Several single case reports have been published in the pediatric literature, mostly focusing on prenatal diagnosis. The authors describe six patients with congenital intracranial teratoma treated at their institution in the past decade. Perioperatively, a marked hemostatic derangement was observed in three of them undergoing surgery: the pathophysiology of this complication is discussed. The surgical indication itself represents an ethical dilemma when treating a large intracranial tumor with partial destruction and replacement of brain structures.

Isolated myocardial non-compaction in an infant with distal 4q trisomy and distal 1q monosomy

We report on a female infant with a partial 4q trisomy and 1q monosomy who presented from birth an isolated non-compaction of the left ventricular myocardium (ILVNC). The baby was born at 37 weeks with a birth weight of 2120 g. At the last clinical examination (age 24 months), she presented a senile-like appearance, narrow palpebral fissures, telecanthus, epicanthus, a broad nasal bridge, low set and posterior angulated ears, a long philtrum, and a mouth with a thin vermilion border and dimple below the lower lip (Fig. 1). She also had an anteriorly displaced anus and rocker-bottom feet. Neurological examination disclosed hypotonia and severe mental retardation. Cerebral MRI showed moderate ventricular dilatation and a hypoplastic corpus callosum. 2D-echocardiography revealed multiple, prominent myocardial trabeculations and numerous recesses at the left ventricular apical and mid-ventricular level, perfused by the main left ventricular cavity (Fig. 2). Systolic and diastolic functions were normal. An ECG showed sinus rhythm with non-specific intraventricular conduction delay. No arrhythmias or embolic events occurred. Chromosomes were scored by R (RBG) banding and fluorescence in-situ hybridisation with chromosomes 1 and 4 specific painting probes. The proband’s karyotype (46,XX der (1)(1pter fi 1q43:: 4q31 fi 4qter) revealed an unbalanced segregation of a balanced t(1;4)(q43;q31) translocation detected in the normal father. The patient was trisomic for the 4q31 fi qter region and monosomic for the 1q43 fi 1qter segment.

Perioperative management of face-to-face craniopagus twins separation

Craniopagus conjoining represents a complex and challenging issue for neurosurgeons as well as for anesthesiologists. A rare face‐to‐face case of conjoined twins underwent surgical separation and presented peculiar differences compared with those already reported in the literature. Even in cases lacking large cerebrovascular sinus connections, the impending risk of large blood loss and hemorrhagic shock in the infant requires a high level of surveillance and the institution of invasive monitoring.