Role of the pupillometer in the assessment of pain in the sedation of pediatric patients.

OBJECTIVE: Pupillometry has been used to assess pain intensity and response to analgesic drugs in adults. The aim of this study was to verify the usefulness and effectiveness of the pupillometer to assess pain and depth of sedation in pediatric patients undergoing painful procedures and to optimize pain management by observing pupillary variations induced by opioids. PATIENTS AND METHODS: This is a prospective, monocentric study conducted in the sedation room of the Pediatric Intensive Care Unit of Fondazione Policlinico A. Gemelli in Rome. A population of 22 pediatric patients who underwent painful procedures was enrolled. Eleven children were sedated by opioid drugs. Heart rate, systolic blood pressure, diastolic blood pressure, bispectral index, maximum pupil size (Size), pupil change (CH), Neurological Pupil Index (NPi) were collected over four times: before starting the procedure; before the painful stimulus (when the patient was sedated); when the painful stimulus was applied; at the end of the procedure. A NeurOptics NPi-200 pupillometer was used for the study. RESULTS: Statistical significance in the variation of haemodynamic parameters was less significant than the variation obtained by analyzing the pupillary parameters: a significant change in NPi and CH in the transition from wakefulness to sedation and from the application of the painful stimulus to awakening was found in both study populations, patients who have received opioids and patients who have not received opioids. Changes in the mean CH of the pupil diameter correlate with the depth of sedation, and the size values vary in relation to the administration of opioids. CONCLUSIONS: Our findings highlight the potential role of pupillometry as a non-invasive method to objectively quantitate pain response in children to reach an efficient analgesic approach.

Taking Care of Relationships in the Intensive Care Unit: Positive Impact on Family Consent for Organ Donation.

BACKGROUND: Organ donation refusal from relatives of potential donors with brain death significantly reduces organ availability. The need for organ donation has increased over time, but the shortage of available donors is the major limiting factor in transplantation. We analyzed the impact of a new systematic communication approach between medical staff and patients' relatives on the rate of consent to organ donation. METHODS: The study was conducted as a single-center, non-randomized, controlled, before-and-after study at an 18-bed intensive care unit (ICU) of a university hospital. We compared the rate of consent for organ donation before and after the introduction of the new communication approach. RESULTS: A total of 291 brain-dead patients were studied. The consent rate increased from 71% in the pre-intervention period (2007-2012) to 78.4% in the post-intervention period (2013-2015), with an 82.75% increase in the 2014 to 2015 period. During these periods, no significant variation of consent to organ donation was recorded at the national and regional levels. CONCLUSIONS: The introduction of a new communication approach between medical staff and relatives of brain-dead patients was associated with a significant increase in the rate of consent to donation. Our results highlight the importance of empathy with relatives in the ICU.

Late-presenting right congenital diaphragmatic hernia with severe hypotrophy of the right lobe of the liver.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) presenting after 30 days of life is unusual and has a variant pattern of presentation. PRESENTATION OF CASE: We present a death case occurred to a 34-days-old infant. The infant arrived to our emergency department in cardiac arrest after having suffered from intermittent acute abdominal pain. Autopsy confirmed the presence of a right CDH, with herniation of the right lobe of the liver into the thorax. DISCUSSION: Most of the cases of CDH are diagnosed prenatally or in the neonatal period. However, some patients do not develop symptoms until after the neonatal period. The relevance of our case is the co-existence of right CDH and important hypotrophy of the right lobe of the liver. CONCLUSIONS: Evidence of this phenomenon represents an absolute novelty in the extant scientific literature. Even if rare, we suggest to suspect the presence of CDH in fetus with disparity in right and left liver lobe at prenatal ultrasound.

[The management of the child with head injury: initial assessment]. / Il management del bambino con trauma cranico: gestione iniziale.

In western countries, head injury is very important in public health; infact it is the main cause of mortality after infancy and a significant cause of long term disability. The patients with severe head injury needs an intensive care unit management, but the initial approach is a critical phase, too. In fact, it is demonstrated that trauma mortality and morbility are significantly reduced by a quick and well conducted approach. This article focuses the initial management of the injured child.

[The management of the child with head injury: our experience]. / Il management del bambino con trauma cranico: nostra esperienza.

OBJECTIVES: To analyze the group of children admitted to our Pediatric Intensive Care Unit (PICU) for neurotrauma and describe the management algorithms adopted by us for pediatric head injury. METHODS: All the children affected by head injury and admitted to PICU since november 1992 to november 2000 have been examined. Injury severity has been classified using the Glasgow Coma Score (GCS), while the long term neurological outcome with the Glasgow Outcome Score (GOS). We have described the clinical presentation, the kind and dynamics of injury and the clinical outcome one month after discharge. RESULTS: 210 children with head injury have been identified. Among them: 38 were affected by mild head injury, 50 by a moderate one and 122 by a severe one. The most frequent cause of injury has been represented by car accidents and motorbike or bicycle falls. The overall outcome has resulted good (GOS 4-5) in all children affected by mild or moderate head injury; on the other side, patients with severe injury have presented the following scores: GOS 1 (dead children) 14.7%, GOS 2 (persistent vegetative state) 1.6%, GOS 3 (severe disability) 22.2%, GOS 4 (mild disability) and GOS 5 (no disability) 61.5%. CONCLUSION: A correct management of children with head injury helps clinicians to improve outcome and to reduce mortality. Therapeutics algorithms suggested by us could be useful for the management of this kind of patients, not only when they are affected by a severe head injury but, also, when they suffer from a mild one, that is the most common event in the emergency room departments.

Antitoxin use and pediatric intensive care for viper bites in Rome, Italy.

In Italy viper bites represent an uncommon event, though envenomation can cause severe complications, more in children than adults, because of dose/body size ratio. We present a case series within a selected population: 10 Italian cases (from Rome surroundings) of viperbites requiring PICU admission, over a 5-year interval. Five children showed a systemic involvement, whereas the remaining patients showed a damage. All were managed and closely monitored in an ICU setting. Relevant clinical findings and therapeutic approach, ICU course and complications have been recorded. Age range was 3-15 years with mean age of 6,9 (SD±4,58) years; 2 patients needed respiratory support beyond oxygen supplementation. Most patients underwent fluid loading, while hemodynamic support was given to4/10. Median PICU stay was 60 hours (IQR=24.0-75.5). No mortality was reported. Indications and precautions for administration of antivenom in the last years have been reviewed: early treatment seems to reduce mortality/morbidity, though representing a threat for children. Current recommendations for the treatment of viper envenomation have been described, based on a literature's review and the application of these knowledges to clinical reality of our PICUs. Therefore, paediatric patients with systemic or rapidly evolving symptoms should be monitored carefully for the development of bite-related complications in an ICU setting mostly in younger children.

The absence of the verumontanum at voiding cystourethrography as a sign of prostate maldevelopment.

Prostate maldevelopment in prune-belly syndrome has only been described at necropsy. No reports are available in the "in vivo" studies. The absence of the verumontanum at voiding cystourethrography correlates with verumontanum and prostate hypoplasia. This radiographic sign can represent the earliest finding in prostate maldevelopment and might contribute to the "in vivo" assessment of the disease, especially in doubtful cases.

Anesthesiological and intensive care considerations in children undergoing extensive cerebral excision procedure for congenital epileptogenic lesions.

INTRODUCTION: Epilepsy is a relatively common condition in childhood with a generally favorable prognosis of the affected population. Nevertheless, a significant minority of the treated children do not respond to the medical treatment so that surgical treatment is necessary. While minor surgical procedures have a negligible incidence of mortality, major ones may carry a significant risk of perioperative complications. The leading cause of mortality is represented by hemorrhagic derangements after high intraoperative and postoperative blood loss, mostly in very young patients. Therefore, restoration of euvolemia, detection and correction of related bleeding disorders represent the major concern for pediatric neuroanesthesiologists and intensivists throughout the perioperative period. The present report is focused on the anesthesia and intensive care management of the surgical epileptic patient. CONCLUSION: Authors recommend that these high-risk procedures should be performed in highly experienced centers where pediatric neurosurgery is performed daily.

Miller-Fisher syndrome mimicking intracranial hypertension following head trauma.

INTRODUCTION: Miller-Fisher syndrome (MFS) is a polyneuropathy with benign outcome characterized by ophthalmoplegia, limb ataxia and tendon areflexia. Impaired consciousness level and intracranial hypertension are very rare symptoms in MFS. CASE REPORT: We describe the case of a 5-year-old girl who showed intracranial hypertension, transient coma and respiratory failure after mild head injury; moreover the patient showed mild ataxia, areflexia, ophthalmoplegia and autonomic disturbances. These symptoms were suggestive of MFS. Electrophysiologic studies and laboratory tests confirmed the diagnosis and immunoglobulins and steroids were given. The child showed a progressive clinical improvement and the final outcome was good. CONCLUSION: This case, initially managed as trauma injury due to the presence of suggestive signs and clinical history, maskered an atypical presentation of Miller-Fisher syndrome, a rare disorder of central nervous system.

Conversion reaction presenting as a polyradiculonevritis.

AIM: To show that conversion reactions should be suspected when symptomatology or instrumental checking does not conform to a precise pathological pattern in its anatomical and physiological keys. METHODS: We report the case of a 14-y-old girl who presented with signs and symptoms of Guillain-Barré polyradiculonevritis. Negative instrumental and laboratory tests led us to consider a possible diagnosis of conversion reaction. RESULTS: After a more detailed history emphasizing the close bond between the patient and her father, a poor peer-relation exchange and psychological stress, we were able to identify conversion reactions through the patient's motor signs. CONCLUSION: Conversion symptoms must be suspected and searched for when the neurologic outline is difficult to clarify, and also in the case of Guillain-Barré syndrome that does not respond to plasmapheresis.

[Lipid metabolism and atherosclerosis risk in renal diseases in pediatric age]. / Metabolismo lipidico e rischio aterosclerotico nelle patologie renali in età pediatrica.

The pathophysiological events regarding the atherosclerotic processes are present already in early childhood. Various clinical trials confirm tight correlation between dyslipidemia and cardiac and cerebrovascular complications. Over the clinical dyslipidemia genetically determined, various systemic diseases can lead to anomalies of the lipidic metabolism. The determination and analysis of the lipidic pattern is essential during childhood and in particular in patients with renal disease for the increased risk of progressive failure of renal function and for the central role of kidney in lipidic homeostasis. Cardiovascular event is the most important cause of mortality in patients with end-stage renal disease. In children with nephrotic syndrome, renal failure, hemodialysis or after renal transplantation, an increase of the serum values of triglycerides, cholesterol, LDL, VLDL is generally observed. The presence of anomalies in lipidic pattern must be considered during the course of renal disease for preventing the progression of renal damage. Thus, the pharmacological therapy and diet permit to prevent the atherosclerotic events.

Cryptorchidism associated with meningomyelocele.

OBJECTIVE: The authors studied 75 male infants with meningomyelocele (MMC) to analyse the association with cryptorchidism. METHODS: All infants were diagnosed at birth and surgically corrected after 24-48 h. Testes were classified undescended in accordance with Kaplan. The associated malformations, the exact levels of the lesion (surgical report) and the presence of hydrocephalus and the cremasteric reflex have each been assessed. RESULTS: In these patients a higher incidence of cryptorchidism (14.8%) was found than in the normal population. Lesion level (L2-L3) and absence of cremasteric reflex was higher in children with associated cryptorchidism. Infants with MMC and undescended testes showed no increased incidence of hydrocephalus and sexual hormone levels remained within the normal range. These results suggest that the cremaster muscle could be a major factor involved in testis descent through the inguinal canal. CONCLUSIONS: The authors propose that spinal cord lesion, as well as neurologic dysfunction, is of major importance in the pathophysiology of cryptorchidism in spina bifida.

Cerebral hemorrhage in Henoch-Schoenlein syndrome.

BACKGROUND: 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.

Pulmonary oedema caused by "liquid ecstasy" ingestion.

In this case report we describe the first recorded case of alveolar gas exchange impairment caused by "liquid ecstasy" ingestion, and perhaps inhalation, by a 4 year old child. The pulmonary gas diffusion disturbance was sufficiently prolonged to raise the suspicion of a direct toxic effect on the alveolar-capillary membrane.

Pulmonary hemorrhage complicating a typical hemolytic-uremic syndrome.

We describe a case of pulmonary bleeding and subsequent acute respiratory distress syndrome (ARDS) in a 20-month-old female suffering from a typical postdiarrheal hemolytic-uremic syndrome (HUS). Acute renal failure was treated early by peritoneal dialysis. It is of interest to underline that thrombocytopenia or any coagulative impairment was absent when this complication occurred, and spontaneous diuresis recovery was ongoing. All examinations failed to identify a unique site of bleeding in the main stem bronchi or trachea. Complete renal and clinical recovery was obtained in spite of this very uncommon complication. Intensivists should be aware of this rare and potentially fatal complication of typical (D+)HUS.

Intramedullary epidermoid cyst presenting with abnormal urological manifestations.

STUDY DESIGN: Report of an epidermoid cyst with intramedullary localization. OBJECTIVE: To describe an atypical presentation of intramedullary epidermoid cyst. SUMMARY OF BACKGROUND DATA: Intramedullary epidermoid cysts are rare entities with a marked variability in the clinical presentation, essentially of neurological pertinence. METHODS: Case report of a spinal epidermoid cyst in a 13-year-old girl presenting with urological symptoms: she had a 12-month history of recurrent low urinary tract infections, urinary frequency and nocturnal enuresis. A urodynamic evaluation was performed and showed the presence of involuntary bladder contractions with detrusor instability and low bladder compliance. Magnetic resonance imaging of the spine demonstrated an intramedullary lesion of the dorsal spinal cord. RESULTS: The mass was excised and 6 months after surgical excision, urological manifestations improved with decreased detrusor hyper-reflexia, increased bladder capacity and compliance and no later report of urinary tract infections. CONCLUSIONS: In our patient, unusual clinical manifestations of the tumor have delayed the diagnosis, but its complete removal has led to remission of symptoms. Detailed neurological examination and investigations are indicated in patients with clinical and urodynamic features, suggestive of neuropathic bladder.

Glycosaminoglycans: urinary excretion in children with myelomeningocele.

BACKGROUND: The main consequences of neurogenic bladder dysfunction are renal damage related to high intravesical pressure, vesicoureteral reflux (VUR) and urinary tract infections (UTIs). Neurologic impairment, UTIs and VUR are known to be linked with a potential for renal scarring. Of paramount importance as predisposing conditions for UTIs in neurogenic bladder are poor bladder drainage and detrusor-sphincter dyssynergy which cause further abnormalities on the internal bladder surface and, consequently, a bladder wall rich in glycosaminoglycans (GAGs). MATERIALS AND METHODS: The aim of this study is to investigate the correlation between GAG excretion and bladder wall degeneration in 43 patients affected by spina bifida (SB) and 40 healthy age-matched control children. RESULTS: The amounts of GAGs excreted vary greatly in SB patients aged from 0 to 5 years, and values are comparable to those observed in normal controls. They are significantly higher in children over 5 years of age. CONCLUSION: The increased excretion of GAGs in older SB patients is an important parameter in the evaluation of the physiopathological condition of the bladder wall and hence may be considered a possible marker for monitoring the beginning of bladder damage.