Intracranial complications of pediatric rhinosinusitis: Identifying risk factors and interventions affecting length of hospitalization.

OBJECTIVE: To identify risk factors and interventions affecting length of hospitalization (LOH) and clinical outcome in children with intracranial complications of rhinosinusitis. METHODS: Retrospective chart review of 12 children hospitalized at 2 academic medical centers for intracranial complications of rhinosinusitis over the past 5 years. RESULTS: 12 patients were identified with an average age at presentation of 13 years old. 92% were male and 75% were African American. The most common presenting symptoms were fever and headache. Localizing neurological symptoms including hemiparesis and aphasia, in addition to seizures occurred in 33% of patients and increased LOH significantly (33 versus 15 days, p = 0.03). Epidural (EA) and subdural abscesses (SA) were the most common intracranial complications. 58% of patients were initially treated with a combination of open neurosurgical (ON) intervention and endoscopic sinus surgery (ESS) and LOH was significantly shorter for these patients compared to those treated otherwise (14 versus 31 days, p = 0.02). Streptococcus species were the most common group of bacteria identified in 75% of cases, with S. anginosus accounting for 42% of cases. The overall average LOH was 21 days with 92% of patients having complete resolution of symptoms by time of discharge. CONCLUSIONS: Treatment of intracranial complications of acute rhinosinusitis can have favorable outcomes after appropriate surgical management. Localizing neurologic symptoms and seizures portend longer hospital stay and recovery time. Shorter hospital stay was seen in those undergoing early combined ON and ESS interventions.

Small size craniotomy in endoscopic procedures: Technique and advantages.

BACKGROUND: Endoscopic procedure has been known as the method of choice for treatment of hydrocephalus with 8.5% complication rate. It seems that good dural closure, reconstructing bone defect and perfect pericranium suturing can decrease the wound complications. Here, we describe the method of minicraniotomy instead of the burr hole in the endoscopic procedure. MATERIALS AND METHODS: A case-control study regarding the cranial opening for endoscopic surgery was done in 45 patients of <12 months age; 15 patients in case group for minicraniotomy and 30 infants as control group for burr hole. They were followed at least 1-month for complications including cerebrospinal fluid collection, wound dehiscence, wound infection, and meningitis. RESULT: Patients were between 1 and 11 months. Hydrocephalus (73%) was the most common etiology for endoscopic surgery in this series, followed by the arachnoid cyst (20%). Two patients in the case group and eight in control group developed complications. Meningitis was found in one infant in the control group. Despite less complications in the case group the difference between two cohorts was not statistically significant. CONCLUSION: Minicraniotomy with providing more space in comparison to burr hole makes dural closure possible. It provides a small bone flap that can be replaced inside the bone defect. In spite of nonsignificant statistical difference between two groups regarding complication rate, we found less wound complications with minicraniotomy. Therefore, we advise this technique for the endoscopic procedure and propose minicraniotomy even in the older population to provide better dural opening, watertight dural closure and reconstructing the bone defect.

Identical twins with lumbosacral lipomyelomeningocele.

Lipomyelomeningocele, a congenital spine defect, is presented as skin-covered lipomatous tissue that attaches to the cord in different ways according to its subtypes. Unlike other types of neural tube defects, the exact cause of this birth defect has not been confirmed yet, but it is proposed to be a multifactorial disease with involvement of both genetic and environmental factors. The authors describe identical twins with lipomyelomeningocele of the same subtype and location without any familial history of similar abnormality. Therefore, the same genetic and/or environmental risk factors could have played a part in their condition.

Congenital cardiac anomalies in myelomeningocele patients.

OBJECTIVE: Myelomeningocele may be isolated but more frequently is associated with other anomalies. Congenital heart disease occurs with different incidence rate in myelomeningocele which is observed more frequently with skeletal malformations. METHODS: This study was undertaken in the Children's Hospital Medical Center between 2010 to 2012 to evaluate 75 myelomeningocele patients for cardiac anomalies, with electrocardiography and echocardiography in addition to clinical examination of the cardiopulmonary system. Demographic information, myelomeningocele location and characteristics, orthopedic deformities, neurological deficits and radiographic findings were studied besides cardiologic assessments. RESULTS: The ages of the patients ranged from 1 day to 4 years. The myelomeningocele locations were lumbosacral, lumbar and sacral area in most cases. Physical examination of the heart was abnormal in 6 children, but echocardiography revealed cardiac anomalies in only two children. Both children were female patients with severe scoliosis, multiple rib deficiencies and associated vertebral anomalies. CONCLUSION: Congenital heart defects are not very common in MMC patients. Female patients with suspicious clinical examinations for cardiac anomalies and associated rib and vertebral anomalies are advised to be investigated by echocardiography to rule out associated cardiac anomalies.

Secondary bicoronal synostosis after metopic craniosynostosis surgical reconstruction.

Re-synostosis after standard surgical procedures for nonsyndromic craniosynostosis is a rare event, which can occur at the same suture or rarely in adjacent sutures. Here, we report 2 patients with primary metopic craniosynostosis who developed bicoronal synostosis in previously opened sutures several months after surgery.

Interfrontal encephalocele: a rare feature of forehead in hydrocephalic myelomeningocele patients. Clinical feature, probable mechanisms, and management.

OBJECTIVE: Myelomeningocele is a complex central nervous system malformation mostly associated with other neural and extraneural anomalies. A rare special feature of the forehead and skull was observed in myelomeningocele. Here, we present several patients with myelomeningocele, severe hydrocephalus, and interfrontal encephalocele accompanied by metopic suture widening and abnormally shaped frontal bones. METHODS: Five children with this feature were enrolled in this series. The age, sex, location of myelomeningocele sac, neurological deficits, hydrocephalus and history of shunt surgery, and follow-up period were evaluated. RESULTS: Patients were aged from 1 to 12 months (mean, 6 months). All patients were male. Most sacs were located in lumbar and lumbosacral areas. Neurological deficits varying from only sphincter problem to paraplegia were found in all patients. Hydrocephalus was found in all children that needed a shunt procedure. Asymptomatic Chiari malformation, interfrontal encephalocele, and corpus callosum agenesis were evident in five, five, and three patients, respectively. CONCLUSION: Anterior fontanel anomalies known as interfrontal encephalocele associated with myelomeningocele have been reported before. The patients have an open metopic suture extending widely to the nasal radix producing hypertelorism accompanied by interfrontal herniation of frontal lobes. The associated hydrocephalus exaggerates the anomaly. Treatment of accompanying hydrocephalus is advised to decrease the severity of frontal lobe herniation. Some patients may need frontal bone reconstruction surgery to provide cosmetic correction at the place of the midline frontal bone defect.

Infections of the spinal subdural space in children: a series of 11 contemporary cases and review of all published reports. A multinational collaborative effort.

INTRODUCTION: Positioned anatomically between the spinal epidural space and the intramedullary compartment, the spinal subdural space remains the least common area of localized infection in the central nervous system. Infectious processes of the subdural spinal space include subdural spinal empyema, subdural spinal abscess, infected spinal subdural cyst, and infectious spinal subdural cyst. To date, there has been no systematic review of these entities in children, with the cumulative knowledge of the pathophysiologic, microbiologic, and demographic characteristics of these infections relegated solely to few small series and case reports. METHODS: A series of 11 recent cases culled from the collaboration of international authors are presented. In addition, an exhaustive MEDLINE search and manual review of the international literature was performed, identifying a total of 73 cases of spinal subdural infections in patients under the age of 21. Data of interest include the age, sex, signs, and symptoms at presentation, spinal location of infection, presence of spinal dysraphism, and other comorbidities, offending organism, treatment, outcome, and follow-up. RESULTS: Patients ages ranged from 4 weeks to 20 years (mean, 6.5 years). Males outnumbered females by a ratio of 2:1. Over half (53 %) of spinal subdural infections in children were associated with spinal dysraphism or other congenital abnormalities of the spine. The commonest organism to infect the spinal subdural space in children is mycobacterium tuberculosis and the thoracic spinal region was most commonly infected. CONCLUSIONS: The disease is usually treated surgically, although a more expectant approach consisting of antibiotics and observation has also been proposed.

Tethered cord with tandem lipomyelomeningoceles, split cord malformation and thick filum.

Lipomyelomeningocele, a closed neural tube defect, usually occurs in lumbosacral area as a single lesion but can be associated with other spinal dysraphism. We report an infant with a very rare presentation of tandem lumbosacral lipomyelomeningoceles, thick filum terminale and split cord malformation.

Risk factors associated with lipomyelomeningocele: a case-control study.

BACKGROUND: In general, it seems that both genetic and environmental factors play important roles in the induction of neural tube defects. Lipomyelomeningocele (LipoMMC) is a rather common type of closed neural tube defect, but only limited studies have investigated the potential risk factors of this anomaly. Therefore, the purpose of this case-control study was to investigate the risk factors involved in LipoMMC formation. MATERIAL AND METHOD: Various risk factors were evaluated in 35 children between 1 month and 10 years of age with LipoMMC in a hospital-based case-control study. The 2 control arms consisted of 35 children with myelomeningocele (MMC group) and 35 children with congenital anomalies other than central nervous system problems (control group). All groups were matched for age and visited the same hospital. A structured questionnaire was used for the collection of all data, including the mothers' weight and height during pregnancy, education, reproductive history, previous abortions, and socioeconomic status, as well as the parents' consanguinity and family history of the same anomalies. RESULTS: Univariate analysis of the children with LipoMMC compared to the control group showed that the use of periconceptional folic acid supplementation was significantly lower in the MMC and LipoMMC groups compared to the control group. In addition, comparison of the MMC and control groups revealed statistically significant differences regarding the use of folic acid and maternal obesity. In multivariate analysis, use of folic acid in the periconceptional period and during the first trimester was an independent risk factor for LipoMMC and MMC. Furthermore, maternal obesity was a significantly positive risk factor for MMC. CONCLUSION: The probable risk factors for LipoMMC were investigated in this case-control study. Consumption of folic acid in the periconceptional period and during the first trimester is an independent protective factor against LipoMMC. It seems that larger studies are needed to examine other possible risk factors.

Ventriculoperitoneal shunt migration and coiling: A report of two cases.

Migration of the proximal and distal catheters of the ventriculoperitoneal shunt is a very rare event. Here, we report two infants with hydrocephalus and ventriculoperitoneal shunt who presented later with shunt coiling and migration. The first infant was admitted with scalp swelling around proximal incision 3 months after shunt insertion and migration and coiling of both ventricular and peritoneal catheters occurred under the scalp at that point. The second patient was referred 1 month after shunting with tense fontanel and vomiting. New brain imaging confirmed the whole shunt inside both lateral ventricles. The possible mechanisms causing this very uncommon complication and the management are explained.

Cranial dermal sinus: presentation, complications and management.

OBJECTIVE: Cranial dermal sinus is a very rare anomaly mostly found in the midline occipital area, presenting with diverse clinical features from asymptomatic dermal sinus to symptomatic situations including meningitis, dermoid tumor or abscess. METHODS: Medical records of 18 patients with 19 cranial dermal sinuses operated at the Children's Hospital Medical Center in Tehran from 1999 to 2011 were retrospectively studied. Their presentations, accompanying anomalies, radiological and surgical findings were evaluated. RESULTS: Patients were aged 1 month to 6 years and were followed for 2-12 years. Most sinuses were located in the midline occipital region. Infection was the most common reason for referral (50%) followed by skin dimple (33.3%). Creamy white discharge was found in 50% of patients. Posterior fossa abscess, hydrocephalus and meningitis were observed in 38.9, 38.9 and 22.2% of children, respectively. All sinus tracts with associated dermoid tumors or abscesses were removed en bloc to decrease the risk of recurrence. CONCLUSION: The sinus tracts are a portal of entry for pathogens which result in serious complications of meningitis or abscess. In this series, 67% of children presented with serious symptoms; therefore, we recommend prophylactic surgery in all cranial dermal sinuses. The exact time of surgery is not obvious as the natural course of the disease is unknown. If any symptom is observed surgery should be done as soon as possible. For asymptomatic sinuses identified by the pediatrician during a regular visit prophylactic surgery is recommended as soon as the child can tolerate operation.

Infected dermoid tumor causing tethered cord after myelomeningocele repair.

Intramedulary dermoid tumors are rare tumors mostly found associated with dermal sinus tracts. Spinal dermoid tumor can occur after myelomeningocele repair. Infected dermoid tumors are reported in patients with dermal sinus tracts as well, but have never been reported subsequent to myelomeningocele surgery. Here, we report a rare association of infected dermoid tumor with tethered cord without dermal sinus tract in a child who had been operated for myelomeningocele during infancy.