Craniometaphyseal dysplasia (CMD) is a very rare bone disorder characterized by abnormally developed metaphyses in long bones and sclerosis of the craniofacial bones. In this paper, the authors report 2 cases of children diagnosed with CMD and chronic intracranial hypertension with deletion in exon 9 of the human ANK gene (ANKH). After intracranial monitoring, a different treatment was chosen for each patient. One of the patients was treated using CSF shunting because ventriculomegaly in the absence of a Chiari malformation was also observed on cerebral MR imaging. The other patient underwent cranial expansion and decompressive craniotomy of the posterior fossa, because ventriculomegaly was excluded after cerebral MR imaging and cervical MR imaging showed a Chiari malformation Type I. The origin of intracranial hypertension in CMD is multifactorial. Previous intracranial pressure monitoring and a thorough understanding of neuroimaging studies are essential to achieve an accurate diagnosis and effective treatment.
Mónica Rivero-Garvía, F. Javier Márquez-Rivas, Antonia García-Iglesias and Raquel Gutiérrez-González
Mónica Rivero-Garvía, Javier Márquez-Rivas and Jorge Giménez-Pando
María José Mayorga-Buiza, Mónica Rivero-Garvía, Javier Márquez-Rivas, Carlos Velásquez-Rodríguez and Emilio Gómez-González
Javier Márquez-Rivas, Mónica Rivero-Garvia, Maria José Mayorga-Buiza and Gregorio Rodríguez-Boto
Mónica Rivero-Garvía, Gregorio Rodríguez Boto, Álvaro Pérez-Zamarrón, Raquel Gutiérrez-González, Issa Subhi Issa Ahmad and Armando Martínez
Jorge Tirado-Caballero, Andres Muñoz-Nuñez, Santiago Rocha-Romero, Mónica Rivero-Garvía, Emilio Gomez-González and Javier Marquez-Rivas
Intracranial pressure (ICP) measurements are imperative for the proper diagnosis and treatment of several neurological disorders. Telemetric sensors have shown their utility for ICP estimation in short-term monitoring in humans. However, their long-term reliability is uncertain. The authors present the case of a 37-year-old woman diagnosed with benign intracranial hypertension and obesity. The patient underwent gastric bypass surgery for ICP control. In order to monitor ICP before and after bariatric surgery, a Neurovent-P-tel sensor was implanted in the left frontal lobe. After gastric bypass, normal ICP values were recorded, and the patient’s visual fields improved. However, the patient experienced incapacitating daily headaches. The authors decided to implant a Codman Microsensor ICP transducer in the right frontal lobe to assess the long-term reliability of the Neurovent-P-tel measurements. A comparison of the recordings at 24 and 48 hours showed good correlation and reliability during long-term monitoring with the Neurovent-P-tel, with minimal zero drift after 11 months of implantation.
Jorge Tirado-Caballero, Mónica Rivero-Garvia, Francisco Arteaga-Romero, Jorge Herreria-Franco, Ángel Lozano-Gonzalez and Javier Marquez-Rivas
Treatment of posthemorrhagic hydrocephalus in preterm infants is a matter of debate among pediatric neurosurgeons. Neuroendoscopic lavage (NEL) has been proposed as a suitable technique for the management of this pathology. The authors present their experience with 46 patients treated with NEL after germinal matrix hemorrhage, describe their technique, and analyze the outcomes.
A retrospective analysis of patients affected by grade III or IV intraventricular hemorrhage (IVH) treated with NEL was performed. Nonmodifiable risk factors such as gestational age, weight at birth, modified Papile grade, and intercurrent diseases were reviewed. Safety analysis was performed, evaluating the incidence of postoperative complications. Effectivity was measured using radiological (frontal horn index and white matter injury), CSF biochemical (proteins, blood, and cellularity), and clinical variables. Permanent shunt rate and shunt survival were analyzed. The motor outcome was measured using the Gross Motor Function Classification System (GMFCS) at 18–24 months, and the neurocognitive outcome was evaluated according to the grade of adaptation to schooling.
Forty-six patients met the inclusion criteria. Patients were treated with a single NEL in 21 cases, 2 lavages in 13 cases, and 3 lavages in 12 cases. The mean gestational age at birth was 30.04 weeks, and the mean weight at birth was 1671.86 g. Hyaline membrane disease was described in 4.8% of the cases, hematological sepsis in 43.47%, persistent ductus arteriosus in 23.9%, and necrotizing enterocolitis in 10.9% of the cases. Modified Papile grade III and IV IVHs were observed in 60.9% and 39.1% of the cases, respectively. Postoperative infection was diagnosed in 10 of the 46 cases, CSF leak in 6, and rebleeding in 3. The mean frontal horn index decreased from 49.54 mm to 45.50 mm after NEL. No white matter injury was observed in 18 of 46 patients, focal injury was described in 13 patients, and diffuse bilateral white matter injury was observed in 15 patients. All CSF biochemical levels improved after lavage. The shunt rate was 58.7%, and shunt survival at 1 year was 50%. GMFCS grades I, II, III, IV, and V were observed in 44.74%, 21.05%, 2.63%, 15.79%, and 15.79% of patients, respectively. Good neurocognitive results were observed in 53.3% of the cases.
Neuroendoscopic lavage was a suitable alternative for the management of IVH in preterm infants in our series. Good motor and neurocognitive results were achieved by this technique, and the permanent shunt rate was reduced compared with historical controls.
Carlos Velásquez, Mónica Rivero-Garvía, Maria Jose Mayorga-Buiza, María de los Ángeles Cañizares-Méndez, Manuel E. Jiménez-Mejías and Javier Márquez-Rivas
This report describes a reliable and simple technique for securing external ventricular drains (EVDs) to the scalp and avoiding pullout complications. The operative technique consists of fixing the drain between 2 hydrocolloid dressings and securing it with staples. A 10-year retrospective analysis of EVD pullout complications was performed in a series of 435 consecutive patients who were treated at a single institution. The EVD pullout complication rate was 0.4%. No complications related to the fixation technique were found. The median operative time required to fix the drain was 60 seconds. The technique presented here is a simple and reliable procedure to fix the EVD to the scalp, preventing pullout complications and thus reducing the morbidity of EVD reimplantation.