Risk factors for shunting at 12 months following open fetal repair of spina bifida by mini-hysterotomy.

OBJECTIVES: Open spina bifida (OSB) is the most common neural tube defect. Prenatal repair reduces the need for ventriculoperitoneal shunting (VPS) due to hydrocephalus from 80-90% to 40-50%. We aimed to determine which variables work as risk factors for VPS at 12 months of age in our population. METHODS: Thirty-nine patients underwent prenatal repair of OSB by mini-hysterotomy. The main outcome was occurrence of VPS in the first 12 months of life. Logistic regression was used to estimate the odds ratios (OR) between prenatal variables and the need for shunting. RESULTS: VPS at 12 months occurred in 34.2% of the children. Larger ventricle size before surgery (62.5% ≥15 mm; 46.2% between 12 and 15 mm; 11.8% <12 mm; p=0.008), higher lesion level (80% >L2, vs. 17.9% ≤L3; p=0.002; OR, 18.4 [2.96-114.30]), and later gestational age at surgery (25.25 ± 1.18 vs. 24.37 ± 1.06 weeks; p=0.036; OR, 2.23 [1.05-4.74]) were related to increased need for shunting. In the multivariate analysis, larger ventricle size before surgery (≥15 mm vs. <12 mm; p=0.046; OR, 1.35 [1.01-1.82]) and higher lesion level (>L2 vs. ≤L3; p=0.004; OR, 39.52 [3.25-480.69]) were risk factors for shunting. CONCLUSIONS: Larger ventricle size before surgery (≥15 mm) and higher lesion level (>L2) are independent risk factors for VPS at 12 months of age in fetuses undergoing prenatal repair of OSB by mini-hysterotomy in the studied population.

Open fetal myelomeningocele repair at a university hospital: surgery and pregnancy outcomes.

PURPOSE: Myelomeningocele (MMC) is an open neural tube defect that causes great morbidity. Prenatal open repair is the standard treatment; however, there are many complications related to the procedure. This study reports preliminary findings of open in utero repair of MMC in a public tertiary hospital in Brazil and describes factors that could be associated with increased surgical morbidity. METHODS: Thirty-nine patients underwent open in utero repair of MMC from October 2015 to August 2019. The Clavien-Dindo classification of surgical complications and a classification system with the preterm definitions of the World Health Organization were used, respectively, for maternal and fetal complications. RESULTS: A total of 28 mothers (71.8%) and 31 fetuses (79.5%) experienced at least one minor to major complication. Three mothers (7.7%) had a severe grade 4 complication. Fetal complications grades 3 to 5 occurred in 13 fetuses (33.3%). Gestational age at surgery and at birth were 24.88 ± 1.16 weeks and 33.23 ± 3.68 weeks, respectively. Preterm delivery occurred in 30 patients (76.9%), membrane rupture in 18 patients (46.2%) and chorioamnionitis in 13 patients (33.3%). CONCLUSION: Open fetal surgery for MMC was performed at a Brazilian public tertiary care center, resulting in three grade 4 maternal complications. Relevant fetal complications were also present. The use of a standard classification system for complications renders studies more comparable and data more useful for counseling patients. Adjustments of perioperative procedures and long-term follow-up are needed to determine the real benefit of open in utero repair of MMC at our hospital.

Brain abnormalities in myelomeningocele patients.

BACKGROUND: Myelomeningocele (MMC) is often related to hydrocephalus and Chiari malformation (CM) type 2; however, other brain abnormalities have been reported in this population. In order to better understand and quantify other forebrain abnormalities, we analyzed magnetic resonance imaging (MRI) of MMC patients treated in utero or postnatal. METHODS: Between January 2014 and March 2017, 59 MMC were treated in our hospital. Thirty-seven patients (32 postnatal and 5 intrautero repair) had brain MRI and were enrolled at the study. MRI was analyzed by two experienced neuroradiologists to identify the supra and infratentorial brain abnormalities. RESULTS: A wide range of brain abnormalities was consistently identified in MMC patients. As expected, the most common were hydrocephalus (94.5%) and CM type II (89.1%). Of note, we found high incidence of corpus callosum abnormalities (86.4%), mostly represented by dysplasia (46%). CONCLUSIONS: The data are consistent with the concept that brain abnormalities related to MMC can be both infratentorial and supratentorial, cortical, and subcortical. More studies are needed to correlate these forebrain abnormalities to long-term functional outcome and their prognostic value for these patients.

Apert syndrome without craniosynostosis.

BACKGROUND: Apert syndrome is a rare form of syndromic craniosynostosis, also known as acrocephalosyndactyly, which is a disorder characterized by a unique set of craniofacial, hand, and foot abnormalities. Diagnosis is made through a genetic analysis, where the mutation of FGFR2, Ser252Trp, and Pro253Arg confirms the diagnosis. CASE PRESENTATION: Although craniosynostosis is the most common characteristic in clinical presentation, we present an atypical case of a one-and-a-half-year-old girl with Apert syndrome confirmed by genetic testing but without craniosynostosis.

Temporal arachnoid cysts: are they congenital?

CASE REPORT: The authors report two cases of arachnoid cysts (ACs) neither detected during pregnancy nor shortly after birth when newborns underwent CT scan evaluation after birth head trauma. ACs were diagnosed at 10 months and 6 years, respectively. The first one becomes symptomatic, and the other one was incidentally found during a head trauma investigation. DISCUSSION: These cases give support for the postnatal pathogenesis for some of the assumed congenital ACs. We collected data from the literature that supports the acquired hypothesis for ACs.

Hydrocephalus: a rare initial manifestation of sporadic intramedullary hemangioblastoma : Intramedullary hemangioblastoma presenting as hydrocephalus.

BACKGROUND: Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. CASE PRESENTATION: A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up. CONCLUSION: Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.

Major clinical features of synostotic occipital plagiocephaly: mechanisms of cranial deformations.

PURPOSE: The clinical diagnosis of most common single-suture craniosynostosis is easily set, based on the stereotype of deformities and knowledge of the mechanisms of cranial deformations. However, synostosis of unilateral lambdoid suture, probably due to its lower incidence and similarity with other non-synostotic deformities affecting the posterior portion of the skull, makes its clinical diagnosis more difficult and imprecise. The aim of this study is to evaluate the most easily and accurate clinical characteristics to be recognized in the synostotic occipital plagiocephaly. METHODS: This study consisted of clinical evaluation of eight patients with synostotic occipital plagiocephaly, whose diagnosis was further corroborated by computed tomography. RESULTS: We identified the following: unilateral occipital flattening in eight out of eight patients (100 %), bulging of ipsilateral mastoid process in eight out of eight (100 %), "edge effect" of ipsilateral lambdoid suture in eight out of eight (100 %), inferior deviation of the ear in eight out of eight (100 %), "Dumbo" ears in eight out of eight (100 %), horizontal slant of the bimastoid line in seven out of eight (87.5 %), tilt of the head viewed from behind in seven out of eight (87.5 %), trapezoidal contour of the skull in top view in six out of eight (75 %), contralateral parietal bossing in six out of eight (75 %), and bossing of the contralateral forehead three out of eight (37.5 %). CONCLUSIONS: The most important clinical features specific to the clinical diagnosis of synostotic occipital plagiocephaly, not present in the positional posterior plagiocephaly, were bulging of the ipsilateral mastoid process, edge effect of the synostotic lambdoid suture, tilt of the head, and slant of the bimastoid line viewed from behind, inferior deviation of the ear, and contralateral parietal bossing.

Frontal-orbital advancement for the management of anterior plagiocephaly.

PURPOSES: The main purposes of this manuscript are to provide an overview of various modalities of surgical correction of anterior plagiocephaly and to emphasize their differences with the classic open frontal-orbital advancement. METHODS AND RESULTS: Advancement of technology provides development of many other ways to achieve the same results. The authors describe the classic open frontal-orbital advancement and compare with other proposed techniques for correction of frontal plagiocephaly. The main limitation of the use of new forms of treatment of the anterior plagiocephaly is the age of the patient. There is still no consensus on criteria for quantitative evaluation of surgical results, and new forms of treatment do not present results with long follow-up. CONCLUSION: Frontal-orbital advancement is the preferred procedure to correct unicoronal synostosis due to its universal indication regardless of the age and degree of deformation of the anterior plagiocephaly.

Hydrocephalus in neurocysticercosis.

INTRODUCTION: Cysticercosis (CC) is the most important of the parasitic diseases of the central nervous system due to its high incidence in the world. CC is the infection with the larval cysts of Taenia solium. It is the most common helminthic infection of the nervous system and is endemic in most underdeveloped countries as well as in industrialized nations. It is estimated that approximately 50,000 people die every year from neurocysticercosis (NCC) worldwide. DISCUSSION: Humans with CC are incidental intermediate hosts, which replace the pig in the life cycle of the T. solium. Children are more frequently affected by parenchyma infestation of cysticercus, of which the main clinical manifestation is epilepsy. Hydrocephalus is more common in adults and is caused by cerebrospinal fluid blockage by ventricular cysts and inflammatory reactions (ependimitis/arachnoiditis). Treatment should be individualized based on clinical presentation, degree of infestation, location and viability of cysticercus, and host response. Hydrocephalus can be controlled only by removal of obstructive intraventricular cysts or associated with either ventriculoperitoneal shunt or endoscopic third ventriculostomy. The degree of infestation and complications related to the shunt represents the most important prognostic factors in the outcome of NCC.

Idiopathic distal lenticulostriate artery aneurysm in a child.

The authors describe a rare case of idiopathic distal lenticulostriate artery (LSA) aneurysm in a 5-year-old boy who presented in the emergency department with a sudden onset of headache. Admission computed tomography scans revealed an intracerebral hemorrhage in the left caudate nucleus with intraventricular extension. Angiographic studies demonstrated a left medial LSA aneurysm. The patient underwent a left parasagittal frontal craniotomy, the lateral ventricle was accessed via the anterior transcallosal approach, and the aneurysm was removed after sectioning of the parent vessel. The child left the hospital after 5 days; at that time he was asymptomatic and without motor impairment. The optimum treatment of aneurysms involving small perforating arteries is controversial and depends mainly on the causative factors. The pathogenesis and treatment of these unusual aneurysms are discussed.

Traumatic brain lesions in newborns.

METHODS: A literature review using the PubMed data base, MEDLINE, EMBASE, Science Direct, The Cochrane Database, Google Scholar, and clinical trials. Selected papers from 1922 to 2016 were studied. We selected 109 papers, through key-words, with inclusion and exclusion criteria. DISCUSSION: This paper discusses the risk factors for birth trauma, the anatomy of the occipito-anterior and vertex presentation, and traumatic brain lesions. CONCLUSION: Birth-related traumatic brain injury may cause serious complications in newborn infants. Its successful management includes special training, teamwork, and an individual approach.

High Intra-Abdominal Pressure Secondary to Obesity as a Determining Factor for Ventriculoperitoneal Shunt Malfunction

The ventriculoperitoneal shunt (VPS) is an established treatment for hydrocephalus. The functioning of the system requires a pressure difference between the cranial and abdominal cavities. The VPS can be particularly problematic in patients with increased intra-abdominal pressure (IAP). We report the case of a 16-year-old girl with VPS since she was 2 months old due to hydrocephalus secondary to myelomeningocele. The patient had been asymptomatic ever since, but she sought the emergency service with intermittent headache and vomiting. A non-enhanced brain tomography, a shunt trajectory X-ray and an abdominal ultrasound revealed no cause of system malfunction. In view of the persistent clinical picture, a revision of the shunt was performed, which revealed adequate intraoperative functioning. She returned with the same symptoms two weeks after surgery. The patient was obese (body mass index [BMI]: 48). We hypothesized intermittent valve malfunction due to increased intra-abdominal pressure. She underwent a ventriculoatrial shunt, without intercurrences. In the postoperative period, the patient presented transient tachycardia and was asymptomatic at the 6-month follow-up. Obesity should be considered an important variable for the inadequate functioning of the VPS due to increased IAP and catheter dystocia to the extraperitoneal cavity. Studies have already correlated the IAP with the BMI, which reaches between 8 mm Hg and 12 mm Hg in obese individuals. Therefore, the BMI can be considered during the selection of valve pressure in systems with non-adjustable valves to prevent insufficient drainage. The recognition of obesity as a cause of VPS malfunction is fundamental to avoid unnecessary surgeries and intermittent malfunction of the system.

A derivação ventriculoperitoneal (DVP) é um tratamento estabelecido para a hidrocefalia; contudo, algumas variáveis podem influenciar na eficácia desta modalidade. O funcionamento do sistema requer uma diferença de pressão entre as cavidades craniana e abdominal. A DVP pode ser particularmente problemática em pacientes com aumento da pressão intra-abdominal (PIA). Neste artigo, relatamos o caso de uma paciente do sexo feminino, de 16 anos, portadora de DVP desde os 2 meses de idade por hidrocefalia secundária a mielomeningocele. Desde então assintomática, procurou o pronto-socorro com queixa de cefaleia e vômitos intermitentes. Uma tomografia de crânio sem contraste, um raio X (RX) do trajeto do cateter distal, e uma ultrassonografia (USG) abdominal não evidenciaram a causa do mau funcionamento do sistema. Diante do quadro persistente, realizou-se uma revisão da derivação, que mostrou funcionamento adequado no período intraoperatório. A paciente retornou com os mesmos sintomas duas semanas após a cirurgia. A paciente era obesa (índice de massa corporal [IMC]: 48). Aventou-se possível funcionamento intermitente da válvula pelo aumento da PIA. A paciente foi submetida a uma derivação ventrículo-atrial, que foi realizada sem intercorrências. No pós-operatório, ela apresentou quadro transitório de taquicardia, e não apresentou sintomas no acompanhamento feito depois de 6 meses. A obesidade deve ser considerada uma variável importante para o funcionamento inadequado da DVP, pelo aumento da PIA e pela associação com distocia do cateter para a cavidade extraperitoneal. Estudos já correlacionaram a PIA com o IMC, que pode atingir entre8 mm Hg e 12 mm Hg em obesos. Logo, o IMC pode ser considerado na seleção da pressão da válvula em sistemas com válvulas não ajustáveis, para prevenir a drenagem insuficiente. O reconhecimento da obesidade de risco para o mau funcionamento da DVP é fundamental para evitar cirurgias desnecessárias e o mau funcionamento intermitente do sistema.

Traumatic brain lesions in newborns / Lesões cerebrais traumaticas em recém nascidos

ABSTRACT The neonatal period is a highly vulnerable time for an infant. The high neonatal morbidity and mortality rates attest to the fragility of life during this period. The incidence of birth trauma is 0.8%, varying from 0.2-2 per 1,000 births. The aim of this study is to describe brain traumas, and their mechanism, anatomy considerations, and physiopathology of the newborn traumatic brain injury. Methods A literature review using the PubMed data base, MEDLINE, EMBASE, Science Direct, The Cochrane Database, Google Scholar, and clinical trials. Selected papers from 1922 to 2016 were studied. We selected 109 papers, through key-words, with inclusion and exclusion criteria. Discussion This paper discusses the risk factors for birth trauma, the anatomy of the occipito-anterior and vertex presentation, and traumatic brain lesions. Conclusion Birth-related traumatic brain injury may cause serious complications in newborn infants. Its successful management includes special training, teamwork, and an individual approach.

RESUMO O período neonatal é um período vulnerável para o recém nascido. As altas taxas de morbidade e mortalidade neonatal atestam a fragilidade da vida durante esta fase. Trauma durante o nascimento é de 0,8%, variando de 0,2 a 2 por 1000 nascimentos. O objetivo deste estudo é descrever o tocotraumatismo, seu mecanismo, considerações anatômicas e fisiopatologia da lesão em recém nascido. Métodos Revisão da literatura utilizando base de dados PubMed, MEDLINE, EMBASE, Science Direct, The Cochrane Detabase, Google Scolar, ensaios clínicos. Os trabalhos selecionados foram de 1922 a 2016. Foram selecionados 109 trabalhos, através de palavras-chave, inclusão e critérios de exclusão. Discussão Este artigo discute os fatores de risco para o trauma do nascimento, a anatomia da apresentação do vértex occipto-anterior e as lesões traumáticas cerebrais. Conclusão Lesão cerebral traumática no nascimento pode causar complicações graves nos recém-nascidos. O tratamento desta condição deve ser especializado, envolvendo trabalho, equipe e abordagem individualizado