Incidence Of Malformations Research Articles

Anorectal Malformations at a University Teaching Hospital in Southern Nigeria

Ano-rectal malformations comprise a wide spectrum of diseases, which can affect males and females, and involve the distal anus and rectum as well as the urinary and genital tracts. The incidence, type or variety of malformations varies from one region or geographical location to another. Ano-rectal anomalies tend to be associated with other congenital anomalies. The aim of this study is to determine the incidence, presentation and types of ano-rectal malformations and their treatment outcome. It is a prospective observational study from March 2012 to February 2019. Information obtained from cases of ano-rectal anomalies who presented to our health institution during the study period was entered into a proforma. Relevant clinical information was obtained and documented in a structured proforma. A total of 60 patients were seen during the study period. Males were 35 (58.3%) while females were 25 (41.7%). Age at presentation ranged from 2 hours -15 years. High anomalies were seen in 34 (56.7%), low anomalies 18 (30%) and intermediate 8 (13.3%). Associated congenital anomalies were seen in 12 (20%) patients. 28 patients had the traditional three-stage procedure of initial colostomy, PSARP and colostomy closure while 13 patients had double staged procedure of initial colostomy and definitive surgery with colostomy closure at the same time. Fifteen (15) patients had single staged procedure (PSARP) without an initial colostomy. Three (3) patients are awaiting colostomy closure after definitive surgery. Post-Operative complications were seen in 16 patients. A mortality of 10 (16.7%) was observed.

Anorectal Malformations at a University Teaching Hospital in Southern Nigeria

Ano-rectal malformations comprise a wide spectrum of diseases, which can affect males and females, and involve the distal anus and rectum as well as the urinary and genital tracts. The incidence, type or variety of malformations varies from one region or geographical location to another. Ano-rectal anomalies tend to be associated with other congenital anomalies. The aim of this study is to determine the incidence, presentation and types of ano-rectal malformations and their treatment outcome. It is a prospective observational study from March 2012 to February 2019. Information obtained from cases of ano-rectal anomalies who presented to our health institution during the study period was entered into a proforma. Relevant clinical information was obtained and documented in a structured proforma. A total of 60 patients were seen during the study period. Males were 35 (58.3%) while females were 25 (41.7%). Age at presentation ranged from 2 hours -15 years. High anomalies were seen in 34 (56.7%), low anomalies 18 (30%) and intermediate 8 (13.3%). Associated congenital anomalies were seen in 12 (20%) patients. 28 patients had the traditional three-stage procedure of initial colostomy, PSARP and colostomy closure while 13 patients had double staged procedure of initial colostomy and definitive surgery with colostomy closure at the same time. Fifteen (15) patients had single staged procedure (PSARP) without an initial colostomy. Three (3) patients are awaiting colostomy closure after definitive surgery. Post-Operative complications were seen in 16 patients. A mortality of 10 (16.7%) was observed.

Developmental toxicity of iron oxide nanoparticles with different coatings in zebrafish larvae

We report on the effect of different coatings (dextran, chitosan, polyethylene glycol, carboxy-silane, and silica) in the toxicity elicited by superparamagnetic iron oxide nanoparticles (SPIONs) in the developing zebrafish (Danio rerio). Animals were exposed to nanoparticle concentrations ranging from 0.125 to 8.0 mM of Fe during the first 5 days after fertilization. Embryotoxicity parameters (survival, hatching rate, and the incidence of anatomical malformations) and behavioral patterns (locomotion during the exploration of a new environment, thigmotaxis, and the escape response to an aversive stimulus) were evaluated. Exposed embryos hatched between 48 and 72 hpf, as expected for the species, but tendencies of either acceleration or delay were observed, depending on the nanoparticle coating. Malformations in exposed and control groups were similar, independent of the coating. Mortality rates were also not significantly affected by exposure to most of the coated SPIONs, except for animals treated with chitosan-coated nanoparticles, which induced 100% mortality at concentrations higher than 2 mM. A similar trend was observed in the behavioral parameters, in which significant adverse effects were mostly caused by chitosan-coated nanoparticles even at low concentrations. The higher toxicity observed for chitosan-coated particles raises concern and deserves further mechanistic investigations, considering the ample use of this compound in pharmaceutical and biomedical applications.

Positive phototaxis as the cause of jaw malformations in larval greater amberjack, Seriola dumerili (Risso, 1810): Mitigation by rearing in tanks with low‐brightness walls

Jaw malformations are a serious problem in fingerlings of the greater amberjack, Seriola dumerili. To establish a countermeasure against these malformations, we conducted a larval behavioural test to identify their root cause, and we investigated the ability of low-brightness rearing tank walls to control their occurrence. Larval distribution was examined in a horizontally illuminated rectangular transparent aquarium (20 cm × 100 cm × 25 cm), and their distribution was biased towards the light source after this lighting was provided from 3 to 13 days post hatching (dph), indicating positive phototaxis; this response disappeared on 22 dph (there are no data between 13 and 22 dph). Larvae were observed to swim phototactically towards the light source and repeatedly collided with the tank wall (walling behaviour). To investigate the ability of wall brightness to control the incidence of jaw malformations, we compared their incidence in a rearing trial with different Munsell colour values for the tank walls: 2.11 (black), 5.85 (grey) and 9.52 (white). The incidence rate of jaw malformations at 25 dph was significantly lower in the tanks with lower-brightness walls, that is, 5.8%, 22.5% and 26.8% for black, grey and white respectively. Larval walling behaviour was most intense for white, followed in order by grey and black. These results indicate that positive phototaxis elicits larval walling behaviour, causing jaw malformations, and that low-brightness tank walls control this process.

ISUOG Interim Guidance on 2019 novel coronavirus infection during pregnancy and puerperium: information for healthcare professionals.

ISUOG Interim Guidance on 2019 novel coronavirus infection during pregnancy and puerperium: information for healthcare professionals.

Mowat-Wilson syndrome in a Chinese population: A case series.

Mowat-Wilson syndrome (MWS) is characterized clinically by a distinctive facial gestalt, intellectual disability, microcephaly, epilepsy, and nonobligatory congenital malformations such as Hirschsprung disease, urogenital anomalies, congenital heart disease, eye malformations. This article summarized the clinical features and molecular findings of 15 Chinese MWS patients. The results revealed a higher incidence of congenital heart disease in Chinese MWS patients compared to that previously reported in Caucasian cohorts, while the incidence of Hirschsprung disease and genitourinary malformation appeared to be lower. This suggests possible ethnicity-related modifying effects in the MWS phenotype.

Neonatal outcome of children born after ICSI with epididymal or testicular sperm: A 10-year study in China

Some studies show that children born after ICSI with non-ejaculated sperm are at increased risk of birth defects, other studies hold the opposite view. Does neonatal outcome including congenital malformations in children born after ICSI with percutaneous epididymal sperm aspiration (PESA) and testicular sperm aspiration (TESA) differ from neonatal outcome in children born after ICSI with ejaculated sperm? In this study, we examined the data from our IVF center from 2006 to 2016, to compare neonatal outcomes and rates of congenital malformations in children born after ICSI with different sperm origin. The results showed the clinical pregnancy rate and implantation rate of non-ejaculated sperm group were significantly higher (P < 0.001) than ejaculated sperm group. There were 775 clinical pregnancies from non-ejaculated sperm group and 2,486 clinical pregnancies from ejaculated sperm group. Most of the clinical pregnancy outcomes were comparable between non-ejaculated sperm group and ejaculated sperm group (p > 0.05): the miscarriage rate per transfer, ectopic pregnancy rate per clinical pregnancy, induced abortion rate per clinical pregnancy and fetal deaths per clinical pregnancy. However, the live delivery rate per transfer of non-ejaculated sperm group was significantly higher than that of ejaculated sperm group (45.4% vs 36.7%, P < 0.001). Moreover, the comparison between the epididymal sperm, testicular sperm and ejaculated sperm groups showed there were no difference in the incidence of congenital malformations of babies live birth. Among singleton gestation live births, there were more girls than boys in both non-ejaculated sperm and ejaculated sperm group. In conclusion, the present study clearly showed no statistical increased risk in neonatal outcomes of newborns were found in the ICSI treatment with epididymal or testicular sperm. It may provide information for consultation for ICSI treatment in PESA or TESA patients.

Nėštumų ir gimdymų baigtys po pagalbinio apvaisinimo

Santrauka. Tyrimo tikslas. įvertinti moterų, pastojusių po pagalbinių apvaisinimo budų (PAB), neštumo, gimdymo eigą ir rezultatus bei išanalizuoti naujagimių baigtis. Tyrimo medžiaga ir metodai. 2016–2018 m. laikotarpiu retrospektyviai tirtos 427 pacientės, gimdžiusios Lietuvos sveikatos mokslų universiteto ligoninės Kauno klinikose (toliau – Kauno klinikos), suskirstytos į tiriamąją (n=215) bei kontrolinę (n=212) grupes. Tiriamoji grupė – tai moterys pastojusios po PAB, kontrolinė grupė – tai atsitiktinai atrinktos savaime pastojusios moterys. Analizuoti neštumo, gimdymo eigos ir naujagimių buklės duomenys remiantis gimdymo registro, gimdymo knygų ir ligos istorijomis. Rezultatai. Daugiavaisiai neštumai PAB grupeje sudarė 37,7 proc., o kontrolinėje grupėje – 10,4 proc., p&lt;0,001. Neštumo komplikacijų: preeklampsijos (lengvos, sunkios), neščiųjų hipertenzijos, neščiųjų diabeto dažnis tarp grupių reikšmingai nesiskyrė, p&gt;0,05. Placentos patologijos (įaugusi placenta ir placentos pirmeiga) dažnesnėsPAB grupėje, atitinkamai – p=0,012 ir p=0,004. Priešlaikinio vaisiaus dangalų plyšimo dažnis tarp grupių nesiskyrė. Vaisiaus ydų dažniai abiejose grupėse nesiskyrė, p=0,302. Cezario pjūvio operacijų (CPO) dažnis didesnis PAB grupėje ir siekia 49,8 proc., p=0,016. Placentos atšokos dažnis grupėse nesiskyrė. Apgar balų vidurkis 1 ir 5 min. grupėse nesiskyrė, p1&lt;0,994 ir p5&lt;0,484, tačiau PAB grupėje buvo didesnis neišnešiotų naujagimių, p=0,015, vaisiaus augimo sulėtejimo (VAS), p=0,005 bei mažesnis stambių vaisių dažnis, p=0,005. Išvados. Svarbiausios su PAB susijusios komplikacijos yra daugiavaisiai neštumai, CPO, su placenta susijusios patologijos, priešlaikinis gimdymas, vaisiaus augimo sulėtėjimas.

Obstetric anesthesia in China: associated challenges and long-term goals.

Obstetric anesthesia in China: associated challenges and long-term goals.

A 20-year long term experience of the Italian Diamond-Blackfan Anaemia Registry: RPS and RPL genes, different faces of the same disease?

Diamond-Blackfan anaemia (DBA) is a rare and heterogeneous disease characterised by hypoplastic anaemia, congenital anomalies and a predisposition for malignancies. The aim of this paper is to report the findings from the Italian DBA Registry, and to discuss the Registry's future challenges in tackling this disease. Our 20-year long work allowed the connection of 50 Italian Association of Paediatric Haematology and Oncology (AIEOP) centres and the recruitment of 283 cases. Almost all patients have been characterised at a molecular level (96%, 271/283), finding a causative mutation in 68% (184/271). We confirm the importance of determination of erythrocyte adenosine deaminase activity(eADA) and of ribosomal RNA assay in the diagnostic pipeline and characterisation of a remission state. Patients with mutations in largeribosomal subunitprotein (RPL) genes had a significant correlation with the incidence of malformations, higher eADA levels and more severe outcomes, compared to patients with mutations in small ribosomal subunit protein (RPS) genes. Furthermore, as a consequence of our findings, particularly the incidence of malignancies and the high percentage of patients aged >18years, we stress the importance of collaboration with adult clinicians to guarantee regular multi-specialist follow-up. In conclusion, this study highlights the importance of national registries to increase our understanding and improve management of this complex disease.

Down-regulation of the insulin signaling pathway by SHC may correlate with congenital heart disease in Chinese populations.

Congenital heart disease (CHD) is one of the most common and severe congenital defects. The incidence of fetal cardiac malformation is increased in the context of maternal gestational diabetes mellitus (GDM). Therefore, we wanted to determine whether abnormalities in the insulin signaling pathway are associated with the occurrence of nonsyndromic CHD (ns-CHD). We used digital gene expression profiling (DGE) of right atrial myocardial tissue samples from eight ns-CHD patients and four controls. The genes potentially associated with CHD were validated by real-time fluorescence quantitative PCR analysis of right atrial myocardial tissues from 37 patients and 10 controls and the H9C2 cell line. The results showed that the insulin signaling pathway, which is mediated by the SHC gene family, was inhibited in the ns-CHD patients. The expression levels of five genes (PTPRF, SHC4, MAP2K2, MKNK2, and ELK1) in the pathway were significantly down-regulated in the patients' atrial tissues (P<0.05 for all). In vitro, the H9C2 cells cultured in high glucose (33 mmol/l) expressed less SHC4, MAP2K2, and Elk-1 than those cultured in low glucose (25 mmol/l). Furthermore, the high glucose concentration down-regulated the 25 genes associated with blood vessel development based on Gene Ontology (GO) term enrichment analyses of RNA-seq data. We considered that changes in the insulin signaling pathway mediated by SHC might be involved in the heart development process. This mechanism might account for the increase in the incidence of fetal cardiac malformations in the context of GDM.

NAD deficiency due to environmental factors or gene–environment interactions causes congenital malformations and miscarriage in mice

Causes for miscarriages and congenital malformations can be genetic, environmental, or a combination of both. Genetic variants, hypoxia, malnutrition, or other factors individually may not affect embryo development, however, they may do so collectively. Biallelic loss-of-function variants in HAAO or KYNU, two genes of the nicotinamide adenine dinucleotide (NAD) synthesis pathway, are causative of congenital malformation and miscarriage in humans and mice. The variants affect normal embryonic development by disrupting the synthesis of NAD, a key factor in multiple biological processes, from its dietary precursor tryptophan, resulting in NAD deficiency. This study demonstrates that congenital malformations caused by NAD deficiency can occur independent of genetic disruption of NAD biosynthesis. C57BL/6J wild-type mice had offspring exhibiting similar malformations when their supply of the NAD precursors tryptophan and vitamin B3 in the diet was restricted during pregnancy. When the dietary undersupply was combined with a maternal heterozygous variant in Haao, which alone does not cause NAD deficiency or malformations, the incidence of embryo loss and malformations was significantly higher, suggesting a gene-environment interaction. Maternal and embryonic NAD levels were deficient. Mild hypoxia as an additional factor exacerbated the embryo outcome. Our data show that NAD deficiency as a cause of embryo loss and congenital malformation is not restricted to the rare cases of biallelic mutations in NAD synthesis pathway genes. Instead, monoallelic genetic variants and environmental factors can result in similar outcomes. The results expand our understanding of the causes of congenital malformations and the importance of sufficient NAD precursor consumption during pregnancy.

Abstract TMP119: Incidence, Presentation and Outcomes of Brain Arteriovenous Malformations in Adult Population in United States

Background: Data regarding national estimates of brain arteriovenous malformation (BAVM) among adults in United States data are scant, often biased by limitations of single center and regional studies. Identification of such patients in nationally representative datasets was not possible until recently due to a new specific ICD-10 code. Objective: To characterize inpatient burden, expenditures, associated comorbidities (including intracerebral hemorrhages and seizures) and treatments of BAVM among adults in United States. Methods: Patients with BAVM were identified from the Nationwide Inpatient Sample (NIS) database for the year 2016 using ICD-10 diagnostic code (Q28.2). The primary outcome was to determine the prevalence of BAVM in the adult (18 and above) in-patient population in the United States. Secondary outcomes included determining inpatient mortality, morbidity, comorbidities, hospital length of stay (LOS) and total hospital charges. Outcome was classified by discharge disposition into none to minimal and moderate to severe disability. Results: Of the total 7,135,090 reported hospital discharges in 2016, an estimated 1733 (0.02%) had the diagnosis of BAVM; 1547 were adults with ages 18 or greater (mean age [SD] 54 (18); 783 (50.6%) were men). Patients with BAVM were whites (n= 921, 59.5%), African-Americans (n=224, 14.4%) and Hispanic (n=212, 13.7%). Underlying hypertension was identified in 677 (43.1%) patients. Clinical presentation was intracerebral hemorrhage (n=260, 16.8%), seizures (n=234, 15.1%) and subarachnoid hemorrhage (n=75, 4.8%). Endovascular treatment was performed in 272 (17.5%) patients and surgical treatment was performed in 45 (2.9%) patients. None to minimal disability was seen in 1023 (66.1%) and moderate to severe disability was seen in 469 (30.3%) of the patients. In hospital mortality was 2.59% (n=40). Average LOS (SD) was 4 days (8.6) with mean hospitalization charges (SD) of $121,186 (153553.1). Conclusion: There are 1547 hospital admissions for BAVM among adults in United States every year. The overall outcomes were good with high rates of none to minimal disability but the hospitalization charges were higher than expected and may be important for resource allocation.

Incidence, Prevalence, and Clinical Presentation of Cerebral Cavernous Malformations.

The incidence, prevalence, and mode of presentation of cavernous malformations is important to better understand the disease, educate patients and practitioners, aid in treatment decisions, and to design clinical trials. Prior to the advent of MRI, cavernous malformations were often diagnosed only when a catastrophic event occurred and/or the lesion was removed. With the more frequent diagnostic use of MRI, it has become clear that cavernous malformations are more prevalent than previously thought and many are identified incidentally. The remainder may present to clinical attention with intracerebral hemorrhage, seizure without hemorrhage, or focal neurologic deficit without overt hemorrhage. The precise reason why some cavernous malformations become symptomatic and others remain asymptomatic is not clear. However, evolving data suggests that brainstem location, estrogen use in women, and low vitamin D may play a role in hemorrhagic presentation.

Incidence of Potential Cerebral Cavernous Malformations in Brain Magnetic Resonance Imaging performed During Health Check-up

Incidence of Potential Cerebral Cavernous Malformations in Brain Magnetic Resonance Imaging performed During Health Check-up

Incidence of congenital thoracic malformations detected by prenatal ultrasound.

The increased and earlier use of prenatal ultrasound has facilitated the detection of congenital thoracic malformations (CTMs). Our Pediatric Pulmonology Institute follows an increasing number of patients with CTMs. Thus, we aimed to evaluate and describe prenatal sonographic findings of CTM, and to estimate changes in detection rates of CTMs over a period of 16years. A retrospective, cross-section analysis of prenatal ultrasound (US) screening tests carried out in a large community-based clinic, comparing two periods: 2001-2007 and 2007-2017. A total of 34716 prenatal US were performed at a median gestational age of 15.4 weeks (range, 11.6-23.9) and 15.7 weeks (range, 12-33.6) in 2001-2007 and 2007-2017, respectively. In 2001-2007, 12016 prenatal US tests detected 19 CTMs, compared to 30 CTMs in 22 700 tests in 2007-2017. Detection rates did not change (1.58/1,000 in 2001-2007 versus 1.32/1,000 in 2007-2017, P = 0.64). The most common abnormality was congenital pleural effusion (CPE) (17 cases, 34.7%), followed by congenital pulmonary airway malformation) and congenital diaphragmatic hernia; 13 cases each, 26.5%. Twenty CTMs, mainly congenital diaphragmatic hernia and CPE, were associated with other fetal lesions. Congenital diaphragmatic hernia and CPE tend to appear with multiple lesions and warrant further attention. The incidence rates stayed stable when comparing the last decade to previous years. Thus, the increased referral of CTM can be attributed to an increase in the number of prenatal screening studies performed, rather than a true higher incidence.

Management strategies for cerebral arteriovenous malformation - An institutional experience

Background: Cerebral Arteriovenous Malformation represent a particularly challenging subset of lesions, given their overall low incidence and the high frequency with which some form of multimodality monitoring may be required for complete obliteration. Materials and Methods: We report retrospective analysis of 9 cases with unruptured cerebral arteriovenous malformation who got admitted in the Banbuntane Hotokokai Hospital, Fujita Health University, and Nagoya, Japan .from the year 2014 to 2017.surgical treatment was appropriate craniotomy and resection based on the location of the arteriovenous malformation with multimodality monitoring with Dual Image Video Angiography, Motor Evoked Potential, and FLOW 800. We studied the following factors: Sex, Age, Arteriovenous Malformation size, Location, occurrence of Intracranial Hemorrhage, Seizure type, Duration of Seizure history, Treatment modality, and Arteriovenous Malformation obliteration. We tested for statistical associations between these factors and seizure presentation and outcomes with Clinical follow-up. Outcome was compared with modified Rankin Scale Results: Out of 9 cases operated for intracranial arteriovenous malformation 5 patients were female and 4 were male. Age [ Mean 39.4 + 16.7] of the patients ranged from 20 to 66 , 5 patients were 25 - 40 and 2 were > 65 and Conclusion: The increasing use of advance imaging techniques will increase the incidence of asymptomatic arteriovenous malformations. Non-ruptured arteriovenous malformation Spetzler Martin Grade 1 or Spetzler Martin Grade 2 have a good outcome for microsurgery [modified Rankin Scale and complete obliteration and there is better outcome with microsurgery.

A Five Years Study of Occurrence and Associated Risk Factors for Birth Defects in a Tertiary Care Hospital in Central Nepal

Introduction: Congenital anomalies account for 7.0% of neonatal deaths in Nepal. The present study was carried out to determine the overall rate of congenital malformations, incidence and prevalence in live births, still birth and incidence affecting various organ systems, at Bharatpur Hospital, Nepal.&#x0D; Methods: All the intramural deliveries between Jan 2015 to Dec 2019 were included in the study. All the newborns were looked for congenital malformations after birth within seven days. Antenatal ultrasonography findings were noted. 2D echocardiography was also used for all congenital heart diseases, along with routine X-ray chest. A total of 131 babies with congenital problem were studied and the information was recorded in WHO NBBD Proforma. Data were recorded in MS Excel and SPSS 16 version was used for analysis. &#x0D; Results: Out of the total 60160 deliveries, 131 (0.21% of total birth) were with congenital malformations, sex wise distribution was 65 (49.5%) females and 63 (48.7%) males and three (1.8%) were ambiguous. Oro-facial malformation (49, 37.4%) was the commonest form of malformation followed by the musculoskeletal system (31, 23.6 %), centre nervous system (31, 23.6%) and congenital malformations of genital organs (8, 6.0%). &#x0D; Conclusions: The incidence of congenital malformation in this study was 0.21%. Females were more common than males and oro-facial malformation was the commonest type of malformation. Lack of antenatal visit, lack of folic acid during pre-conception period and low socioeconomic status were the commonest risk factors.

Uranium concentration in umbilical cord may increase the risk for orofacial clefts

BackgroundOrofacial clefts (OFCs) are common kind of congenital malformations. The teratogenicity of uranium (U) has been documented in animal study that maternal exposure to U can increase incidence of external malformations including cleft palate. However, there is limited evidence of the association of in utero exposure to U with OFCs risk in humans. ObjectiveThis study aimed to investigate the association between in utero exposure to U and the risk of OFCs and its subtypes. MethodAll subjects were from a case-control study in Shanxi Province, northern China. Eighty-four OFCs cases and 142 healthy controls were included in this study. We used U concentration in umbilical cord as biomarkers to represent intrauterine exposure, which was detected by inductively coupled plasma mass spectrometry. Unconditional logistic regression was used to investigated the association between U level and the risk of OFCs and its subtypes. ResultsThe median of U concentration in umbilical cord is 0.745 ng/g in case group and 0.455 ng/g in control group. When the U concentration was divided into two categories, high level of U exposure increased the risk of OFCs (OR: 2.08, 95% CI: 1.13–3.86) and its subtype cleft lip with cleft palate (CLP) (OR: 2.72, 95% CI: 1.21–6.14). When divided into three categories, high level of U elevated the risk for OFCs (OR: 2.40, 95% CI: 1.14–5.06) and CLP (OR: 3.04, 95% CI: 1.20–7.74). Meanwhile, a dose-response relationship between the U concentration and the risk of total OFCs (P for trend = 0.009) and CLP (P for trend = 0.007) was found. ConclusionOur study found that in utero exposure to high level of U was associated with increased risk of OFCs and its subtype CLP.

Analysis of pediatric neurosurgical diseases: A single-center study

Objective To summarize the clinical epidemiological characteristics of pediatric neurosurgical diseases at a single center. Methods A total of 5 790 children (≤15 years old) with neurosurgical diseases admitted to Department of Neurosurgery, Xijing Hospital, Air Force Medical University from January 2008 to June 2018 were retrospectively enrolled. Their clinical data were analyzed and included the distribution of various diseases and clinical characteristics such as age, gender and lesion location. Results All 5 790 children included 2 259 cases (39.0%) of congenital malformation, 1 561 cases of tumor (27.0%), 953 cases of trauma (16.5%), 360 cases of cerebrovascular disease (6.2%), 159 cases of inflammation (2.7%), 91 cases of functional disorder (1.6%) and 407 cases of other diseases (7.0%). The gender ratio (male ∶female) was 1.76 ∶1.00. In all kinds of diseases, the proportion of male children was higher than that of female children, and the difference was statistically significant (P<0.001). The overall peak age of onset was 0-3 years old. Congenital malformations and tumors were predominant in 0-3 years old (42.9%) and 13-15 years old (26.3%), respectively. Three most frequent congenital malformations were arachnoid cyst (29.6%), hydrocephalus (28.2%) and tethered cord syndrome (20.0%). Temporal lobe was the most common site of arachnoid cyst (45.4%). Three most common tumors were glioma (19.7%), craniopharyngioma (9.0%) and medulloblastoma (8.8%). The ratios of brain tumors to spinal canal tumors, supratentorial tumors to infratentorial tumors and low-grade tumors to high-grade tumors were 5.61 ∶1.00, 1.73 ∶1.00 and 1.67 ∶1.00, respectively. Primary brain injury(365 cases) accounted for 58.6% of traumatic diseases and 59.2% (the highest proportion) of primary brain injury was brain contusion and laceration. In cerebrovascular diseases, the proportion of spontaneous hemorrhage (27.2%) was the highest. The most common inflammatory disease was inflammatory granuloma(22.6%). In addition, with the increase of age, the incidence of congenital malformation decreased gradually, while the incidence of cerebrovascular diseases increased gradually(both P<0.001). Conclusions Pediatric neurosurgical diseases have unique clinical epidemic characteristics. Congenital malformations and tumors are more common, and the incidence of arachnoid cysts and gliomas seems to be the highest. In various diseases, the incidence of male is higher than that of female, and the peak ages are different. Key words: Child; Neurosurgery; Epidemiology; Single-center study