Malformations In Children Research Articles

Computed Tomography of Congenital Lung Malformations in Children: A Primer for Radiologists

Computed Tomography of Congenital Lung Malformations in Children: A Primer for Radiologists

Associated Malformations in Children with Orofacial Clefts in Portugal: A 31-Year Study.

Background:Orofacial clefts are among the most common congenital craniofacial malformations and may be associated with other birth defects. However, the proportion and type of additional anomalies vary greatly between studies. This study assessed the prevalence and type of associated congenital malformations in children with orofacial clefts, who attended the largest cleft lip and palate tertiary referral center in Portugal.Methods:Consecutive children with orofacial clefts who attended at least 1 consultation in our Clefts Unit between 1981 and 2012 were studied. Demographic and clinical data regarding the number and type of associated malformations were retrospectively collected and analyzed.Results:Of the 701 patients studied, 219 (31.2%) had associated congenital malformations. These malformations were more frequent in children with cleft palate (43.4%) than in children with cleft lip and palate (27.5%) or with cleft lip only (19.4%). Within the group with associated anomalies, 73 cases (33.3%) had conditions related with known chromosomal defects, monogenic syndromes or sequences, and 146 cases (66.7%) had multiple congenital anomalies of unknown origin. From those, head and neck malformations were the most common (60.3%), followed by malformations in the cardiovascular (28.3%) and musculoskeletal systems (26%).Conclusions:The overall prevalence of associated malformations of nearly 1 in 3 children with orofacial clefts stressed the need for a comprehensive evaluation of these patients by a multidisciplinary cleft team. Moreover, one-third of the children had multiple congenital anomalies of known origins. Thus, early routine screening for other malformations and genetic counseling might be valuable for orofacial clefts management.

Capillary Malformations Treated With Sequential Pulsed Dye and Nd:YAG Laser Therapy: A Retrospective Study

Introduction and objectiveCapillary malformations are the most common vascular malformations in childhood. The current treatment of choice is pulsed dye laser (PDL) therapy, but this frequently does not result in complete resolution. The search for alternative treatment strategies thus continues. In this study we describe our experience with the use of sequential dual-wavelength PDL and Nd:YAG laser therapy in patients with capillary malformations. Material and methodsWe conducted a retrospective, descriptive study of patients with capillary malformations treated with dual-wavelength PDL and Nd:YAG laser therapy between 2006 and 2011. Four dermatologists rated the effectiveness of treatment on a scale of 10 to 0. We also investigated the potential value of the following factors as predictors of better treatment response: sex, malformation size and color, and presence of associated hypertrophy. Adverse effects were also analyzed. ResultsWe studied 71 patients and most of them experienced a statistically significant improvement after treatment. More favorable responses were observed for violaceous malformations, lesions with associated hypertrophy, and smaller lesions. Adverse effects were reported for 26.76% of patients, and the most common effect was the appearance of isolated areas of skin atrophy. ConclusionsWe consider that sequential dual-wavelength PDL and ND:YAG laser therapy is an effective alternative for treating capillary malformations in selected patients.

Congenital Pulmonary Malformations in Children: Diagnosis and Management in a Pediatric Department in a Low Source Country

Congenital pulmonary malformations (CPM) comprise a group of anatomical abnormalities of the respiratory tree including congenital cystic malformations, bronchopulmonary sequestrations, bronchogenic cyst, bronchial atresia and congenital lobar emphysema.Socioeconomic, demographic, and hospital factors were determinants of survival of CPM. Antenatal diagnosis should be developed in order to improve management and prognosis of CPM in country with low resource.

CT-Scan Presentations of Brain Malformations in Children: About Three Cases in Regional Hospital of Ngaoundere-Cameroon

Brain malformations are rare, difficult to diagnose and have unpredictable evolution. They are the major causes of epilepsy, psychomotor development abnormalities and other neurological disorders. The neuroimaging technique of choice for diagnosis of these malformations is magnetic resonance imaging (MRI), but unfortunately MRI is expensive, and is not available in a poor resource country like Cameroon. CT scan associated to clinical signs can help to suspect or to confirm a malformation. The authors report here three cases of malformations discovered during cranial CT scan at the regional hospital of Ngaoundéré. They are Dandy Walker malformation, Sturge Weber’s disease and hemimegalencephaly. These cases contribute to the knowledge of this rare event, and emphasize the importance of CT scan on their diagnosis in the absence of MRI.

Sonographic Characteristics of Congenital Pulmonary Airway Malformations in Children: A Report of 21 Cases and Review of Literature

Sonographic Characteristics of Congenital Pulmonary Airway Malformations in Children: A Report of 21 Cases and Review of Literature

Abdominal Lymphatic Malformation in Children

Abdominal Lymphatic Malformation in Children

The implications of Milrinone Lactate in the Postoperative Clinical Management of Surgical Interventions for Congenital Heart Malformations in Children

The purpose of the study was to prove to efficacy of Milrinone in the management of open heart surgery in children with congenital heart malformations, the link between Milrinone efficacy and the prevention of low cardiac output syndrome and Milrinone side effects. We conducted a retrospective study on a group of 24 patients, admitted to the Tg Mures Emergency Institute for Cardiovascular Diseases and Transplant, between August 2016 and February 2017. Milrinone was administered to children that underwent open heart surgery for different congenital heart malformations, using doses between 0.25 and 0.75 mcg per kg bodyweight, in continuous intravenous drip, before de-clamping of the Aorta, the procedures being conducted in extracorporeal circulation. We recorded demographic data, biological parameters of renal function, myocardial function and hemodynamic parameters, before and after surgery. The administration of Milrinone determined a reduction of incidence of low cardiac output syndrome, registering only 4 deaths, the survival rate being 83.33%, 13 cases presenting complications. Postoperatively we registered a significant improvement of the mean heart rate. Milrinone proved efficient in the re-establishment of hemodynamic parameters in patients with this type of clinical manifestations. Using Milrinone in children that undergo open heart surgery determines a decrease in incidence of low cardiac output syndrome, its� presence in the pharma market being necessary.

Malformaciones capilares tratadas con aplicación secuencial de láser de colorante pulsado y Nd:YAG: estudio retrospectivo

Introduction and objectiveCapillary malformations are the most common vascular malformations in childhood. The current treatment of choice is pulsed dye laser (PDL) therapy, but this frequently does not result in complete resolution. The search for alternative treatment strategies thus continues. In this study we describe our experience with the use of sequential dual-wavelength PDL and Nd:YAG laser therapy in patients with capillary malformations. Material and methodsWe conducted a retrospective, descriptive study of patients with capillary malformations treated with dual-wavelength PDL and Nd:YAG laser therapy between 2006 and 2011. Four dermatologists rated the effectiveness of treatment on a scale of 10 to 0. We also investigated the potential value of the following factors as predictors of better treatment response: sex, malformation size and color, and presence of associated hypertrophy. Adverse effects were also analyzed. ResultsWe studied 71 patients and most of them experienced a statistically significant improvement after treatment. More favorable responses were observed for violaceous malformations, lesions with associated hypertrophy, and smaller lesions. Adverse effects were reported for 26.76% of patients, and the most common effect was the appearance of isolated areas of skin atrophy. ConclusionsWe consider that sequential dual-wavelength PDL and ND:YAG laser therapy is an effective alternative for treating capillary malformations in selected patients.

Long-Term Follow-Up of Lymphatic Malformations in Children Treated with Sildenafil.

Lymphatic malformations (LMs) are challenging to treat. Reports on the benefits of sildenafil for LM management have been mixed. This study evaluated long-term clinical outcomes of pediatric LM patients after sildenafil treatment. A retrospective chart review was performed on pediatric LM patients treated with sildenafil in the past 5years. Patients were also contacted to complete a survey of comprehensive questions about their LM after sildenafil and subsequent interventions. Of 12 patients identified, 10 (83.3%) participated in the follow-up survey. The average age was 8years (range 4-16yrs), median treatment duration was 9months, and the average time of follow-up after sildenafil was 4years; one patient is still taking sildenafil. Ten patients surveyed (83.3%) reported positive therapeutic response, with improvement in the size and compressibility of their LM during posttreatment clinical visits. Six received additional interventions (four sirolimus, one sclerotherapy, one surgery) after sildenafil was discontinued, with all but one reporting a positive response to subsequent interventions. Minor side effects at the time of sildenafil treatment included mild flushing, dizziness, and transient nausea, but no adverse effects were reported at the long-term follow-up. This is the first report of long-term follow-up of pediatric LM patients treated with sildenafil. Our findings suggest that sildenafil is beneficial for the symptomatic treatment of LMs. Additional analysis on the role of sildenafil as adjuvant therapy is necessary to optimize the use of this medication in the management of complex LMs.

The Public Health Burden of Lymphatic Malformations in Children: National Estimates in the United States, 2000-2009.

Describe inpatient resource utilization trends in children with lymphatic malformations using a national database. Kids' inpatient database, years 2000-2009. Subjects included children 18 years and under with International Classification of Diseases (ICD), 9th revision code: 228.1-lymphangioma, any site. Data elements were extracted and used to calculate related inpatient costs and trended over time. No significant increase in admission rates was noted over time, p = 0.5128. Average total charges per admission were $30,995. There is a clear and increasing trend of total charges per admission; even when adjusted for inflation, this has increased disproportionately. In 2009, the mortality rate increased to 2.58%, which was significantly higher than in previous years, p = 0.0346. In multivariate analysis for mortality, the only factor that was noted to be significant was between survey years 2000 and 2009. The odds ratio (OR) for mortality was 2.97, 95% CI: [1.423-6.202], which indicated that there was an almost three times higher likelihood of mortality in 2009 than in 2000. Admission rates appear to remain stable for pediatric inpatients with lymphatic malformations but resource utilization related to charges has been increasing from 2000 to 2009. Controlling for inflation does not explain our observed trend in total charge increases. Previously, surgical resection was the most commonly performed procedure, and now the trend has shifted away from operative intervention. Mortality rates, while low, experienced a rise in 2009. Further investigation may be warranted.

Relation of Stress during Pregnancy with Congenital Malformations in Children Admitted in Various Clinical Settings in Lahore

<p><strong>Objectives:</strong><strong> </strong>To study relation of stress in pregnancy and congenital malformations in newborns delivered at various hospitals of public sector in Lahore.<strong></strong></p><p><strong>Materials and Methodology:</strong><strong> </strong>Descriptive cross sectional study design was used. The study was conducted at various clinical settings of Lahore for one month. Mothers of 40 malformed neonates were taken as sample. A data collection tool (a questionnaire) was developed and the mothers were interviewed regarding various type of stress such as poor socio-economic conditions, divorce and separation, death of a close relative, post dramatic stress disorders, serious illness, catastrophic events and pregnancy related stress including history of multiple miscarriages. Questionnaire was pretested before the data. SPSS computer software was used for entry, compilation and analysis of data.<strong></strong></p><p><strong>Results:</strong><strong> </strong>Out of 40 subjects, 14 (35%) of mothers suffered from stress while 26 (65%) did not suffer from stress during pregnancy. Out of 14, 12.5% were having facial defects (cleft lip, cleft palate), 10% had neural tube defects while those with cvs, git, and urogenital defects were 7.5%. 2.5% and 2.5% respectively.</p><p><strong>Conclusion:</strong><strong> </strong>This study suggests that prevalence of congenital malformations has a significant association with the events causing stress in life during pregnancy. Therefore it is one of the overlooked but important aggravating factor in the development of malfunctions. Out of 40 subjects 35% of mothers suffered from stresses like poor socioeconomic conditions, separation and deaths in the family. The number of conotruncal, or facial and neural tube defects was significantly higher in our subjects having stress.<strong></strong></p>

Choledochal Malformation in Children: Lessons Learned from a Dutch National Study

IntroductionA choledochal malformation (CM) is a rare entity, especially in the Western world. We aimed to determine the incidence of CM in the Netherlands and the outcome of surgery for CM in childhood.MethodsAll pediatric patients who underwent a surgical procedure for type I–IV CM between 1989 and 2014 were entered into the Netherlands Study group on choledochal cyst/malformation. Patients with type V CM were excluded from the present analysis. Symptoms, surgical details, short-term (<30 days) and long-term (>30 days) complications were studied retrospectively.ResultsBetween January 1989 and December 2014, 91 pediatric patients underwent surgery for CM at a median age of 2.1 years (0.0–17.7 years). All patients underwent resection of the extrahepatic biliary tree with restoration of the continuity via Roux-en-Y hepaticojejunostomy. Twelve patients (12%) were operated laparoscopically. Short-term complications, mainly biliary leakage and cholangitis, occurred in 20 patients (22%), without significant correlations with weight or age at surgery or surgical approach. Long-term postoperative complications were mainly cholangitis (13%) and anastomotic stricture (4%). Eight patients (9%) required radiological intervention or additional surgery. Surgery before 1 year of age (OR 9.3) and laparoscopic surgery (OR 4.4) were associated with more postoperative long-term complications. We did not observe biliary malignancies during treatment or follow-up.ConclusionSurgery for CM carries a significant short- and long-term morbidity. Given the low incidence, we would suggest that (laparoscopic) hepatobiliary surgery for CM should be performed in specialized pediatric surgical centers with a wide experience in laparoscopy and hepatobiliary surgery.

Congenital Malformations in Children of Epileptic Mothers with Intrauterine Exposures to Antiepileptic Drugs and Comparison with a Control Group. Update from Pinar del Rio Epilepsy Pregnancy Multicenter Study, Experience 1996–2016. (P6.237)

Congenital Malformations in Children of Epileptic Mothers with Intrauterine Exposures to Antiepileptic Drugs and Comparison with a Control Group. Update from Pinar del Rio Epilepsy Pregnancy Multicenter Study, Experience 1996–2016. (P6.237)

Spectrum of Congenital CNS Malformations in Children with Seizure Disorder

Spectrum of Congenital CNS Malformations in Children with Seizure Disorder

A Diagnostic Program of Vascular Tumor and Vascular Malformations in Children According to Modern Classification.

The study included 205 inpatient children within the time period of the years 2010-2015. Types of vascular anomalies (VAs), age of patients, diagnostic procedures, and anatomical localization of VAs were analyzed. 65 patients of first year of life had vascular tumors, with prevalence of infantile hemangiomas (IHs) in 57 (87.7%) patients. 45 children had IHs localized within soft tissues, whereas 7 patients suffered from IHs of the liver, and 5 children from IHs of the respiratory tract. Most patients with soft tissue IHs were diagnosed only with ultrasound; СT or MRI diagnostics were performed on 5 (8.8%) patients, and biopsy was carried out in 2 (4.4%) children. Vascular malformations (VM) were diagnosed in 140 (68.3%) patients. Ultrasound investigation (US) was the screening method. MRI was performed to confirm the diagnosis of low-flow VM, whereas for high-flow VM CT angiography and selective angiography were useful. Venous malformations were diagnosed in 17 (12.1%) patients, and 112 (80.0%) had cystic LM, among them children under the age of 2 years prevailed. Arteriovenous malformations were diagnosed in 5 (3.8%) patients, ages 2-14 years. Clinical manifestations of vascular anomalies have clear age features. Among hospitalized children vascular tumors add up to 31.7% and VM - up to 68.3%.

Relationship Between Sacral Dimples and Spinal Cord Malformations in Children

Relationship Between Sacral Dimples and Spinal Cord Malformations in Children

Congenital Visceral Malformations in Children with Down Syndrome (DS) Followed in the Pediatric Unit of the CNHU, Cotonou, Bénin

Introduction: Mortality in Down syndrome (DS) remain high and often in relation with visceral malformations. The aim of this work was to describe the congenital visceral malformations associated with DS in children followed in the pediatric unit of National Health Teaching Hospital of Cotonou, in order to improve their management.Patients and methods: It was a prospective and descriptive survey conducted on children who were treated for DS at the pediatric unit of the National Health Teaching Hospital of Cotonou from October 2015 through July 2016. All children had a diagnosis of DS based on karyotype with trisomy 21 and underwent heart, gastrointestinal and kidney ultrasonographies.Results: A total of 36 children were included. The sex ratio was 1.25 and infants under 12 months were the most represented age group (55.56 %). The mean age at diagnosis was 18.8 months. The majority of mothers were aged than 35 years. Clinical features were dominated by up slanting palpebral fissures and hypertelorism. The visceral malformations were observed only in heart with a frequency of 55.55%. Atrio-ventricular septal defect (27.78%) and patent ductus arteriosus (16.76%) were predominant. Nondisjunction trisomy 21 was found in all children.Conclusion: Congenital visceral malformations associated to DS in children at pediatric unit of the National Health Teaching Hospital of Cotonou were exclusively cardiac with atrioventricular septal defect as major type. Early diagnosis of DS with malformations screening are essential for better management of affected children.

Prevalence and CT-Scan Presentations of Brain Malformations in Children at a University-Affiliated Mother and Child Hospital (Cameroon)

Background: Brain malformations (BMs) are congenital abnormalities of the shape or structure of the brain, with corpus callosum agenesis known as the most frequent. Diagnosis of most BMs can be prenatal with ultrasound and fetal magnetic resonance imaging (MRI); post-natal diagnosis is based on transfontanellar ultrasound, CT-scan, and head MRI which is the imaging gold standard technique. MRI has been recently introduced and the CT-scan was previously the reference technique for the diagnosis of BMs in our context. Almost no publication has been made in sub-Saharan Africa on the clinical and CT scan characteristics of Brain malformations in children. Objective: The aim of this study was, in the absence of MRI, to describe the clinical and CT-scan presentations of brain malformations in children at the Yaounde Gynaeco-Obstetric and Pediatric Hospital (YGOPH). Patients and method: This was a cross-sectional descriptive study conducted from February to May 2016 at the YGOPH, including all children of 15-year-old and less with BM diagnosed on CT-scan at YGOPH between April 2006 and March 2016. The studied variables were clinical (age at diagnosis, sex, clinical manifestations) and CT findings (type of cerebral malformation). The data was analyzed using the SPSS 20.0 software with the estimation of hospital prevalence of BMs, frequencies and associations. The chi-square test was used to seek for an association between variables. The threshold of significance was p p = 0.007) with disorders of brain separation (DBS) while the presence of cutaneous lesions was associated (p = 0.013) with anomalies of the cortical development (ACD) especially tuberous sclerosis complex. Conclusion: BMs are infrequent, dominated by tuberous sclerosis complex and agenesis of the corpus callosum. They are mainly revealed by convulsions and psychomotor retardation. Efforts are needed to improve antenatal diagnosis and facilitate access to cerebral MRI.

Outcome of Laparoscopic Treatment of Anorectal Malformations in Children.

Operative correction of anorectal malformations (ARMs) remains a challenge in pediatric surgery. The study aimed to evaluate the outcomes of laparoscopic treatment of ARM in children. From 2007 to 2014, we performed 104 laparoscopic-assisted anorectal pull-through procedures in children with a mean age of 11.3±0.4months and ARMs. Clinical assessment, surgical durations, complications, and postoperative outcome were investigated. The mean duration of the operation was 126.5±17.2min. Mean intraoperative bleeding was 20±5.7g. Three (2.9%) children required conversion to laparotomy. One (0.9%) child developed a pelvic abscess, requiring an additional intervention. Three to six months after laparoscopic-assisted anorectal pull-through, 72 patients were hospitalized for stoma closure. Good functional results were achieved in 39 (54.2%) patients. On examination of the perineum, 11 (15.3%) patients were found to have mucosal prolapse. The circular symmetric anal reflex to tactile stimulation was confirmed in 53 (73.6%) patients and a tactile weakened anal reflex in 5 (6.9%) children. At the 1-year follow-up, constipation was present in nine (12.5%) patients and soling in two (2.8%) patients. A barium enema study performed after the operation showed good outcomes in 67 (93.1%) patients and fair outcomes in 5 (6.9%) patients. None had a poor outcome. Our experience confirms that laparoscopic-assisted anorectal pull-through enabled complete correction of ARM in the meanwhile avoiding damage to the rectum and anus. Also, the technique of double bipolar myostimulation of muscle complex in laparoscopic ARM might lead to these better results.