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Asian Journal of Surgery Jun 2024
Topics: Humans; Fatal Outcome; Postoperative Complications; Encephalocele; Male; Female; Plastic Surgery Procedures
PubMed: 38531742
DOI: 10.1016/j.asjsur.2024.03.115 -
Congenital Malformations in the Moroccan Surveillance System: Contribution to Prevalence Estimation.Global Health, Epidemiology and Genomics 2024Congenital malformations (CMs) are a group of structural or functional anomalies present at birth. These anomalies result in a high rate of mortality, morbidity, and...
BACKGROUND
Congenital malformations (CMs) are a group of structural or functional anomalies present at birth. These anomalies result in a high rate of mortality, morbidity, and disability in children. CMs are a major public health problem and place a heavy burden on healthcare systems in both developed and developing countries including Morocco, which has had a CMs surveillance system in place since 2011. The aim of this study is to determine the prevalence of CMs in Morocco.
METHODS
In this study, the epidemiology of CMs in Morocco was assessed retrospectively using the national surveillance system data gathered from case notification forms from 2017 to 2021.
RESULTS
The main results showed that the prevalence of CMs in Morocco is 3.91/1000 live births (LBs), and the minimum annual prevalence of CMs was reached in 2017 (3.10/1000 LBs) while the maximum annual prevalence was recorded in 2018 (4.55/1000 LBs). The majority of CMs are unspecified CMs (68.55%), neural tube defects (NTDs) account for (21.13%), and orofacial clefts (OFCs) account for (10.32%). In addition, the majority of CMs (61.73%) were from rural areas. According to region, the Dakhla-Oued Eddahab region recorded the highest prevalence of CMs in Morocco, with 8.81/1000 LBs, while the lowest prevalence was recorded in the Rabat-Sale-Kenitra region, with 2.02/1000 LB.
CONCLUSIONS
This study reveals that the national prevalence of CMs is high and may be underestimated, as most of the CMS reported is unspecified. The use of a CM registry with detailed reporting of all CMs and the promotion of preventive measures are urgently recommended.
Topics: Infant, Newborn; Child; Female; Humans; Prevalence; Retrospective Studies; Cleft Lip; Cleft Palate; Neural Tube Defects
PubMed: 38529480
DOI: 10.1155/2024/9570798 -
European Journal of Medical Research Mar 2024The current convention for treatment of children with myelomeningocele (MMC) is timely surgical intervention combined with long-term follow-up by a multidisciplinary...
BACKGROUND
The current convention for treatment of children with myelomeningocele (MMC) is timely surgical intervention combined with long-term follow-up by a multidisciplinary specialized team. This study aims to investigate the outcomes of MMC patients treated at Namazi Hospital.
METHODS
All children presenting to Namazi Hospital with myelomeningocele between May 2001 and August 2020 were eligible for this study. For those with a documented telephone number, follow-up phone surveys with the patient's caregivers, on top of the review of the medical documents were carried out to assess mortality, morbidities, and the functional outcome of the care provided to them.
RESULTS
A total of 125 patients were studied (62 females). All of the patients were followed up for a mean duration of 6.28 years (range 1-23 years). The majority were located in the lumbosacral area. All of the patients underwent postnatal surgical intervention for MMC in Namazi Hospital. Mean age at surgery was 9.51 days. There were statistically significant differences between urinary and bowel incontinence and presence of scoliosis, MMT grading of the lower limbs, school attendance, number of readmissions, and requirement of laminectomy at the initial surgical intervention.
CONCLUSIONS
This study is the first to characterize the long-term outcomes of MMC patients in Iran. This study illustrates that there is a great need for improved access to and coordination of care in antenatal, perioperative, and long-term stages to improve morbidity and mortality.
Topics: Child; Humans; Female; Pregnancy; Infant, Newborn; Meningomyelocele; Follow-Up Studies; Iran; Referral and Consultation; Retrospective Studies
PubMed: 38528560
DOI: 10.1186/s40001-024-01667-0 -
Romanian Journal of Morphology and... 2024Acrania is a fetal malformation characterized by complete or partial absence of the calvaria above the orbits and supraciliary ridge. No exact mechanism is demonstrated... (Review)
Review
Acrania is a fetal malformation characterized by complete or partial absence of the calvaria above the orbits and supraciliary ridge. No exact mechanism is demonstrated for this anomaly but disturbances in mesenchymal migration during the fourth week of development are the most documented. The key sonographic features of acrania are absent calvaria and dorsally bulging brain (Mickey Mouse head). Due to the normal process of ossification of cranial bones, the diagnosis can be established only after 11 weeks of gestation. Early detection is extremely important. The prognosis is extremely poor so elective termination of pregnancy is the treatment of choice. In this paper, we discuss the things we know about pathogeny and ultrasonographic features of fetal cranial malformations based on a case diagnosed late during gestation.
Topics: Pregnancy; Female; Humans; Ultrasonography, Prenatal; Ultrasonography; Neural Tube Defects; Skull; Brain
PubMed: 38527993
DOI: 10.47162/RJME.65.1.16 -
Brazilian Journal of Anesthesiology... 2024Myelomeningocele (MMC) is a neural tube defect disease. Antenatal repair of fetal MMC is an alternative to postnatal repair. Many agents can be used as tocolytics during... (Comparative Study)
Comparative Study
BACKGROUND
Myelomeningocele (MMC) is a neural tube defect disease. Antenatal repair of fetal MMC is an alternative to postnatal repair. Many agents can be used as tocolytics during the in utero fetal repair such as β2-agonists and oxytocin receptor antagonists, with possible maternal and fetal repercussions. This study aims to compare maternal arterial blood gas analysis between terbutaline or atosiban, as tocolytic agents, during intrauterine MMC repair.
METHODS
Retrospective cohort study. Patients were divided into two groups depending on the main tocolytic agent used during intrauterine MMC repair: atosiban (16) or terbutaline (9). Maternal arterial blood gas samples were analyzed on three moments: post induction (baseline, before the start of tocolysis), before extubation, and two hours after the end of the surgery.
RESULTS
Twenty-five patients were included and assessed. Before extubation, the terbutaline group showed lower arterial pH (7.347 ± 0.05 vs. 7.396 ± 0.02 for atosiban, p = 0.006) and higher arterial lactate (28.33 ± 12.76 mg.dL vs. 13.06 ± 6.35 mg.dL, for atosiban, p = 0.001) levels.
CONCLUSIONS
Patients who received terbutaline had more acidosis and higher levels of lactate, compared to those who received atosiban, during intrauterine fetal MMC repair.
Topics: Humans; Retrospective Studies; Terbutaline; Female; Meningomyelocele; Adult; Tocolytic Agents; Pregnancy; Vasotocin; Cohort Studies; Blood Gas Analysis
PubMed: 38521500
DOI: 10.1016/j.bjane.2024.844495 -
Anesthesiology Jun 2024
Topics: Humans; Ultrasonography, Interventional; Neural Tube Defects; Female; Anesthesia, Caudal; Adult; Nerve Block
PubMed: 38498665
DOI: 10.1097/ALN.0000000000004899 -
Journal of Surgical Case Reports Mar 2024Congenital scoliosis is one of the most common deformities of the spine in children. Intraspinal anomalies are always accompanied with congenital scoliosis....
Congenital scoliosis is one of the most common deformities of the spine in children. Intraspinal anomalies are always accompanied with congenital scoliosis. Diastematomyelia is one of the most common intraspinal pathologies in congenital scoliosis. To date, there is no standard method for correcting the congenital spinal deformity associated with diastematomyelia. We present a clinical case of simultaneous correction of congenital scoliosis by an internal corrector with excision of diastematomyelia. The patient tolerated the surgery well without any complications. She was discharged home with improved symptoms without need for additional therapy. The patient's guardians consented to the procedure and to the publication of her image.
PubMed: 38495042
DOI: 10.1093/jscr/rjae153 -
World Neurosurgery May 2024Chiari malformation is characterized by inferior displacement of the cerebellar tonsils through the foramen magnum, frequently resulting in strain related headaches, and...
BACKGROUND
Chiari malformation is characterized by inferior displacement of the cerebellar tonsils through the foramen magnum, frequently resulting in strain related headaches, and motor/sensory dysfunction. Chiari decompression technique varies significantly, possibly contributing to frequent revisions. We reviewed revision Chiari decompressions at our institution to determine the primary indications for revision and outcomes after revision.
METHODS
We retrospectively reviewed patients who underwent revision of Chiari decompression at our institution from 2005 to 2020. Demographics, indications for revision surgery, operative techniques, imaging findings, and preoperative/postoperative symptoms were collected. χ test was performed to determine statistical significance using a P < 0.05. Independent predictors of operative outcomes were identified.
RESULTS
A total of 46 patients (91% females, mean age 38.8 years) were included for analysis. The median time to revision surgery was 69.1 months (range 0-364 months) with headache (n = 37, 80%) being the most commonly recurring symptom. Large craniectomy (n = 28, 61%) was the most frequent indication for revision surgery. Thirty-two (70%) patients underwent cranioplasty, 20 (43%) required duraplasty, 15 (33%) required arachnoid dissection, and 15 (33%) required tonsillar reduction during revision surgery. Postrevision follow-up (at 8.9 ± 5.2 months average, range 1-18 months), revealed an average reduction in all Chiari-related symptoms relative to symptoms before the revision.
CONCLUSIONS
The most common indication for revision Chiari decompression was a large craniectomy resulting in cerebellar ptosis. We found that tonsillar reduction paired with modest craniectomy achieved near-complete resolution of symptoms with minimal complications. For patients with recurrent or persistent sequelae of Chiari malformation after decompression, revision may reduce symptom severity.
Topics: Humans; Arnold-Chiari Malformation; Female; Male; Reoperation; Adult; Decompression, Surgical; Retrospective Studies; Middle Aged; Treatment Outcome; Young Adult; Cohort Studies; Decompressive Craniectomy; Postoperative Complications
PubMed: 38490446
DOI: 10.1016/j.wneu.2024.03.026 -
Comptes Rendus Biologies Mar 2024Congenital heart defects (CHD) affect 1 in 100 live births and result from defects in cardiac development. Growth of the early heart tube occurs by the progressive... (Review)
Review
Congenital heart defects (CHD) affect 1 in 100 live births and result from defects in cardiac development. Growth of the early heart tube occurs by the progressive addition of second heart field (SHF) progenitor cells to the cardiac poles. The SHF gives rise to ventricular septal, right ventricular and outflow tract myocardium at the arterial pole, and atrial, including atrial septal myocardium, at the venous pole. SHF deployment creates the template for subsequent cardiac septation and has been implicated in cardiac looping and in orchestrating outflow tract development with neural crest cells. Genetic or environmental perturbation of SHF deployment thus underlies a spectrum of common forms of CHD affecting conotruncal and septal morphogenesis. Here we review the major properties of SHF cells as well as recent insights into the developmental programs that drive normal cardiac progenitor cell addition and the origins of CHD.
Topics: Humans; Heart; Heart Defects, Congenital; Myocardium; Stem Cells; Morphogenesis
PubMed: 38488639
DOI: 10.5802/crbiol.151 -
Taiwanese Journal of Obstetrics &... Mar 2024
Topics: Pregnancy; Female; Humans; Pregnancy Trimester, First; Neural Tube Defects; Pregnancy Trimester, Second; Smoking
PubMed: 38485328
DOI: 10.1016/j.tjog.2024.01.027