scholarly journals Recurrent congenital diaphragmatic hernia: A single center experience

2021 ◽  
Vol 43 (1) ◽  
Author(s):  
Żaneta Słowik-Moczydłowska ◽  
Andrzej Kamiński

Congenital Diaphragmatic Hernia (CDH) occurs in 1:4000 live births. It’s morbidity and mortality rates are significant. Recurrence is one of the recognized complications occurring after a successful initial repair. Poorly known are the rate of recurrence and factors that may influence the re-herniation. Poorly described are its morphology and treatment. Medical records of 95 neonates with CDH who had undergone repair at tertiary pediatric teaching hospital between 2007-2018 were retrospectively reviewed. Prior to surgery, peri-operative and recurrence data were collected. The follow-up was 1 to 12 years after the initial repair. Recurrence occurred in 22/84 (26%) patients. In 5 patients we observed more than one recurrence, total number of 27 (32%) re-herniations; 16/22 (72%) recurrences occurred within first 12 months; 7/27 (26%) recurrences were symptomatic which gives the symptomatic recurrence rate 8.3%; 20/27 (74%) were asymptomatic. There were no significant differences between groups considering LHR, gestational age, birth weight, severity of pre-surgery and post-surgery stabilization period. Significantly higher Apgar scores were in the group without recurrence. Among subjects with recurrent CDH majority had a left-sided defect, no hernia sac, no liver herniation, diaphragm was primarily sutured, abdominal wall primarily closed. Even though none of those factors was significantly related with the recurrence. Recurrence in CDH remains a significant issue for long-term surgical morbidity, especially more apparent in the first year of life. As long as the risk factors of re-herniation remains unclear, the most important seems to be routine follow-up protocol allowing for CDH recurrence detection.

2020 ◽  
Vol 96 (5) ◽  
pp. 569-575 ◽  
Author(s):  
Roberta Ivanira Silva do Carmo ◽  
Fernando Maia Peixoto-Filho ◽  
Arnaldo Bueno ◽  
Marlon Fonseca ◽  
Saint Clair dos Santos Gomes Junior

2020 ◽  
Vol 96 (5) ◽  
pp. 569-575
Author(s):  
Roberta Ivanira Silva do Carmo ◽  
Fernando Maia Peixoto‐Filho ◽  
Arnaldo Bueno ◽  
Marlon Fonseca ◽  
Saint Clair dos Santos Gomes Junior

2014 ◽  
Vol 49 (9) ◽  
pp. 1363-1366 ◽  
Author(s):  
Lisette Leeuwen ◽  
Karen Walker ◽  
Robert Halliday ◽  
Jonathan Karpelowsky ◽  
Dominic A. Fitzgerald

Author(s):  
Wajid Jawaid ◽  
Kit Sampat ◽  
Paul D. Losty

Abstract Introduction A developing body of literature suggests that the presence of a hernia sac in fetuses with congenital diaphragmatic hernia (CDH) may indicate improved prognosis. By examining a large cohort of CDH newborns admitted to a single United Kingdom specialist center, we aimed to establish if presence of hernia sac is a robust predictor of improved survival. Materials and Methods All CDH patients admitted to a single center were recruited. Postneonatal presentations and Morgagni hernias were excluded. Demographics, defect type, laterality, survival, and hernia recurrence were recorded. Results In this study, 192 CDH newborns were managed from 1997 to 2017; 39 were excluded (10 Morgagni and 29 postneonatal); 22 (14%) neonates had a hernia sac. Survival in patients with a hernia sac was 21/22 (95%) versus 107/124 (86%) in cases without hernia sac (p = 0.2). There was no difference in hernia sac proportion by gender (male:female 15 vs. 13.2%, p = 0.8). Conclusion In contrast to studies showing a survival advantage, albeit with smaller patient numbers, we report a statistical nonsignificant benefit of hernia sac. Better survival outcomes at this specialist center with CDH patients without a hernia sac than reported in other published studies are likely responsible for the lack of statistical significance observed, despite a larger cohort. National and international CDH registries yielding “big data” may provide further answers on the utility of a CDH hernia sac as a new prognostic scoring tool.


2019 ◽  
Vol 24 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Bianca K. den Ottelander ◽  
Robbin de Goederen ◽  
Marie-Lise C. van Veelen ◽  
Stephanie D. C. van de Beeten ◽  
Maarten H. Lequin ◽  
...  

OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.


PEDIATRICS ◽  
1986 ◽  
Vol 77 (5) ◽  
pp. 664-669
Author(s):  
Michael K. Georgieff ◽  
Judy C. Bernbaum

To document the incidence of and neonatal factors associated with abnormal shoulder girdle muscle tone in premature infants at follow-up, we studied 125 consecutively admitted infants weighing &lt; 1,750 g treated in The Children's Hospital of Philadelphia intensive care nursery and subsequently seen in the Neonatal Follow-up Program up to 18 months of age. Fifty-seven infants (46%) displayed abnormal shoulder girdle muscle tone which presented clinically as scapular retractions. These infants had significantly lower birth weights (P &lt; .001) and gestational age (P &lt; .001) as well as a higher incidence of acute and chronic pulmonary disease (P &lt; 0.01) and CNS insults (P &lt; .05) when compared with infants without scapular retractions. The 57 infants with scapular retractions were further divided into two groups: 42 infants (74%) in whom scapular retractions were associated with generalized mild hypertonicity and 15 infants (26%) in whom scapular retractions compensated for trunk and neck hypotonicity. The infants with scapular retractions and hypotonicity had a significantly higher incidence of neonatal neurologic morbidity including seizures, major resuscitations, and birth asphyxia (P &lt; .01) when compared with the infants with scapular retractions and hypertonicity. Shoulder girdle tone abnormalities in the first year of life inhibit crawling, sitting, and object manipulation and, therefore, may manifest as delays in motor development. Identification of infants with significant neonatal risk factors for tone abnormalities is important to allow for earlier therapeutic intervention.


2020 ◽  
Vol 7 (4) ◽  
pp. e757
Author(s):  
Andrea Ines Ciplea ◽  
Annette Langer-Gould ◽  
Anna Stahl ◽  
Sandra Thiel ◽  
Annette Queisser-Wahrendorf ◽  
...  

ObjectiveTo determine whether potential breast milk exposure to interferon-beta (IFN-β) or glatiramer acetate (GA) is safe for the infant.MethodsWe identified 74 infants born to 69 women with MS who breastfed under IFN-β (n = 39), GA (n = 34), or both (n = 1). Women had been enrolled into the German Multiple Sclerosis and Pregnancy Registry during pregnancy. Data were obtained from standardized, telephone-administered questionnaires completed by the mother during pregnancy and at 1, 3, 6, and 12 months postpartum and the infant's take-home medical record.ResultsThe median duration of exposed breastfeeding was 8.5 months (wide interquartile range: 4.9–12.7 months). Physical growth curves during the first year of life were consistent with national, sex-specific growth curves. Median body measurements were consistent with national medians. Most children (n = 71, 96%) had normal motor and language development. Gross motor delay was reported in 3 children, of whom 1 remained delayed at last follow-up (3.9 years old) and 2 were normal by 0.9 and 4.1 years old. The proportion of children hospitalized at least once (girls n = 2, 7%, and boys n = 6, 14%) and the proportion of children with at least one episode of systemic antibiotic use during the first year of life (girls n = 7, 23%, and boys n = 8, 18%) are consistent with national averages.ConclusionPotential breast milk exposure to IFN-β or GA did not increase the risk of common adverse infant outcomes in the first year of life. Taken together with the benefits of breastfeeding and low biological plausibility of risk, women with MS who wish to resume IFN-β or GA postpartum can be encouraged to breastfeed.


2011 ◽  
Vol 1 (1) ◽  
pp. 6
Author(s):  
Claire R. Jackson ◽  
Gordan A. MacKinlay ◽  
Merrill McHoney

Thoracoscopic repair of congenital diaphra­gmatic hernia (CDH) has been described by a number of authors and is increasingly widely practiced. We present a technical learning point. CDH is associated with the presence of a hernia sac in around 20% of all cases. In this case the presence of a ruptured hernia sac complicated the thoracoscopic repair of a left sided CDH, as it was initially difficult to recognise. Once the anatomy was clarified the repair was satisfactorily completed and the child made a rapid postoperative recovery. This technical note is to warn other surgeons that a tunnel like appearance of the diaphragmatic defect may in fact be due to the presence of a torn hernia sac, which requires excision before closure of the defect.


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