Laryngotracheoesophageal Cleft Type IV in a Preterm Neonate. A Case Report and Literature Review.

We present a case of a preterm neonate with a type IV laryngo-tracheo-oesophageal cleft, an uncommon congenital malformation, resulting from the failure of separation of the trachea and the oesophagus during fetal development, often associated with other deformities as well. Data in the literature shows that the long-term morbidity from the entity has declined over the last decades, even though prognosis remains unfavourable for types III and IV. This report emphasizes the complex issues neonatologists are faced with, when treating neonates with this rare disorder in the first days of life, what will raise suspicion of this rare medical entity, and that direct laryngoscopy/bronchoscopy finally depicts the exact extension of the medical condition. At the same time extensive evaluation for coexisting congenital anomalies should be performed. For all the above reasons, these neonates should be treated in specialized tertiary pediatric centers for multidisciplinary prompt management, which may improve, the outcome.

Respiratory problems in full term newborns, which parameters are related to the length of in-patient stay?

OBJECTIVE: To analyze respiratory problems of full term newborn babies during their first hours of life, compare parameters related to the length of neonatal intensive care unit (NICU) in-patient stay, and discuss perinatal care practices, which could prevent consequences, and/or reduce associated costs of NICU hospitalization. MATERIAL AND METHODS: Retrospective chart review between July 31st 2017 and March 2nd 2018 in a tertiary maternity hospital. Chi-square testing analyzed qualitative data. Mann-Whitney test was used for quantitative variables. Multiple linear regression models determined odds ratios for associations were found to be significant in univariate analyses. RESULTS: 86 babies with respiratory problems were studied. Delivery mode was vaginal in 11.6% and caesarean section in 88.4% of newborns. About 52.3% of admitted newborns had transient tachypnea and improved relatively quickly. Newborns resuscitated in (P = 0.004) or admitted to the NICU from the delivery room (P = 0.000) displayed greater need for O2 administration. Twenty neonates were intubated; 16 of them were administered surfactant. Infection (P < 0.05), abnormal chest x-ray (P = 0.022), and the severity of respiratory system morbidity (P < 0.05) prolonged neonatal in-patient stay. Respiratory problems (P = 0.003) and intubation (P = 0.032) incurred greater hospitalization costs. Breastfeeding initiation before the 3rd day of life (P = 0.031), and O2 administration for more than 72 h (P = 0.036) were significantly associated with the length of in-patient stay in the multivariate regression analysis. CONCLUSION: Mandating the presence of a pediatrician in the delivery room would optimize justified direct NICU admissions and reduce the possibility of delayed diagnosis of respiratory distress during rooming-in. NICU in-patient stay in full term neonates should be based on combining diagnostic markers of sepsis, such as procalcitonin or interleukin-6. A specific protocol regarding surfactant administration in intubated full term infants is also necessary. The severity of respiratory system morbidity is not the key determinant of infant feeding. Maternal information regarding infant health and breastfeeding abilities may improve breastfeeding rates.

Congenital Diaphragmatic Eventration in the Neonatal Period: Systematic Review of the Literature and Report of a Rare Case Presenting with Gastrointestinal Disorders.

BACKGROUND: The term congenital diaphragmatic eventration (CDE) refers to an anatomical abnormality of the diaphragm. It is a very rare condition; however, early and prompt diagnosis is of very great importance due to possible life-threatening complications. Most severely affected patients are neonates, usually presented with respiratory distress symptoms. The aim of this study was to systematically review the existing literature and to consolidate data on CDE in neonates as well as to report a case of a neonate with congenital diaphragmatic eventration of the left hemidiaphragm and clinical signs and symptoms of the gastrointestinal tract. METHODS: An electronic search of the PubMed and Scopus databases was performed regarding studies evaluating the clinical presentation, diagnosis methods, treatments, and outcomes of CDE in the neonatal population. RESULTS: Data from 93 studies were integrated into our review, reporting 204 CDE cases, and according to them, the male/female ratio was 1/1 with a predominance of right-sided eventration. The diagnosis was primarily established by chest X-ray; surgical intervention was the most frequent treatment. The recurrence rate was 8.3% (9/109 cases). CONCLUSIONS: Early and accurate diagnosis of CDE and repair of the diaphragm can prevent complications, reduce morbidity, and improve the quality of patient's life.

Monkeypox disease and pregnancy. Where are we today? A review of literature.

Monkeypox has emerged as a significant human pathogen, posing severe risks in vulnerable populations. At present, there is not enough data available as to whether pregnant women are more vulnerable to monkeypox infection, or they suffer more severe symptoms, and studies on this issue as well as to the possible adverse effects on the developing fetus are limited. The aim of this review, was to bring together what is known so far about monkeypox virus transmission, the clinical course of the disease, and associated maternal-fetal outcomes. Furthermore, to summarize the current available recommendations on the prevention and management of monkeypox infection during pregnancy, in order to help obstetricians and neonatologists navigate through this new challenging area and provide the best available care to their patients.

Subcapsular Liver Hematoma-A Life-Threatening Condition in Preterm Neonates-A Case Series and Systematic Review of the Literature.

The subcapsular hematoma (SLH) of the liver is a rare finding in living infants. The clinical presentation of rupture is non-specific, with the signs of hypovolemic shock dominating. The causes are several, with prematurity, trauma and sepsis playing a leading role in the creation of an SHL. Umbilical vein catheterization and an increased bleeding tendency have also been associated with this usually fatal diagnosis. Abdominal ultrasonography, among other imaging methods, comprises the gold standard examination for early diagnosis. It should be differentiated from other possible causes of shock, such as sepsis and intraventricular hemorrhage, which have similar clinical presentation. We report a case series of three very low birth weight preterms (VLBW), with an SHL, during the first days of life, one of which survived from this usually catastrophic condition. A comprehensive review of the literature regarding this clinical entity was also conducted. A high index of suspicion is essential for early identification of such a case, with conservative or surgical treatment being the way to go.

Breastfeeding in Neonates Admitted to an NICU: 18-Month Follow-Up.

Introduction: The admission of neonates to Neonatal Intensive Care Units (NICUs) has been identified as a primary inhibiting factor in the establishment of breastfeeding. The aims of this study were to (1) estimate the prevalence and duration of breastfeeding in infants/toddlers who had been admitted to an NICU in Greece and (2) to investigate factors, associated with the NICU stay, which affected the establishment and maintenance of breastfeeding in infants/toddlers previously admitted to the NICU. Materials and methods: Data for this cohort study were retrieved from interviews with mothers of infants/toddlers who had been admitted to our NICU as neonates during the period of 2017−2019. Interviews were conducted based on a questionnaire regarding the child's nutrition from birth to the day of the interview, including previous maternal experience with breastfeeding. Information related to the prenatal period, gestation age, delivery mode, duration of NICU stay, and neonatal feeding strategies during their hospital stay were recorded. Results: The response rate to the telephone interviews was 57%, resulting in 279 mother−infant pairs being included in this study. The results showed that 78.1% of children received maternal milk during their first days of life. Of all infants, 58.1% were exclusively breastfed during their first month, with a gradual decrease to 36.9% and 19.4% by the end of the third and sixth months of life, respectively. The prevalence of breastfed children reached 14.7% and 7.5% at the ages of twelve and eighteen months, respectively. In the multivariate analysis, prematurity emerged as an independent prognostic factor for the duration of exclusive and any breastfeeding (aHR 1.64, 95% CI: 1.03−2.62; and 1.69, 95% CI: 1.05−2.72, respectively; p < 0.05). Additionally, the nationality of the mother, NICU breastfeeding experience, the administration of maternal milk during neonatal hospital stay, and previous breastfeeding experience of the mother were independent prognostic factors for the duration of breastfeeding. Conclusions: Although breastfeeding is a top priority in our NICU, the exclusive-breastfeeding rates at 6 months were quite low for the hospitalized neonates, not reaching World Health Organization (WHO) recommendations. Mothers/families of hospitalized neonates should receive integrated psychological and practical breastfeeding support and guidance.

The effect of body and mind stress-releasing techniques on the breastfeeding of full-term babies; a critical analysis of published interventional studies.

OBJECTIVE: To critically assess the effectiveness of complementary/alternative medicine modalities, comprising body/mind stress-releasing techniques, on the breastfeeding (BF) success of mothers of full-term newborns. METHODS: Literature review in Medline and other available database sources until December 2015, and critical analysis of pooled data. The study selection included randomized-controlled trials, case-control studies, and case series. RESULTS: The total number of analyzed studies was 9. Among them, three represented level-I, two level-II, one level-III, and three level-IV evidence. The pooled maternal population was 2135. Interventions that could influence cognition management seem effective in improving BF initiation and duration (strength of recommendation B), manual relaxation techniques in promoting BF initiation (strength of recommendation B), and auditory-mediated mind guidance in promoting BF duration (strength of recommendation C). The implementation of environmental sensory stimulations improves maternal perception about breastfeeding practice (strength of recommendation C). CONCLUSIONS: Certain methods of complementary and alternative medicine show positive effect on breastfeeding. Further studies with good quality evidence, dedicated follow-up of the families after discharge, and strict definitions of breastfeeding and weaning are necessary to confirm the effectiveness of the identified interventions.

Factors affecting breastfeeding duration in Greece: What is important?

AIM: To investigate factors associated with breastfeeding duration (BD) in a sample of mothers living in Greece. METHODS: Four hundred and twenty-eight mothers (438 infants) were initially recruited in a tertiary University Hospital. Monthly telephone interviews (1665 in total) using a structured questionnaire (one for each infant) were conducted until the sixth postpartum month. Cox regression analysis was used to assess factors influencing any BD. RESULTS: Any breastfeeding rates in the first, third, and sixth month of the infant's life reached 87.5%, 57.0% and 38.75%, respectively. In the multivariate analysis, maternal smoking in the lactation period [hazard-ratio (HR) = 4.20] and psychological status (HR = 1.72), and the introduction of a pacifier (HR = 2.08), were inversely associated, while higher maternal education (HRuniversity/college vs primary/high school = 0.53, HRmaster's vs primary/high school = 0.20), and being an immigrant (HR = 0.35) were positively associated with BD. CONCLUSION: Public health interventions should focus on campaigns against smoking during lactation, target women of lower educational status, and endorse the delayed introduction of pacifiers.

Immigrant status as important determinant of breastfeeding practice in southern Europe.

AIM: Breastfeeding is universally accepted as the optimal way to nourish infants. There is evidence that socio-demographic factors, including immigrant status, are related to infant feeding practices. The aim of the present study was to identify the factors which are associated with breastfeeding initiation and duration, with special focus on the role of immigrant status of the mother in breastfeeding practice. A sample of mothers giving birth and living in Athens, Greece, was investigated. METHODS: 428 mothers (438 infants) were recruited in the maternity ward of a Tertiary University Hospital, and were interviewed using a structured questionnaire. Monthly telephone interviews were subsequently conducted until the sixth postpartum month. Multivariate logistic regression models were used to quantify the association of socio-demographic parameters with breastfeeding initiation. Cox regression analysis was employed to assess related factors that might influence breastfeeding duration. RESULTS: Being an immigrant was positively associated with exclusive as well as partial breastfeeding initiation (OR 7.97, 95% CI 1.02-62.19). Immigrant mothers were also 0.35 times less likely (95% Ci 0.21-0.58) to stop breastfeeding earlier, compared to the native ones. Several other factors were deemed important either for breastfeeding initiation or its duration but not for both aspects of breastfeeding practice. CONCLUSION: Maternal immigrant status was found to be consistently associated with breastfeeding initiation and duration in this study sample. Health professionals, health policy makers and politicians should remain attuned to the cultural backgrounds which have created strong breastfeeding traditions, to further promote breastfeeding practice in Western countries.

Factors affecting breast-feeding initiation in Greece: What is important?

OBJECTIVE: to investigate the association between breast-feeding initiation and socio-demographic, lifestyle-related, clinical and lactation-related factors in a sample of mothers living in Greece. DESIGN: cross-sectional study. SETTING: Tertiary University Hospital, maternity ward. PARTICIPANTS: 428 mothers were interviewed (43.2% response rate) from February until December 2009, using a structured face-to-face questionnaire after at least 24 hours from childbirth. MEASUREMENTS AND FINDINGS: 71.0% of mothers were Greeks and 29.0% immigrants; the mean age was 32.0 years. 44.4% initiated exclusive breast feeding, 7.9% artificial milk -feeding and 47.7% partial feeding. In the multivariate analysis, exclusive breast feeding was inversely related to maternal body mass index (BMI) at the beginning of gestation (odds ratio (OR)=0.93, 95% confidence intervals (95%CI)=0.89-0.98) and caesarean section (OR=0.54, 95% CI=0.35-0.84). Lactation-related factors which favourably affected exclusive breast-feeding initiation included previous breast-feeding experience (OR=2.29, 95% CI=1.39-3.78), information about breast feeding (OR=2.38, 95% CI=1.41-4.01) and rooming-in (OR=1.62 95% CI=1.03-2.54), whilst any breast feeding was favourably affected by encouraging women to breast feed (OR=5.42, 95% CI=1.90-15.50), providing information about breast feeding (OR=6.92, 95% CI=2.53-18.89), and rooming-in (OR=6.93 95% CI=2.01-23.88), and negatively associated with caesarean section (OR=0.11, 95% CI=0.03-0.39). Being an immigrant mother was also positively associated with any breast-feeding initiation (OR=7.97, 95% CI=1.02-62.19). Maternal age, education and income, as well as, smoking status, were not associated with any breast-feeding initiation. KEY CONCLUSIONS: maternal BMI and immigrant status, information provided by midwives and encouragement, rooming-in and mode of childbirth (caesarean section), were found to be important for breast-feeding initiation in this study population. No other indicator of socio-demographic status was found to be associated with breast-feeding initiation. IMPLICATIONS FOR PRACTICE: focus should be given to pregnant women with higher BMI at the beginning of pregnancy, and women who had undergone caesarean section. Breast-feeding information and encouragement should be provided to all women in the maternity ward, along with the dedicated practice of rooming-in, in order to promote and increase breast-feeding initiation rates.

Cochlear implantation for single-sided deafness: the outcomes. An evidence-based approach.

The aim of the present paper is to critically review the current evidence on the efficacy of cochlear implantation as a treatment modality for single-sided deafness (SSD), and/or unilateral tinnitus. Systematic literature review in Medline and other database sources was conducted along with critical analysis of pooled data. The study selection includes prospective and retrospective comparative studies, case series and case reports. The total number of analyzed studies was 17. A total of 108 patients with SSD have been implanted; 66 patients due to problems associated with SSD, and 42 primarily because of debilitating tinnitus. Cochlear implantation in SSD leads to improved sound localization performance and speech perception in noise from the ipsilateral side with an angle of coverage up to (but not including) 90(°) to the front, when noise is present in the contralateral quartile (Strength of recommendation B). Speech and spatial hearing also subjectively improve following the insertion of a cochlear implant (Strength of recommendation B); this was not the case regarding the quality of hearing. Tinnitus improvement was also reported following implant placement (Strength of recommendation B); however, patients need to be advised that the suppression is mainly successful when the implant is activated. The overall quality of the available evidence supports a wider use of cochlear implantation in SSD following appropriate selection and counseling (overall strength of recommendation B). It remains to be seen if the long-term follow-up of large number of patients in well conducted high quality studies will confirm the above mentioned results.

Diagnostic challenges and safety considerations in cochlear implantation under the age of 12 months.

AIM: To review the current knowledge on cochlear implantation in infancy, regarding diagnostic, surgical and anesthetic challenges. STUDY-DESIGN: Meta-analysis. EBM level: II. MATERIALS/METHODS: Literature-review from Medline and database sources. Related books were also included. STUDY SELECTION: Meta-analyses, prospective controlled studies, prospective/retrospective cohort studies, guidelines, review articles. DATA SYNTHESIS: The diagnosis of profound hearing loss in infancy, although challenging, can be confirmed with acceptable certainty when objective measures (ABR, ASSR, OAEs) and behavioural assessments are combined in experienced centres. Reliable assessment of the prelexical domains of infant development is also important and feasible using appropriate evaluation techniques. Overall, 125 implanted infants were identified in the present meta-analysis; no major anesthetic complication was reported. The rate of surgical complications was found to be 8.8% (3.2% major complications) quite similar to the respective percentages in older implanted children (major complications ranging from 2.3% to 4.1%). CONCLUSION: Assessment of hearing in infancy is feasible with adequate reliability. If parental expectations are realistic and hearing aid trial unsuccessful, cochlear implantation can be performed in otherwise healthy infants, provided that the attending pediatric anesthesiologist is considerably experienced and appropriate facilities of pediatric perioperative care are readily available. A number of concerns, with regard to anatomic constraints, existing co-morbidities or additional disorders, tuning difficulties, and special phases of the developing child should be also taken into account. The present meta-analysis did not find an increased rate of anesthetic or surgical complications in infant implantees, although long-term follow-up and large numbers are lacking.

Efficacy assessment and complications of surgical management for superior semicircular canal dehiscence: a meta-analysis of published interventional studies.

Superior semicircular canal syndrome (SSCS) includes vestibular and audiological symptoms which result from the introduction of a third mobile window into the osseous cochlea. Surgical repair is considered in cases of incapacitating symptoms. The present paper aims at comparing the different surgical approaches and modes of dehiscence repair, regarding their respective efficacy and potential pitfalls. A systematic literature review and meta-analysis of pooled data were performed. Study selection included prospective- and retrospective-controlled studies, prospective- and retrospective-cohort studies, ex vivo studies, animal models, case-reports, systematic reviews and clinical guidelines. A total of 64 primary operations for SSC repair were identified; 56 ears were operated for vestibular and 7 for auditory complaints. A total of 33 ears underwent canal plugging, 16 resurfacing, and 15 capping. Success rates were 32/33, 8/16, and 14/15, respectively. The observed differences were statistically significant (P=0.001). Resurfacing proved less effective than both plugging (P=0.002), and capping (P=0.01) techniques. Temporalis fascia was commonly used as sealing material and was combined with bone-pâté/bone-wax (plugging), bone-graft (resurfacing), or hydroxyapatite-cement (capping). Most operations were performed via middle-fossa approach; higher success rates were associated with plugging and capping techniques. SNHL and disequilibrium were the most frequent complications encountered. Most cases were followed for 3-6 months. Precise criteria regarding follow-up duration and objective success measures are not determined. Surgical repair of SSCS is considered as a valid therapeutic option for patients with debilitating symptoms. Consensus regarding strict follow-up criteria and objective assessment of success is necessary before larger scale operations can be implemented in clinical practice.

Novel approaches to treating sensorineural hearing loss. Auditory genetics and necessary factors for stem cell transplant.

Sensorineural hearing loss is a chronic disease, with a serious impact on human communication and quality of life. Exposure to various factors can lead to irreversible hearing impairment, as the auditory epithelium in humans comprises terminally differentiated cells. By contrast, the inner ear of lower vertebrates and invertebrates shows regenerative capacity. Efforts to regenerate the damaged human inner ear may involve renewed cell proliferation, or transplanting cells that can differentiate into sensory cells. Literature review. Animal studies, in vitro studies, retrospective-cohort studies, community-based case-controls, clinical guidelines, and review articles. Embryonic stem cells, inner ear stem cells, and stem cells from other tissues (i.e., neural tissue, hematopoietic system) may be candidates for restoring the auditory epithelium. Transcriptional regulation of p27kip1 is the primary determinant of terminal mitosis and the final number of postmitotic progenitors of hair and supporting cells. Basic helix-loop-helix transcription factor Math1 was found to be necessary and sufficient for the production of auditory hair cells. Notch signaling seems to play a major role in the regulation of Math1, through lateral inhibition. Brn3c, Gfi1, and Barhl1 are also specific transcription factors that have been implicated in hair cell maintenance and consequent survival. Evidence concerning development, maintenance, and regeneration of hair cells is still at an embryonic stage. Combined data, as attempted in the present study, will lead to a more successful management of deafness.

Sensory cell regeneration and stem cells: what we have already achieved in the management of deafness.

BACKGROUND/OBJECTIVE: Genetic manipulation of the cell-cycle exit, induction of new hair cells (HCs) through gene modification therapy, and introduction of stem cells (SCs) into damaged cochleas potentially offer exciting new strategies in treating sensorineural hearing loss. MATERIALS AND METHODS: Literature review from Medline and database sources. STUDY SELECTION: Ex vivo models, animal studies, in vitro studies, and review articles. DATA SYNTHESIS: Embryonic SCs, neural SCs, or bone marrow SCs survive in the mammalian inner ear after transplantation. The scala media and the modiolus seem more functionally appropriate injection sites. The clear evidence that transplanted neural SCs can adopt the morphologic phenotypes of HCs was the most significant milestone achieved in the related research. The normal cytoarchitecture in the organ of Corti may also be restored through mouse atonal homologue 1 transgene expression and transduction of the nonsensory cells, producing clinically measured improvement in hearing thresholds. Embryonic SC-derived neurons have the potential for synapse formation with auditory HCs and reinnervation of the auditory epithelia. However, fluctuations in survival rates, functional recovery of the spiral-ganglion neurons, integration to the host tissue, and potential immune barriers are also areas of utmost importance. CONCLUSION: There is an already exciting progress in the fields of sensory cell regeneration and SC research in an attempt to restore hearing or prevent deafness. However, further understanding of the underlying mechanisms of auditory genetics, continuing investigation of the human genome, refinement of the delivering techniques, and specification of the therapeutic strategies have to be developed before functional regeneration of the cochlea can be achieved in clinical practice.

Auditory neuropathy: endocochlear lesion or temporal processing impairment? Implications for diagnosis and management.

BACKGROUND/OBJECTIVE: Auditory neuropathy/dys-synchrony, characterized by absent auditory brainstem responses, normal otoacoustic emissions or cochlear microphonics, and word discrimination disproportional to the pure-tone audiogram, may be accompanied by perceptual consequences that could jeopardize language acquisition in affected children. However, the related evidence is constantly changing leading to a serious debate. The aim of the present paper is to review the current knowledge on auditory neuropathy/dys-synchrony, and to present the therapeutic strategies that can be employed in its management, taking into account the potentially underlying pathophysiology. MATERIALS/METHODS: Literature review from Medline and database sources. Related books were also included. STUDY SELECTION: Controlled clinical trials, prospective and retrospective cohort studies, nested-based case-control and analytical family studies, laboratory and electrophysiological studies, animal models, case-reports, joint statements and review articles. DATA SYNTHESIS: Auditory neuropathy/dys-synchrony, in contrast to what is widely believed, is a very frequent disease, responsible for approximately 8% of newly diagnosed cases of hearing loss in children per year. Hyperbilirubinemia and hypoxia represent major risk factors, whereas generalized neuropathic disorders, or a genetic substrate involving the otoferlin gene, are responsible for the phenotype of auditory neuropathy/dys-synchrony in certain cases. Auditory nerve myelinopathy and/or desynchrony of neural discharges are the most probable underlying pathophysiologic mechanisms. Genetic testing may be helpful in cases of non-syndromic prelingual children. Auditory neuropathy/dys-synchrony management aims at restoring the compromised processing of auditory information, either through conventional amplification and/or alternative forms of communication, or by cochlear implantation (combined with intensive speech and language therapy). CONCLUSION: Auditory neuropathy/dys-synchrony is more frequent than considered in the past, especially amongst hearing-impaired children. Accurate diagnosis, based on subjective and objective hearing assessment techniques (including the various electrophysiological assessment measures), and timely treatment of the affected children is of paramount importance, with hearing aids, intensive speech and language therapy (and sign language when indicated) providing the mainstay of habilitation, and cochlear implantation representing a valid therapeutic alternative.

Grommets in otitis media with effusion: the most frequent operation in children. But is it associated with significant complications?

INTRODUCTION: Otitis media with effusion is one of the most frequent diseases in children, and its management requires the attention of general practitioners, pediatricians and ear, nose and throat (ENT) surgeons. The main complications associated with tympanostomy tube insertion, are: (1) purulent otorrhea (10-26% of cases), in which local otic preparations might be effective, and biofilm-resistant tubes may decrease this complication in the future; (2) myringosclerosis (39-65% of operated ears), with usually no serious sequelae; (3) segmental atrophy (16-75% of cases); (4) atrophic scars and pars flaccida retraction pockets (28 and 21% of operated ears, respectively); (5) tympanic membrane perforations (3% of cases, although with T-tubes, the incidence may be as high as 24%); (6) cholesteatoma (1% of cases), although tympanostomy tubes may sometimes prevent, rather than contribute to its development; (7) granulation tissue (5-40% of instances), when the duration of tube retention is prolonged. CONCLUSION: It would appear that the complications associated with tympanostomy tube insertion are more frequent than anticipated, reaching 80% of operated ears under specific circumstances and in certain subgroups of children. These complications may resolve with conservative management, but in persistent cases surgical removal of the tubes is mandatory.