Pierre robin sequence as birth asphyxia in a new born

Journal of Pediatric Nursing, 2001

Pierre Robin sequence (PRS) is associated with long-term respiratory, nutritional, and developmental difficulties. Hypoxic complications, including cerebral impairment, cor pulmonale, and failure to thrive may be prevented or minimized with early detection and comprehensive care. This article reviews the embryological development of PRS and outlines the medical and nursing implications for treatment. Partnership with parents and a coordinated community care team are essential for successful management of infants and children with PRS.

Fetal Diagnosis and Therapy, 2015

Fetal ultrasonography is an important tool used to prenatally diagnose many craniofacial conditions. Pierre Robin sequence (PRS) is a rare congenital deformation characterized by micrognathia, glossoptosis, and airway obstruction. PRS can present as a perinatal emergency when the retropositioned tongue obstructs the airway leading to respiratory compromise. More predictable and reliable diagnostic studies could help the treating medical team as well as families prepare for these early airway emergencies. The medical literature was reviewed for different techniques used to prenatally diagnose PRS radiologically. We have reviewed these techniques and suggested a possible diagnostic pathway to consistently identify patients with PRS prenatally.

Prenatal Diagnosis, 2015

Objective To assess the outcome of fetuses who had sonographic features suggestive of Pierre Robin Sequence (PRS). Method All prenatal ultrasounds that mentioned 'posterior cleft palate', or 'micro or retrognathia' or 'PRS' over 13 and 20 years, respectively, at two obstetrical centers were reviewed. Medical records for children with isolated PRS monitored over 20 years at a PRS referral center for prenatal anomalies and the severity of neonatal feeding and respiratory functional disorders were utilized for comparison. Results From a prenatal ultrasound database of 166 000 cases, 157 had one or more of the sonographic signs suggestive of PRS and had follow-up available. Of them, 33 (21%) had confirmed PRS, 9 (6%) were normal and 115 (73%) had chromosomal aberrations, associated malformations or neurological anomalies. Visualization of a posterior cleft palate in addition to retro-micrognathia had a positive predictive value of 100% for PRS. The distribution of functional severity grades was similar in cases suspected prenatally as in 238 cases of PRS followed in the referral center in Necker Hospital. Conclusion Only a minority of cases of fetal retrognathia have complete PRS; the majority have other severe conditions. Prenatal prediction of functional severity of isolated PRS is not possible.

Acta Scientific MEDICAL SCIENCES, 2023

The early hour life of newborn is an ambiguous and challenging period in human life which need careful and intelligent monitoring to obtain better life outcomes. Pierre Robin Sequence one of the rare syndrome that is observed shortly after or before the birth. PRS is a group of malformation in the baby that develop during pregnancy. It is called a casade because one of its definite features − an undeveloped lower jaw − initiate a casade of other deformities while the baby is developing in uterus and can ultimately lead to postnatal upper airway obstruction and life threatening condition that lead to eventual death. Aim: The purpose of the study was to highlight the significance of three-dimensional ultrasound in the screening of fetal micrognathia and highlight the diagnostic features of infant with Pierre Robin syndrome; to establish an early assessment approach for better outcomes of infant life. Methodology: A systematic literature review depends on collecting data from an evidence based studies. Searches were made of twenty five electronic databases: the Cochrane Oral Health Group's Trials Register, The Cochrane Central Register of Controlled Trials (CENTRAL), EMBASE, PsycINFO, Scopus and Web of science, MEDLINE (PubMed). Results: There is a strong association between breathing difficulty, feeding difficulty in infant and the small size of mandible which affect on fetus shelf life. Conclusion: PRS is a rare anomaly so raise the awareness to this syndrome should be increased between different physicians. Prenatal diagnosis is very important step for appropriate treatment decision by parents before delivery and to arrange with a multidisplinary team to assessment the condition postnatal to reduce the short and long term complications and treatments.

JAMA pediatrics, 2016

Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis, and upper airway obstruction. Currently, no consensus exists regarding the diagnosis and evaluation of children with RS. An international, multidisciplinary consensus group was formed to begin to overcome this limitation. To report a consensus-derived set of best practices for the diagnosis and evaluation of infants with RS as a starting point for defining standards and management. Based on a literature review and expert opinion, a clinical consensus report was generated. Because RS can occur as an isolated condition or as part of a syndrome or multiple-anomaly disorder, the diagnostic process for each newborn may differ. Micrognathia is hypothesized as the initiating event, but the diagnosis of micrognathia is subjective. Glossoptosis and upper airway compromise complete the primary characteristics of RS. It can be difficult to judge the severity of tongue base airway obstruction, and the pos...

Journal of Stomatology, Oral and Maxillofacial Surgery, 2018

Pierre Robin syndrome (PRS) is characterized of a triad of clinical signs: micrognathia, glossoptosis and obstruction of the upper airways frequently associated with palatal cleft. It is a heterogenic pathological entity and it can be found as isolated disease (nsPRS) or in association with other syndromes (sPRS), with more pronounced symptoms and systemic involvement. This review aims to summarize the principal features of PRS, analysing the different aspects of the disease. Epidemiological data highlight incidence, severity and mortality of PRS; pathophysiological mechanism reports the etiology and pathogenesis of the disease distinguishing between isolated and syndromic form. Because of the clinical importance of PRS, it's fundamental to describe the features of the Robin sequence to clearly define its primary and secondary clinical signs useful to diagnosis. A complete evaluation of the syndrome allows to choose the most appropriate therapeutic treatment, opting for conservative or surgical management, in order to improve the quality of life of the patient.

2017

Pierre Robin sequence (PRS) is classically described as a triad of micrognathia, glossoptosis, and airway obstruction. Infants frequently present at birth with a hypoplastic mandible and difficulty breathing. The smaller mandible displaces the tongue posteriorly, resulting in obstruction of the airway. Typically, a wide U-shaped cleft palate is also associated with this phenomenon. PRS is not a syndrome in itself, but rather a sequence of disorders, with one abnormality resulting in the next. However, it is related to several other craniofacial anomalies and may appear in conjunction with a syndromic diagnosis, such as velocardiofacial and Stickler syndromes. Infants with PRS should be evaluated by a multidisciplinary team to assess the anatomic findings, delineate the source of airway obstruction, and address airway and feeding issues. Positioning will resolve the airway obstruction in ~70% of cases. In the correct position, most children will also be able to feed normally. If the ...

Plastic surgery (Oakville, Ont.), 2017

Pierre Robin sequence (PRS) is a triad of micrognathia, glossoptosis, and respiratory distress. There is no standard clinical classification used in the management of neonatal airway in patients with PRS. The goal of our study was to review the presentation and management of patients with PRS and formulate a clinical grading system and treatment algorithm. A 10-year retrospective review of all neonates diagnosed with PRS was performed after obtaining institutional ethics approval. Patients were identified using our cleft lip and palate program database. Inclusion criteria were 2 of the following 3 clinical features-glossoptosis, retrognathia, or airway obstruction. We collected demographic data, clinical information (coexisting airway morbidity, maxillary-mandibular discrepancy, type of intervention used, complications, and outcomes (feeding, length of stay, and airway status) during the first year of life. Sixty-three patients met our inclusion criteria. Of these, 55 (87%) had clef...

International journal of pediatric otorhinolaryngology, 1990

Robin Sequence is a well-described disorder with a reported incidence of 1 in 2000 births. It is characterized by glossoptosis and micrognathia often associated with a cleft palate. Respiratory obstruction at the level of the tongue occurs in many affected children. Prior to routine intensive care unit management, it was the primary factor in the mortality reported in patients with moderately severe to severe deformity. Treatment of this obstruction is controversial and may include a long stay in an intensive care setting. We report two cases that demonstrate properly placed and constructed nasopharyngeal tubes allow a non-surgical treatment with early discharge of the patient and without the risks and morbidity of surgery or the complications of tracheostomy.

European Journal of Pediatrics, 2002

Pierre Robin sequence (posterior U-shape cleft palate, glossoptosis, retrognathia) (PRS) is a frequent and heterogeneous neonatal condition of obscure origin. We show here that orodigestive and cardiorespiratory functional disorders are very frequent in PRS and that these functional disorders, as well as anatomical and embryological data, argue for the involvement of brainstem dysfunction in the pathogenesis of some cases of isolated PRS. A total of 66 infants consecutively admitted for isolated PRS were followed-up with observations and investigations focused on their orodigestive and cardiorespiratory disorders. Neonatal clinical examination and neonatal anatomical aspects of the three orofacial features of the sequence were evaluated. Feeding difficulties and respiratory disorders were recorded and infants were classified according to three grades of severity. The relation between functional severity grade and neonatal orofacial features was evaluated, as well as the relation between functional severity grade and specific criteria characterising oesophageal and laryngeal motility and cardiac orthoparasympathetic imbalance. In the first weeks of life, sucking and swallowing disorders (100%), excessive regurgitation (94%), upper airways obstruction (50%), and cardiac vagal overactivity (59%) were noted. Correlation of anatomical features with functional severity grades was poor except for extreme forms of glossoptosis and retrognathia. Specific anomalies of oesophageal motility, pharyngolaryngeal tone and parasympathetic cardiac regulation were described. These anomalies were more frequent in children with the two higher grades of functional severity. Conclusion: infants with Pierre Robin sequence have early and severe anomalies of orodigestive and cardiorespiratory function which do not appear to be related solely to anatomical features and which require proper medical management. We suggest a prenatal and neonatal brainstem dysfunction as a neuroembryological hypothesis to explain the onset of some cases of Pierre Robin sequence.