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At least 19 recordsLinked to original sources
PubMedDate not supplied

[Hyperphalangia in Pierre-Robin syndrome].

Pierre Robins syndrome associated with symmetric hyperphalangia of the index fingers is called Catel-Manzke syndrome. Connate radial deviation of the index fingers is caused by an odd bone. The deformity is demonstrated by two personal cases. The deviation is progressive and because of increasing functional limitation the operative correction of the index fingers at the age of 2-3 years is advised. Surgical technique and success of treatment are presented.

Child, Preschool↗
PubMed1985

[Management of Pierre-Robin syndrome].

Pierre Robin syndrome continuously raises problems of intensive care that are difficult to surmount. A peak has to be overcome, the date of which varies as a function of the severity of the affection, following which the infant remains a neonate at high risk. Intensive care of these infants during the neonatal period involves avoidance of the sequence of disturbances provoked by the disorders of ventilation and deglutition. Surveillance of biological variables, feeding and the fight against glossoptosis are mainly the concern of medical procedures: from orthostatic nipples to continuous gastric feeding, from procubitus to intubation with assisted ventilation, and including pharyngeal catheters and physiotherapy. Surgery is required only exceptionally at this stage, mainly in the form of hyomandibulopexy.

Abnormalities, Multiple↗
PubMed2006

Chiari I malformation, caudal regression syndrome, and Pierre Robin Syndrome: a previously unreported combination.

CASE: The authors report a child born with a constellation of findings including Chiari I malformation, Pierre Robin syndrome, and caudal regression syndrome. In addition, this child was found to have a fatty-infiltrated filum terminale and a low conus medullaris. DISCUSSION AND CONCLUSION: In isolation, the Chiari I malformation has been reported in patients with caudal regression and Pierre Robin syndrome. Speculation has been given to the possibility of a common insult to early mesodermal development and each of these pathological entities. To our knowledge, this is the first report of a single patient that possessed all three entities (i.e., Chiari I malformation, Pierre Robin syndrome, and caudal regression syndrome). Further case reports as this may shed light on the dysembryology that results in each of these diagnoses.

Arnold-Chiari Malformation↗
PubMed1985

[An original intubation technic in a child with Pierre Robin syndrome].

Infants with Pierre Robin syndrome often present the anaesthesiologist with the challenge of upper airway obstruction and difficult tracheal intubation. An unconventional method for solving this problem was used in a 2 week old 2.2 kg term male infant who presented with severe micrognathia, a widely cleft palate and extreme glossoptosis. Hyperextension of the head in the prone position distracted the epiglottis from the glottis. Blind nasotracheal intubation was then used. The curve of the nasotracheal tube made it pass behind the epiglottis into the larynx.

Anesthesia, General↗
PubMedDate not supplied

Fiber-optic bronchoscopic guidance for intubating a neonate with Pierre-Robin syndrome.

The Pierre-Robin anomalad features micrognathia, glossoptosis, and frequently a cleft palate. Tracheal intubation may be challenging and sometimes impossible. Reported is a case in which a tracheal guide wire was passed via the suction port of a flexible pediatric bronchoscope. The bronchoscope was then removed, and an endotracheal tube was threaded over the wire. The technique is safe and allows rapid endotracheal intubation in pediatric patients with difficult upper airways.

Airway Obstruction↗
PubMed1990

Management of upper airway obstruction in the Pierre Robin syndrome.

Eight patients with Pierre Robin syndrome (PRS), were treated according to our management protocol. All our patients initially were given a trial of conservative, positional treatment, with high caloric gavage feeding. In five patients this therapeutic approach resulted in a good weight gain, with no significant respiratory distress. The remaining three patients showed no improvement, failed to thrive and therefore underwent the tongue to lip adhesion (TLA) procedure. Two patients then improved dramatically; whereas one continued with respiratory distress and failure to thrive and required tracheostomy. We conclude that when symptoms of respiratory distress and failure to thrive coexist in patients with PRS despite conservative management, surgical intervention to the airway is mandatory. TLA should be the first surgical procedure considered and if the ultimate goals of weight gain and respiratory comfort are still not achieved then tracheostomy seems inevitable.

Airway Obstruction↗
PubMed1990

[Labioglossopexy in Pierre Robin syndrome. Our experiences apropos of 70 cases].

Pierre Robin syndrome is typically characterized by glossoptosis and retrognathia associated with cleft palate, respiratory and deglutition disturbances. Two morphological tendencies arise out of this 70-patient series, 50 of whom were managed with the same therapeutical regimen, which included labioglossopexy and was associated with only one case of death: "deforming" type Pierre Robin syndrome most commonly follows a favorable course with respect to both function and facial deformations, provided labioglossopexy is carried out sufficiently early. Indeed, this surgical operation allows for prompt restoration of normal function, thereby limiting the dysfunction-deformation vicious circle. This form of Pierre Robin syndrome may find its origin in the embryo's abnormal amniotic environment. "Malformation"-associated Pierre Robin syndrome often presents with combined anomalies, respiratory disturbances that may evolve into severe airway problems, impaired deglutition and abnormal brain development; in most cases, labioglossopexy will dramatically improve the immediate state of the child, although long-term prognosis as regards malformation is rather guarded.

Adolescent↗
PubMed1998

[Jejunal enteral feeding in a severe case of reflux esophagitis in an infant with Pierre-Robin syndrome].

A case of Pierre-Robin syndrome associated with gastroesophageal reflux and oesophagitis is described. The infant was firstly fed with a hydrolysed-casein formula by gastrostomy, but there was not any improvement of his clinical status, in spite of an appropriate nutrient intake. For this reason, a jejunal enteral feeding, with a semi-elemental formula and then with a hydrolysed-casein formula was started. During this nutritional treatment, the infant showed an adequate weight gain and after 52 days the weight increased from 2880 g to 4580 g.

Enteral Nutrition↗
PubMed1984

On the pathogenesis of cleft palate in the Pierre Robin syndrome.

In a series of Pierre Robin syndrome (PRS) and isolated cleft palate patients (ICP) both U- and V-formed clefts were observed with equal frequency, but the PRS clefts were in average slightly wider. There were totally submucous clefts among the PRS patients. There was no statistical difference between the groups in the prevalence of clefts in the relatives of the patients. The incidence of (genetically influenced) conical elevations in the lower lip was lowest in the noncleft subjects, high in ICP and highest in PRS children. The incidence of hypodontia, which acceptedly is genetically influenced, was also highest in the PRS group. Thus the foetal malposition with the tongue between the palatal shelves does not seem to play any decisive role in the pathogenesis of most PRS clefts. The cause for the PRS is more likely a genetically influenced growth disturbance in the maxilla and the mandible, which due to the organogenetic differences leads to diverging end results, micrognathia and cleft.

Anodontia↗
PubMed1995

Laryngeal mask airway guided tracheal intubation in a neonate with the Pierre Robin syndrome.

Endotracheal intubation in infants with the Pierre Robin syndrome may sometimes be impossible to accomplish by conventional means. To aid difficult tracheal intubation many different techniques have been described. We present a case, in which we successfully intubated a small-for-date newborn boy with the Pierre Robin syndrome by using a modified laryngeal mask airway (no. 1) as a guide for the endotracheal tube. The technique is easy to perform, less traumatic and less time-consuming than multiple attempts at laryngoscopy or blind tracheal intubation.

Equipment Design↗
PubMed1989

A comparative clinical study of Pierre Robin syndrome and isolated cleft palate.

Thirty seven infants with Pierre Robin syndrome were compared with children who had isolated cleft palate. A female preponderance was seen in both groups. Skeletal II jaw relation was observed in 32% of the children with isolated cleft palate. Associated malformations such as hypertelorism and strabismus were more common in infants with Pierre Robin syndrome and also in those with isolated cleft palate combined with a skeletal II jaw relationship when compared with infants who had cleft palate with a skeletal I jaw relationship. The frequency of hypodontia also was greater in the former two groups than in the latter. Furthermore, no difference was found in the frequencies of U- and V-shaped clefts. The frequencies of near relatives with clefts were also not different between groups. Whilst it may be possible to observe from these findings an association between Pierre Robin syndrome and isolated cleft palate, it is difficult to conclude that they support any of the postulates regarding the aetiology of Pierre Robin syndrome.

Child, Preschool↗
PubMed1978

Prosthodontic treatment for Pierre Robin syndrome.

A brief review of the Pierre Robin syndrome and the various methods of treatment employed for children with the condition have been outlined. A suggested treatment approach utilizing an acrylic resin obturator with an adjustable wire tongue restraint has been discussed.

Acrylic Resins↗
PubMed1983

[A new concept of Pierre Robin syndrome and disease: dysneurulation of the rhombencephalon].

Pierre Robin's syndrome, a disorder apparently constituted by peripheral signs, is in fact the result of early major disturbances of ontogenesis of motor and regulatory organization of the fetal rhombencephalon. This is confirmed by the presenting signs in neonates with Pierre Robin's syndrome: --electrophysiological deglutition and sucking disorders as demonstrated on electromyography; --disorders in tone of tongue, pharyngeal and laryngeal muscles; --cardiac and respiratory regulatory disorders as shown by central and obstructive apneas with diminished oxygen pressure and bradycardia of central origin during sleep; and gastro-esophageal reflux. Associated signs indicating that Pierre Robin's syndrome is a separate disease entity are: --evidence of a rhomboencephalic neurocristopathy (malformation of 3rd and 4th aortic arch arteries, thymic and parathyroid hypoplasia) associated with a central rhomboencephalic lesion and resulting in dysneurulation; --mesencephalic lesion as seen in Stickler's syndrome and prosencephalic lesion as in Binder's syndrome, indicating more diffuse cephalic dysneurulation. The common origin of Di George's and Pierre Robin's syndromes is emphasized, the neonatal microretrognathism of the latter syndrome being a bulbar sign. Pierre Robin's syndrome has a poor prognosis, as there is a fatal outcome in one out of four neonates affected, and it appears to be an affection that is the clinical expression of an early major anomaly of cephalic neurulation.

DiGeorge Syndrome↗
PubMed1983

The hand in the Pierre Robin syndrome.

In reviewing eight cases of children with the Pierre Robin syndrome, we found three cases to have abnormalities of the extremities such as syndactyly, hypoplastic digits, and a Poland syndrome. These hand abnormalities have not been previously emphasized in patients with the Pierre Robin syndrome.

Abnormalities, Multiple↗
PubMed1990

[Ophthalmologic manifestations of the Pierre Robin syndrome. Report of a case of microphthalmia].

The classical description of the Pierre Robin syndrome includes micrognathia, glossoptosis, airway obstruction, and usual presence of a cleft palate. The Pierre Robin syndrome is currently defined as the combination of retrognathia, cleft palate, and respiratory distress. This last is mixed, with a peripheral component due to glossoptosis and a central component due to brain stem immaturity. The main ocular manifestations found in the Pierre Robin syndrome are congenital glaucoma and severe congenital mypopia responsible for retinal detachment. Microphthalmia is infrequent. We report the case of a neonate with severe Pierre Robin syndrome and major microphthalmia documented by CT scan.

Female↗