Cases reported "Pierre Robin Syndrome"

Filter by keywords:



Filtering documents. Please wait...

1/41. Mandibular distraction osteogenesis in a neonate.

    Children with craniofacial anomalies are predisposed to airway obstruction and frequently require airway intervention. tracheotomy is performed when the airway obstruction is severe and refractory to other less invasive interventions. tracheotomy is associated with significant morbidity, and there is a trend noted in the literature toward achieving earlier decannulation by the institution of definitive structural changes to the mandible. Mandibular distraction osteogenesis has been shown to alleviate airway obstruction in the pediatric population. We report a case in which mandibular distraction osteogenesis was successfully carried out in a neonate with acute airway obstruction at birth as a result of combined Pierre Robin sequence and klippel-feil syndrome. After 1 year, the patient still had an adequate airway with tolerable scarring and no neurologic sequelae.
- - - - - - - - - -
ranking = 1
keywords = airway obstruction, airway, obstruction
(Clic here for more details about this article)

2/41. A modified nasopharyngeal tube to relieve high upper airway obstruction.

    Infants with high upper airway obstruction (UAO) are managed with a variety of techniques to relieve their UAO. Among these techniques, the least invasive and safest is the nasopharyngeal tube (NPT). However, the traditional NPT is not always satisfactory, and tracheostomies need to be done. We recently described a modified NPT technique that, in contrast to the traditional tube, does not add airway dead space and resistance, is easy to use, is well-tolerated, has proven highly successful, and allows the simultaneous use of oxygen nasal prongs. This modified NPT has many advantages over the traditional NPT as a temporary management of high UAO that resolves with growth of the infant. This report highlights the respiratory care of 10 infants with high UAO (pierre robin syndrome, down syndrome, goldenhar syndrome, isolated microngathia, and idiopathic hypotonia) who were managed with a modified NPT. The modified NPT described potentially reduces the need for surgical intervention to relieve high UAO in infants.
- - - - - - - - - -
ranking = 1.5602229576729
keywords = airway obstruction, airway, obstruction
(Clic here for more details about this article)

3/41. Late presentation of upper airway obstruction in Pierre Robin sequence.

    A retrospective review was carried out of 11 consecutive patients with the Pierre Robin sequence referred to a tertiary paediatric referral centre over a five year period from 1993 to 1998. Ten patients were diagnosed with significant upper airway obstruction; seven of these presented late at between 24 and 51 days of age. failure to thrive occurred in six of these seven infants at the time of presentation, and was a strong indicator of the severity of upper airway obstruction. growth normalised on treatment of the upper airway obstruction with nasopharyngeal tube placement. All children had been reviewed by either an experienced general paediatrician or a neonatologist in the first week of life, suggesting that clinical signs alone are insufficient to alert the physician to the degree of upper airway obstruction or that obstruction developed gradually after discharge home. The use of polysomnography greatly improved the diagnostic accuracy in assessing the severity of upper airway obstruction and monitoring the response to treatment. This report highlights the prevalence of late presentation of upper airway obstruction in the Pierre Robin sequence and emphasises the need for close prospective respiratory monitoring in this condition. Objective measures such as polysomnography should be used, as clinical signs alone may be an inadequate guide to the degree of upper airway obstruction.
- - - - - - - - - -
ranking = 3.3834748655686
keywords = airway obstruction, airway, obstruction
(Clic here for more details about this article)

4/41. Subperiosteal release of the floor of the mouth musculature in two cases of Pierre Robin sequence.

    Many management approaches have been considered to relieve upper respiratory obstruction in patients with Pierre Robin sequence, but the choice of treatment is determined by the severity of the obstruction. These options include prone positioning, the use of a nasal trumpet, and surgery. One surgical technique is the subperiosteal release of the floor of the mouth musculature. The theory behind this procedure is that this musculature is under tension, and therefore it pushes the tongue upward and backward, resulting in respiratory obstruction. In theory, the release of this musculature from the mandible should alleviate the tension and hence clear the obstruction. In an attempt to objectively evaluate this theory, we performed subperiosteal release surgery on two infants. Our first patient required an emergent tracheostomy on postoperative day 2 because of the onset of surgically induced airway edema. To avoid this complication in the second patient, we performed a tracheostomy at the same time as surgery. Pre- and postoperative magnetic resonance imaging in the second patient revealed only a minimal change in the anatomy of the floor of the mouth musculature. We believe the subperiosteal release of the floor of the mouth musculature requires further evaluation before it can be considered to be effective in the surgical treatment of respiratory obstruction in Pierre Robin sequence.
- - - - - - - - - -
ranking = 0.049396228331806
keywords = airway, obstruction
(Clic here for more details about this article)

5/41. Oesophageal perforation presenting as oesophageal atresia in a premature neonate following difficult intubation.

    Iatrogenic oesophageal perforation in neonates is well recognized in the medical and surgical literature with intubation injury listed as a possible contributing mechanism besides nasogastric tube placement and suctioning. Diagnosis can be difficult and sometimes confused with other entities. With early diagnosis, nonsurgical management often leads to complete resolution in neonates. We report the case of a 1-day-old premature neonate who was brought to the operating room with the preliminary diagnosis of proximal oesophageal atresia with stump perforation and distal tracheo-esophageal fistula. His intubation for respiratory distress at birth had been difficult due to Pierre-Robin sequence with micrognathia. Oesophagoscopy in the operating room revealed a patent oesophagus but perforations in the pharynx and in the proximal oesophagus with the nasogastric tube entering the pharyngeal perforation. Oesophageal perforation and the limitations of the difficult airway algorithm in small neonates are discussed.
- - - - - - - - - -
ranking = 0.024539536240868
keywords = airway
(Clic here for more details about this article)

6/41. airway obstruction following palatoplasty: analysis of 247 consecutive operations.

    OBJECTIVE AND methods: Between February 1987 and September 1997, 247 patients underwent primary repair of a cleft of the secondary palate by one surgeon, using the double-opposing Z-plasty (Furlow) technique. This retrospective study reviews perioperative and postoperative airway compromise among these patients. The purposes of this study were to identify factors associated with airway obstruction following palatoplasty and to analyze the management of those patients. Although infants experiencing airway problems following Wardill-Kilner and Von Langenbeck palatoplasty have been described, airway complications in a group of Furlow repair patients has not been previously reported. RESULTS: Fourteen patients (5.7%) had airway problems. The average age of these patients was 18 months, which was not significantly different from those without airway problems. airway obstruction occurred as late as 48 hours after the completion of surgery. Twelve of the 14 patients had severe airway compromise requiring continued postoperative intubation, reintubation, or tracheostomy (one). There were no deaths. Thirteen of the 14 patients with postoperative airway problems (93%) had other congenital anomalies in addition to clefting, a named congenital disorder, or both. Seven of those 13 had Pierre Robin sequence. In contrast, only 40 of the 233 patients without airway problems (17%) had additional congenital anomalies or named disorders. Presence of other congenital anomalies was associated with a significantly increased risk of airway obstruction (p =.005). CONCLUSION: patients with cleft palate with the Pierre Robin sequence or other additional congenital anomalies had an increased risk of airway problems following palatoplasty. awareness of this risk permits identifying those patients prior to surgery so that they can be monitored and managed appropriately, minimizing the likelihood of major complications or death.
- - - - - - - - - -
ranking = 0.85998588683156
keywords = airway obstruction, airway, obstruction
(Clic here for more details about this article)

7/41. Rigid nasendoscope with video camera system for intubation in infants with Pierre-Robin sequence.

    We describe an alternative intubation technique using a rigid nasendoscope and a video camera monitor system in two infants with Pierre-Robin sequence presenting for palatoplasty. After induction with an inhalational anaesthetic technique, the tracheas of the infants could not be intubated with direct laryngoscopy using a wisconsin blade. In the absence of a flexible paediatric fibrescope, a rigid endoscope (2.7 mm, 70 degrees lateral illumination) was passed orally to provide a view of the glottis on the monitor screen. A tracheal tube, bent into a J-shape using a stylet, was inserted orally and manipulated into the trachea, under video guidance. This technique proved to be simple, permitting a favourable view of the glottis. It should be considered for passing a tracheal tube through the vocal cords in infants who present with a difficult airway.
- - - - - - - - - -
ranking = 0.024539536240868
keywords = airway
(Clic here for more details about this article)

8/41. prenatal diagnosis of Pierre-Robin sequence as part of Stickler syndrome.

    Stickler syndrome or hereditary progressive arthro-ophthalmopathy, is an autosomal dominant condition characterized by ocular manifestations, arthritic changes, orofacial features and deafness, in variable degrees.We report the first case of prenatal diagnosis of Stickler syndrome in a child with a Pierre-Robin sequence (PRS) causing a polyhydramnios. When isolated polyhydramnios is not explained by immunological, metabolic or infectious causes, swallowing difficulty due to PRS must be considered. As PRS is aetiologically heterogeneous, the prognosis depends on the cause. Genetic investigations and familial history must be taken into account. Here, in a context of familial Stickler syndrome, making the prenatal diagnosis of PRS as part of Stickler syndrome allowed us to reassure the parents and to anticipate airway trouble at the child's birth.
- - - - - - - - - -
ranking = 0.024539536240868
keywords = airway
(Clic here for more details about this article)

9/41. Pierre Robin sequence in a patient with ectrodactyly-ectodermal dysplasia-clefting syndrome: a case report and review of the literature.

    Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is a rare condition first identified in 1970. The majority of cases are reported to be autosomal dominant with variable expression and penetrance. The most common clinical feature is ectodermal dysplasia. Ectrodactyly, cleft lip with or with out cleft palate and urogenital anomalies are also main clinical features of EEC syndrome. Pierre Robin (PR) sequence has the clinical findings of micro-retrognathia, retroposed tongue (glossoptosis), cleft of the secondary palate, and upper airway obstruction. Etiologically PR sequence is heterogeneous. It has been reported that less than 20% of cases are isolated, non-syndromic PR sequence. We present a case of a newborn male who was referred to our craniofacial clinic with EEC syndrome and PR sequence. This case represents a unique clinical association. We found no other reports in the literature of these two clinical entities occurring together. Upper airway obstruction of PR sequence, secondary to glossoptosis and micro-retrognathia, gives this association clinical relevance. In addition, this clinical association may represent a new gene locus associated with EEC syndrome.
- - - - - - - - - -
ranking = 0.6142733685728
keywords = airway obstruction, airway, obstruction
(Clic here for more details about this article)

10/41. pierre robin syndrome: case report.

    A case of a female neonate with pierre robin syndrome with frequent cyanotic episodes and feeding difficulties which could not be adequately managed by positioning and oral airway placement is presented. tongue-anterior mandible fusion procedure was performed with satisfactory results.
- - - - - - - - - -
ranking = 0.024539536240868
keywords = airway
(Clic here for more details about this article)
| Next ->


Leave a message about 'Pierre Robin Syndrome'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.