Fibreoptic bronchoscopic nasotracheal intubation of a neonate with Pierre Robin syndrome

A case of nasotracheal intubation using a fibreoptic bronchoscope and the Seldinger technique is described. A guide wire was passed through the suction channel of the fiberscope after the epiglottis and the vocal cords were seen; the fiberscope was removed and a nasotracheal tube passed over the wire into the trachea.     

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.     

Pierre Robin syndrome and pulmonary hypertension

Five infants with Pierre Robin syndrome developed evidence of carbon dioxide retention and congestive cardiac failure despite measures to alleviate upper airway obstruction. Investigations included chest radiography, electrocardiography, echocardiography, and cardiac catheterization; pulmonary hypertension was diagnosed. In two cases raised main pulmonary artery pressures of 40 mm Hg and 120 mm Hg were recorded. Relief of upper airway obstruction was achieved by tracheostomy in three cases and nasopharyngeal intubation in two cases, with reversal of signs of cor pulmonale in each. Four patients progressed well with no recurrence of cardiac problems but one died suddenly one month after apparently successful management by tracheostomy.     

Acute airway obstruction in an infant with Pierre Robin syndrome after palatoplasty

This report describes a complication of post-operative oedema of the palate, tongue and pharynx after a Perko-revised cleft palate repair, which resulted in a life-threatening airway obstruction in an infant with Pierre Robin syndrome. Although infants experiencing airway problems after Wardill-Kilner, von Langenbeck and Furlow palatoplasty have been described, airway complications in a group of Perko-revised repair children have not been previously reported. We speculate that this complication, which occurred in the absence of a history of previous airway problems, is due to prolonged operating time and excessive pressure exerted on the base of the tongue by the Kilner-Doughty retractor. Acknowledgments of this risk permits to identify those patients prior to surgery so that they can be managed appropriately.     

Airway management in two of newborns with Pierre Robin Sequence: the use of disposable vs multiple use LMA for fiberoptic intubation

In this article, we discuss the use of LMAs as a conduit to intubate the trachea of two Pierre Robin Sequence infants. Multiple use LMAs will admit larger diameter tracheal tubes (TT) than their disposable counterparts. Increased friction with the surface of the TT makes passing even small diameter tubes through the lumen of the disposable LMA difficult.     

Pierre Robin syndrome associated with bilateral femoral aplasia, shortening of upper extremities, right kidney agenesis and left kidney malformation

A 5-year-old female infant with a extremely rare combination of Pierre Robin syndrome and multiple malformations of the limbs and kidneys is presented. Orthopaedic, hearing, intellectual and speech disabilities and treatment difficulties are discussed.     

Clinical experience with the Pierre Robin sequence

The Pierre Robin sequence manifests itself in the neonatal period with symptoms of respiratory distress and feeding difficulties. We report our experience in the clinical management of this entity over 14 years and present an appraisal of risk factors for the surgical treatment of the cleft lip/palate and late outcome. Between 1986 and 1999, out of a series of 159 consecutive patients operated in our department for cleft palate closure, 30 had Pierre Robin sequence. Conservative pediatric management included positioning and cardiorespiratory monitoring. In the case of failure of the conservative treatment, endotracheal intubation and/or different surgical procedures were used. The cleft palate was closed according to Maleks technique. Associated malformations were found in 10 cases (33%) with cardiorespiratory immaturity in five of those patients. Acute respiratory distress was present in 12 newborns (40%). Among them, endotracheal intubation was necessary in seven cases and maintained for 1 to 4 days in three patients. Glossopexy or subperiosteal release of the floor of mouth musculature was done in four of these patients followed by tracheotomy in two. Nasogastric feeding was needed in 14 infants (47%) for a variable period with a mean of 57 days (range 1–210 days). Polysomnography studies, done in 24 patients, showed significant obstructive apnea episodes in 10 infants. Gastroesophageal reflux was found in 11 patients (37%). Hearing loss was found in 29 infants (96%) with a mean threshold of 40 dB. Sensorineural hearing abnormalities were revealed in 10 patients (33%). Palate repair was done at the average age of 5 months (range 3–12 months). Difficulties of intubation were encountered in all the cases with severe malformations (five infants). Myringotomy and/or ventilation tubes were used in 21 infants at the same time of palate closure. An early palatal plate was used before surgery in 18 cases (60%). Immediate postoperative complications included two local hemorrhages and in one of them a surgical exploration was needed to control the bleeding. Postoperative oronasal fistula occurred in eight patients (23%) and further surgery was needed for five of them (16%). Newborns who had associated malformation or severe respiratory distress presented more postoperative complications than those with minimal clinical problems (6/10 vs 2/20 patients respectively, p=0.007).     

牵引成骨术治疗新生儿Pierre Robin综合征呼吸阻塞

目的 探讨应用牵引成骨术治疗新生儿Pierre Robin综合征呼吸阻塞的可行性.方法 2007至2009年,用骨牵引技术治疗8例新生儿Pierre Robin综合征.行双侧下颌骨斜行截骨,安置下颌骨牵张器,术后第1天开始牵引,每天3次,每次0.4 mm,每天牵引1.2 mm,直至延长到所需长度.结果 8例患儿均按设计要求顺利完成牵引,无感染发生,无口角歪斜等面神经损伤症状.骨牵引达到预期的长度,约12～20 mm,平均15 mm.Pierre Robin综合征患儿的阵发性青紫、吸气性呼吸困难及哺乳困难等症状均消失.结论 牵引成骨术是治疗新生儿Pierre Robin综合征严重呼吸阻塞比较理想的手术方法,是可行和安全的.     

A dynamic fixation of the base of the tongue to the mandible using de-epithelized tongue flap in the Pierre Robin Syndrome

Summary A 20-months-old infant who suffered from the Pierre Robin Syndrome and subsequently of right heart failure is presented. The baby was operated on successfully, fixing the base of the tongue to the mandible by means of a de-epithelized tongue muscle flap, simulating the course of the genioglossus muscle. Alternative traditional methods to deal with the problem are listed.The authors dedicate this paper to the dear memory of their teacher, the late Prof. Zvi Neuman, Former Head of the Department of Plastic and Maxillofacial Surgery, who died on March 22, 1977     

Kyphomelic dysplasia,Pierre Robin Sequence and pregnant

We present the anaesthetic management of a parturient with kyphomelic dysplasia and Pierre Robin Sequence who underwent elective caesarean delivery. Potential anaesthetic issues and management strategies are discussed.     

Unexpected difficult intubation in the patient with Morning Glory syndrome

Morning Glory syndrome is an uncommon congenital optic disc anomaly with occasional systemic associations. A case of unsuspected difficult intubation in a three-year old patient is described in this case report.     

Impossible laryngeal intubation in an infant with Fraser syndrome

Congenital webbing of the vocal cords is rare, and is usually incompatible with life. We report a case of an infant with Fraser syndrome who required a surgical airway because of a severe stenosis of her airway secondary to a glottic web. The decision process leading to tracheostomy in this neonate is described. The pertinent features of Fraser syndrome in relation to airway management are discussed.     

Respiratory distress in Pierre Robin: successful use of pharyngeal tube

Background/Purpose

The study describes a safe and least aggressive method to resolve airway obstruction in children born with a Pierre Robin sequence (PRS).

Methods

In a retrospective study, we analyzed the assessment of airway obstruction at birth and for the following months. The definition of PRS was based on the anatomical anomaly triad cleft palate, micro/retrognathia, and glossoptosis with some degree of airway obstruction. We defined 3 categories of children depending on their difficulties of breathing or eating at birth.

Results

From 1984 to 2004, 48 children were born in our hospital with a diagnosis of PRS. There were 32 children with nonsyndromic PRS (nsPRS) and 16 with syndromic PRS (sPRS): respectively, 40% (13) and 32% (5) had slight respiratory and/or feeding problems; 26% (8) and 56% (9), isolated feeding difficulties; 34% (11) and 12% (2), severe respiratory and feeding problems. Pharyngeal tube was used in 8 children with nsPRS and in 2 with sPRS. Neonatal surgery was not necessary. Primary palatoplasty was performed at almost the same time as for the patients with isolated cleft palate.

Conclusions

Children born with PRS have a good prognosis at birth provided that adequate respiratory support is given using either positive airway pressure mask or pharyngeal tube.     

A simple homemade lighted stylet for neonates and infants: a description and case report of its use in an infant with the Pierre Robin anomalad

The glossoptosis and micrognathia associated with Pierre Robin anomalad can make tracheal intubation by conventional laryngoscopy quite difficult. Lighted stylets may be helpful in the successful intubation of infants with this anomalad, but those currently available that are small enough to accommodate 3.0 mm ID tracheal tubes have two major drawbacks limiting their utility: an insufficiently rigid stylet component and a nonadjustable, overly bright light. We describe a lighted stylet that can be easily assembled in the operating room which overcomes these problems and allowed us to successfully intubate a six-day-old with severe Pierre Robin anomalad.     

Pierre Robin sequence: appearances and 25 years of experience with an innovative treatment protocol

Purpose

The aim of the study was to evaluate the largest number of Pierre Robin sequence (PRS) cases to date and its treatment outcome.

Design

This is a retrospective study.

Settings

The study was conducted in a tertiary care university hospital.

Patients

One hundred eighty-eight patients with PRS have been subjected to analysis, defined by the clinical triad of glossoptosis, retro/micrognathia, and cleft or agenesis of the palate, for incidence, risk factors, associated syndromes, other concomitant abnormalities, airway and feeding difficulties, and outcome regarding the neonatal and perinatal surgical and nonsurgical management.

Results

The incidence of PRS was 6.02%. Risk factors were found in 41.5% of mothers with children with PRS. Eleven other syndromes/appearances were associated. Feeding difficulties were seen in 51.8% of the patients. Glossopexy (6.9%) and tracheotomy (2.13%) for airway management were performed very seldom. The suction and drinking plate as well as the surgical treatment protocol may be considered to be the reasons for reduced airway (up to 91.0%), feeding problems (up to 79.9%), and mortality rate (2.1%).

Conclusion

The Pierre Robin sequence, as seen as a heterogeneous group, presents with variation of the cleft palate defects with glossoptosis and concomitant micrognathic mandible. The surgical management and certain prepalatal intervention as nonsurgical management with an innovative treatment strategy were evaluated.     

Unsuspected sublingual mass causing difficult intubation in an infant

A case is described of a difficult intubation in an infant presenting for cleft palate closure, due to an unsuspected sublingual mass. The report stresses the fact that multiple anomalies can coexist in an infant and contribute to difficult intubation through different mechanisms. Various airway management strategies are explored.