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Robin sequence: review of treatment modalities for airway obstruction in 110 cases
International Journal of Pediatric Otorhinolaryngology 65 (2002) 45 – 51 www.elsevier.com/locate/ijporl
Robin sequence: review of treatment modalities for airway obstruction in 110 cases Hsueh-Yu Li *, Lun-Jou Lo a, Ka-Shun Chen b, Kin-Sun Wong c, Kai-Ping Chang a
Department of Otolaryngology, Plastic and Reconstructi6e Surgery, Chang Gung Memorial Hospital, Ghang Gung Uni6ersity, 199, Tung- Hwa North Road, Taipei, Taiwan, ROC b Department of Anesthesiology. Chang Gung Memorial Hospital, Chang Gung Uni6ersity, Taipei, Taiwan, ROC c Department of Pediatrics, Chang Gung Children’s Hospital, Taipei, Taiwan, ROC Received 6 February 2002; received in revised form 24 April 2002; accepted 29 April 2002
Abstract Objecti6e: The objective of this paper is to assess the treatment protocols used in our institution for the management of airway obstruction among patients with Robin sequence and to suggest a rationale for management based on the findings. Method: A retrospective study of 110 children with Robin sequence seen from 1988 to 1997 at the Chang Gung Memorial Hospital was done. Results of the management in airway obstruction and feeding difficulty were studied. Results: Prone posturing was effective in the treatment of mild airway obstruction in 82 patients who had noisy breathing sounds. Twenty-eight infants required endotracheal intubation due to severe respiratory distress. Seven received a tongue to lip adhesion (TLA) operation. Three of these patients showed a relief of airway obstruction, while four of them needed a tracheotomy to maintain a patent airway because of wound dehiscence. Two other patients underwent tracheotomy without TLA. All the six patients who received tracheotomy were eventually decannulated. A nasopharyngeal tube was inserted in two other patients, and one of them improved only temporarily. With regards to feeding difficulties, 46 patients needed nasogastric tube feeding, while the rest were aided through the use of a cleft palate bottle and nipple. A gastrostomy was not needed in any of the patients in this series. Conclusion: The morbidity and mortality among Robin sequence patients had been widely decreased through the teamwork of the pediatrician, anesthesiologist, otolaryngologist, dentist, and plastic surgeon. Based on our experience, conservative management was sufficient for those patients with mild airway obstruction, while endotracheal intubation was required for patients with cyanosis, respiratory failure and sleep apnea. Tracheotomy was a better option than TLA for airway management among patients with failed extubation. © 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Robin Sequence; Airway obstruction; Tracheotomy
1. Introduction
* Corresponding author.
Robin sequence is a congenital anomaly that includes micrognathia (Fig. 1), glossoptosis and
0165-5876/02/$ - see front matter © 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 2 ) 0 0 1 3 1 - 3
46
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
cleft palate (Fig. 2)[1]. Because of micrognathia, the tongue tends to roll back into the throat, and may be sucked into the hypopharynx or trapped in the cleft palate during breathing or swallowing. This occurs especially when the patient is in a supine position [2]. The affected children may present with noisy breathing, snoring, stridor, cyanosis, difficulty in feeding, and aspiration pneumonia. The latter problem may cause more serious consequences such as cor pulmonale or asphyxiation [3]. To date, there are only a few long-term systematic studies of this disease. This paper studies cases of Robin sequence seen in our hospital over a period of 10 years in order to provide a better understanding of the disease’s clinical presentation and hence present a rationale for the management of airway obstruction among such patients.
Fig. 1. Robin sequence patient with micrognathia.
2. Materials and methods The medical records of 110 patients with Robin sequence seen at our institution from 1988 to 1997 were reviewed. The data gathered included: sex, age when treatment was sought, family history, birth weight, birth history, reason why treatment was requested, the presence of other congenital abnormalities and middle ear effusion, the patient’s feeding status, and the treatment methods used to manage airway obstruction and their results. All patients were followed for a minimum of 1 year.
3. Results Of the 110 cases there were 60 males and 50 females. Eleven patients were born premature. Birth weights ranged from 1850 to 4440 g, with an average of 3068 g. Ninety-four subjects (85%) were seen within the first 3 months of birth, six were seen at age 3–12 months, three were seen at age 1– 2 years, two subjects at age 2–5 years, and five subjects at age 5–8 years. Reasons for seeking help included the need for cleft palate repair (64 cases), difficulty in feeding (18 cases), and serious difficulty in breathing (28 cases, 15 of whom presented with aspiration pneumonia). Seventyfive cases were diagnosed with pure Robin sequence, while the rest presented with other congenital abnormalities aside from micrognathia, glossoptosis and cleft palate. Six patients had a family history of cleft palate. Seventy patients had middle ear effusion, and received a ventilation tube for a total of 94 times (some patients received a tube more than once). Forty-six patients required feeding via nasogastric tubes (or feeding tubes), while the rest were fed with the aid of obturators or cleft palate bottles. None of the patients required a gastrostomy. Conservative airway management in the form of placing the patient in a prone position was initially done on all the patients. The airway obstruction in 82 cases (74.5%) was relieved by this procedure (their breathing difficulties gradually disappeared as they grew older) while in 28 cases (25.5%) it persisted. Two of these patients had
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
47
Fig. 2. Robin sequence patient with a U-shaped cleft palate.
had nasopharyngeal tubes inserted (Fig. 3), one of whom obtained relief, but had an episode of aspiration pneumonia and was treated by intubation at another hospital. An endotracheal tube was inserted in the other patient due to poor response. The other 26 patients were intubated for a maximal duration of 3 weeks. Seventeen of these patients were eventually decannulated while the rest underwent further management. Seven patients underwent tongue to lip adhesion (TLA)(Fig. 4). Among them the breathing problems of three patients were relieved while in four patients the wounds ruptured necessitating a tracheotomy to maintain a patent airway. Two other patients underwent a tracheotomy directly. These six patients had tracheostomy tubes for a period of 8 –36 months (average, 23 months) and manifested normal breathing after removal of the tubes. One patient with congenital heart disease died because of heart failure and one was lost to follow-up. A schematic diagram depicting our management protocol is shown in Fig. 5.
4. Discussion Airway obstruction among patients with Robin sequence can be treated through various methods based on the severity of the obstruction. These methods include adoption of a prone position [3], insertion of a nasopharyngeal tube [4], TLA procedure [5], endotracheal intubation and tracheostomy [6]. Putting the patient in a prone position causes the tongue to fall forward, thus enlarging the passageway for air in the throat. It is usually the first treatment option for airway obstruction in Robin sequence patients. Tomaki et al. [7] reported that this method alone could achieve improvement in 70% of the cases. In the present study, 74.5% of the patients were able to breathe with better ease after being placed in a prone position. This position is generally maintained for 5–6 months until the breathing becomes smoother and the patient can be put in a supine position. When placement in a prone position fails to alleviate airway obstruction, some clinicians advo-
48
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
cate the use of nasopharyngeal tubes. This is done by inserting the tube into one nostril and fixing its other end behind the tongue base at the top of the larynx [2]. This method not only ensures sufficient space for the passage of air within the tube, but it also prevents the tongue from falling backwards, and therefore reduces the buildup of negative pressure in the back of hypopharynx [2]. Olson et al. [8] stated that with the cooperation of the parents, this method was worthy of greater use, because it allowed the patients to be discharged from the hospital earlier. Later investigation [8] showed that although this method was simple, it was difficult to keep the tube in an appropriate position in the throat because of constant movement of the patient’s head. Furthermore, coughing or swallowing actions could change the position of the tube end. If the tube moved upward, the base of tongue could fall back again, causing a renewed obstruction of the airway. If
the tube moved downward, it could irritate the larynx, and stimulate the gag reflex [5]. For these reasons, patients with severe airway obstruction who require a long-term intubation are not candidates for this treatment method. The authors feel that allowing the patient to go home with a nasopharyngeal tube in place, without proper monitoring of blood oxygen levels, is a potentially dangerous and inappropriate practice. Between the two patients who were fitted with nasopharyngeal tubes in this study, one obtained relief of symptoms after 3 weeks, and the tube was removed but later developed aspiration pneumonia; while the other patient was switched to an endotracheal tube after 4 weeks because of failure to obtain improvement of symptoms. Indeed, the authors feel that placement of the nasopharyngeal tubes should be limited to short periods among in-hospital patients. When the patient’s breathing improves, the tube can then be removed and the
Fig. 3. A nasopharyngeal tube inserted behind the base of the tongue to keep the airway patent.
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
49
Fig. 4. Robin sequence patient after tongue to lip adhesion.
patient kept under close observation. If there is no improvement of breathing or the breathing difficulty recurs, endotracheal intubation or tracheotomy may be required. The goal of the TLA procedure is to fix the tongue in a forward position on the lower jaw, to prevent it from falling backward and obstructing the airway. Although this is a simple method of treatment, it is not always successful. There are several reasons for this: (1). The muscles of the tongue are extremely strong, and often cause the wound to tear. (2). Robin sequence patients frequently present with other neuromuscular disorders in addition to a pure mechanical obstruction of the airway [9]. (3). When the airway of these patients are observed via a fiberscopic examination during sleep, the mechanism which causes the airway obstruction is found to be not only the dorsum of the tongue moving backwards (Type 1) but also the tongue becoming caught in the cleft of the palate (Type 2), the lateral pharyngeal wall moving medially (Type 3), and the pharyngeal wall contracting in a circular manner (Type 4)[10].
In addition, fixing the tongue in the appropriate position is extremely difficult. If the tongue is fixed too far backward, its dorsum may rise and cause renewed airway obstruction. If the tongue is fixed in a position too far forward, it may pull the
Fig. 5. Schematic diagram showing the results of the management of airway obstruction in Robin sequence patients. TLA: Tongue to lip adhesion.
50
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
epiglottis straight, thereby inhibiting its natural function in preventing foreign objects from entering the trachea during swallowing, and making children prone to aspiration pneumonia [5]. All these possibilities should be thoroughly discussed with the patient’s parents prior to surgery. The present study included seven patients who underwent the TLA procedure, three of which were successful. The remaining four did not show satisfactory results. Two patients who improved quickly had had fibroscopic examinations prior to surgery, which revealed that the cause of their airway obstruction was the backward and downward movement of the tongue (Type 1). Therefore, the authors feel that patients who do not respond to prone position therapy should first undergo fiberoptic examination before TLA is performed. If the examination shows that the airway obstruction is Type 1, TLA procedure could be attempted. If result of TLA is poor then a tracheotomy may need to be performed. Robin sequence patients with cyanosis, respiratory failure and sleep apnea require immediate endotracheal intubation to keep airway patent. It is often difficult to intubate these patients, as their tongues are far back in the throat where they block the larynx [11]. In our experience, choosing a straight anesthetic laryngoscope and inserting from the side of the tongue instead of directly from the middle is the best method. The endotracheal tube should have a stylet and no muscle relaxants should be used. An experienced pediatric anesthesiologist best performs this type of intubation. Among the patients in this study, the duration of endotracheal intubation did not exceed 3 weeks before definitive management. There were no serious consequences observed in this population. In the past, tracheotomy had not been considered as the ideal method of maintaining an open airway in Robin sequence patients [12] Previous reports suggested that the obstruction of the airway was caused solely by the backward and downward movement of the root of the tongue. Because tracheotomy did not address this problem and only created a detour, it was not considered to be a direct therapy. Higher mortality rate of tracheotomy in pediatric patients in the past
and the requirement for a longer hospital stay, usually several months until the tube was removed, as compared with the several weeks’ stay required for the TLA procedure, made the latter procedure more favorable [12]. In the recent years, however, many authors have discovered that the results obtained in using TLA procedure for the treatment of airway obstruction in Robin sequence were not as good as the original report [5]. Conversely, pediatric tracheotomy has benefited from improvements in surgical technique and clinical care in the recent years. This had caused the mortality rate to drop and allowed the patients to be discharged as soon as their condition stabilized so that they could be cared for at home. This had greatly reduced the required hospital stay. As the patients got older, their mandibles developed, and their ability to control the muscles of the tongue improved. A gradual enlargement in the size of the airway could be seen such that the tracheotomy tube could eventually be removed. In this series, the indications for tracheotomy were extubation failure or disruption of TLA causing airway compromise. The tubes were removed after an average of about 2 years. Generally, the tubes were kept in place even after the patient had undergone corrective surgery for cleft palate since the tube not only provided a route for general anesthesia during surgery but also helped to prevent possible deterioration in breathing following palatoplasty. At our institution, the Von Langenbeck palatoplasty was performed on Robin sequence patients at the age of 1 year unless they were first seen at an older age. Airway obstruction in these patients was always alleviated or controlled before palatal surgery. The severity of breathing difficulties did not influence the timing of surgery, and there was no postoperative airway compromise in this series. Nevertheless, the authors suggest that the oxygen saturation of the patients be monitored during the postoperative period in order to immediately detect the possible appearance of airway obstruction. Mandibular distraction osteogenesis has been performed in the treatment of upper airway obstruction in patients with craniofacial deformities in the recent years [13]. The results were excellent
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
in allowing the decannulation of patients with tracheostomies and the elimination of refractory sleep apnea [14]. The advantages of this new technique include the increase in airway size at the level of the tongue base, and cosmetic improvement of the facial contour. The disadvantages could include mandibular asymmetry, overcorrection of the length, and tooth injury [15]. Feeding problems are extremely common among patients with Robin sequence. The degree of feeding difficulty is usually related to the severity of the airway obstruction. Thus, treatment should primarily seek to improve the airway. Some specially designed assisting devices for infants including cleft palate bottles and nipples can allow patients to suckle without expending too much effort. Proper education of the parents is also important to guide them how to feed their children to avoid aspiration and choking. For patients who cannot feed, temporary placement of nasogastric tubes (or feeding tubes) is necessary. Once the clinical symptoms improve, bottle-feeding is begun as soon as possible. All the patients in this study could eat and drink or suckle normally at the time of follow-up and none of them required gastrostomy.
5. Conclusion In this 10-year retrospective study we found that airway obstruction in most Robin sequence patients manifested in noisy breathing. Three fourths of the subjects (82 out of 110) experienced relief of symptoms through conservative management, while one fourth (28 out of 110) required endotracheal intubation to maintain airway patency. Among those who required intubations, more than half of the patients (17 out of 28) were extubated without need for further airway treatment while the rest required definitive management. Among the interventions used to treat airway difficulties we found that TLA was not as successful as expected especially for patients with severe airway obstruction. Fibroscopic airway examination to determine the type of obstruction prior to TLA may be a useful tool. Nasopharyngeal tubes provided inadequate relief. Tra-
51
cheotomy proved to a more reliable method than TLA in managing failed extubations. Acknowledgements The authors would like to thank Dr Kai-Fong Hung for assisting with this paper. References [1] J.D. Smith, Treatment of airway obstruction in Pierre Robin syndrome, Arch. Otolaryngol. 107 (1981) 419 – 421. [2] B. Benjamin, P. Walker, Management of airway obstruction in Pierre Robin, Intern. J. Pediatr. Otolaryngol. 22 (1991) 29 – 37. [3] U. Frohberg, R.T. Lange, Surgical treatment of Robin sequence and sleep apnea syndrome, J. Oral. Maxillofac. Surg. 51 (1993) 1274 – 1283. [4] D.P. Heaf, P.J. Helms, R. Dinwiddie, et al., Nasopharyngeal airways in Pierre Robin syndrome, J. Pediatr. 100 (1982) 670 – 689. [5] A. Augarten, M. Sagy, J. Yahav, Z. Barzilay, Management of upper airway obstruction in the Pierre Robin syndrome, Brit. J. Oral. Maxillofac. Surg. 28 (1990) 105 – 108. [6] A.P. Bath, P.D. Bull, Management of airway obstruction in Pierre Robin sequence, J. Laryngol. Otol. 111 (1997) 1155 – 1157. [7] S.M. Tomaski, G.H. Zalzal, H.M. Saal, Airway obstruction in Pierre Robin sequence, Laryngoscope 105 (1995) 111 – 114. [8] T.S. Olson, D.B. Kearn, S.M. Pransky, A. Seud, Early home management of patients with Pierre Robin sequence, Int. J. Otorhinolaryngol. 20 (1990) 45 – 49. [9] R.J. Shprintzen, The implication of the diagnosis of Robin sequence, Cleft Palate Craniofac. J. 29 (1992) 205 – 209. [10] A.E. Sher, R.J. Shprintzen, M.J. Thorpy, Endoscopic observations of obstructive sleep apnea in children with anomalous upper airway: Predictive and therapeutic value, Int. J. Pediatr. Otorhinolaryngol. 11 (1986) 1335 – 1346. [11] S.D. Handler, T.P. Keon, Difficult laryngoscopy/intubation: The child with mandibular hypoplasia, Ann. Otol. Laryngol. 92 (1983) 401 – 404. [12] W.S. Line, D.B. Haawkins, E.J. Kahistrom, et al., Tracheostomy in infants and young children: The changing perspective, Laryngoscope 96 (1986) 510 – 515. [13] J.D. Sidman, D. Sampson, B. Templeton, Distraction osteogenesis of the mandible for airway obstruction in children, Laryngoscope 111 (2001) 1137 – 1146. [14] S.R. Cohen, C. Simms, F.D. Burstein, Mandibular distraction osteogenesis in the treatment of upper airway obstruction in children with craniofacial deformities, Plast. Reconstr. Surg. 101 (1998) 312 – 318. [15] A.K. Gosain, Distraction osteogenesis of the craniofacial skeleton, Plast. Reconstr. Surg. 107 (2001) 278 – 280.
Robin sequence: review of treatment modalities for airway obstruction in 110 cases Hsueh-Yu Li *, Lun-Jou Lo a, Ka-Shun Chen b, Kin-Sun Wong c, Kai-Ping Chang a
Department of Otolaryngology, Plastic and Reconstructi6e Surgery, Chang Gung Memorial Hospital, Ghang Gung Uni6ersity, 199, Tung- Hwa North Road, Taipei, Taiwan, ROC b Department of Anesthesiology. Chang Gung Memorial Hospital, Chang Gung Uni6ersity, Taipei, Taiwan, ROC c Department of Pediatrics, Chang Gung Children’s Hospital, Taipei, Taiwan, ROC Received 6 February 2002; received in revised form 24 April 2002; accepted 29 April 2002
Abstract Objecti6e: The objective of this paper is to assess the treatment protocols used in our institution for the management of airway obstruction among patients with Robin sequence and to suggest a rationale for management based on the findings. Method: A retrospective study of 110 children with Robin sequence seen from 1988 to 1997 at the Chang Gung Memorial Hospital was done. Results of the management in airway obstruction and feeding difficulty were studied. Results: Prone posturing was effective in the treatment of mild airway obstruction in 82 patients who had noisy breathing sounds. Twenty-eight infants required endotracheal intubation due to severe respiratory distress. Seven received a tongue to lip adhesion (TLA) operation. Three of these patients showed a relief of airway obstruction, while four of them needed a tracheotomy to maintain a patent airway because of wound dehiscence. Two other patients underwent tracheotomy without TLA. All the six patients who received tracheotomy were eventually decannulated. A nasopharyngeal tube was inserted in two other patients, and one of them improved only temporarily. With regards to feeding difficulties, 46 patients needed nasogastric tube feeding, while the rest were aided through the use of a cleft palate bottle and nipple. A gastrostomy was not needed in any of the patients in this series. Conclusion: The morbidity and mortality among Robin sequence patients had been widely decreased through the teamwork of the pediatrician, anesthesiologist, otolaryngologist, dentist, and plastic surgeon. Based on our experience, conservative management was sufficient for those patients with mild airway obstruction, while endotracheal intubation was required for patients with cyanosis, respiratory failure and sleep apnea. Tracheotomy was a better option than TLA for airway management among patients with failed extubation. © 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Robin Sequence; Airway obstruction; Tracheotomy
1. Introduction
* Corresponding author.
Robin sequence is a congenital anomaly that includes micrognathia (Fig. 1), glossoptosis and
0165-5876/02/$ - see front matter © 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 2 ) 0 0 1 3 1 - 3
46
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
cleft palate (Fig. 2)[1]. Because of micrognathia, the tongue tends to roll back into the throat, and may be sucked into the hypopharynx or trapped in the cleft palate during breathing or swallowing. This occurs especially when the patient is in a supine position [2]. The affected children may present with noisy breathing, snoring, stridor, cyanosis, difficulty in feeding, and aspiration pneumonia. The latter problem may cause more serious consequences such as cor pulmonale or asphyxiation [3]. To date, there are only a few long-term systematic studies of this disease. This paper studies cases of Robin sequence seen in our hospital over a period of 10 years in order to provide a better understanding of the disease’s clinical presentation and hence present a rationale for the management of airway obstruction among such patients.
Fig. 1. Robin sequence patient with micrognathia.
2. Materials and methods The medical records of 110 patients with Robin sequence seen at our institution from 1988 to 1997 were reviewed. The data gathered included: sex, age when treatment was sought, family history, birth weight, birth history, reason why treatment was requested, the presence of other congenital abnormalities and middle ear effusion, the patient’s feeding status, and the treatment methods used to manage airway obstruction and their results. All patients were followed for a minimum of 1 year.
3. Results Of the 110 cases there were 60 males and 50 females. Eleven patients were born premature. Birth weights ranged from 1850 to 4440 g, with an average of 3068 g. Ninety-four subjects (85%) were seen within the first 3 months of birth, six were seen at age 3–12 months, three were seen at age 1– 2 years, two subjects at age 2–5 years, and five subjects at age 5–8 years. Reasons for seeking help included the need for cleft palate repair (64 cases), difficulty in feeding (18 cases), and serious difficulty in breathing (28 cases, 15 of whom presented with aspiration pneumonia). Seventyfive cases were diagnosed with pure Robin sequence, while the rest presented with other congenital abnormalities aside from micrognathia, glossoptosis and cleft palate. Six patients had a family history of cleft palate. Seventy patients had middle ear effusion, and received a ventilation tube for a total of 94 times (some patients received a tube more than once). Forty-six patients required feeding via nasogastric tubes (or feeding tubes), while the rest were fed with the aid of obturators or cleft palate bottles. None of the patients required a gastrostomy. Conservative airway management in the form of placing the patient in a prone position was initially done on all the patients. The airway obstruction in 82 cases (74.5%) was relieved by this procedure (their breathing difficulties gradually disappeared as they grew older) while in 28 cases (25.5%) it persisted. Two of these patients had
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
47
Fig. 2. Robin sequence patient with a U-shaped cleft palate.
had nasopharyngeal tubes inserted (Fig. 3), one of whom obtained relief, but had an episode of aspiration pneumonia and was treated by intubation at another hospital. An endotracheal tube was inserted in the other patient due to poor response. The other 26 patients were intubated for a maximal duration of 3 weeks. Seventeen of these patients were eventually decannulated while the rest underwent further management. Seven patients underwent tongue to lip adhesion (TLA)(Fig. 4). Among them the breathing problems of three patients were relieved while in four patients the wounds ruptured necessitating a tracheotomy to maintain a patent airway. Two other patients underwent a tracheotomy directly. These six patients had tracheostomy tubes for a period of 8 –36 months (average, 23 months) and manifested normal breathing after removal of the tubes. One patient with congenital heart disease died because of heart failure and one was lost to follow-up. A schematic diagram depicting our management protocol is shown in Fig. 5.
4. Discussion Airway obstruction among patients with Robin sequence can be treated through various methods based on the severity of the obstruction. These methods include adoption of a prone position [3], insertion of a nasopharyngeal tube [4], TLA procedure [5], endotracheal intubation and tracheostomy [6]. Putting the patient in a prone position causes the tongue to fall forward, thus enlarging the passageway for air in the throat. It is usually the first treatment option for airway obstruction in Robin sequence patients. Tomaki et al. [7] reported that this method alone could achieve improvement in 70% of the cases. In the present study, 74.5% of the patients were able to breathe with better ease after being placed in a prone position. This position is generally maintained for 5–6 months until the breathing becomes smoother and the patient can be put in a supine position. When placement in a prone position fails to alleviate airway obstruction, some clinicians advo-
48
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
cate the use of nasopharyngeal tubes. This is done by inserting the tube into one nostril and fixing its other end behind the tongue base at the top of the larynx [2]. This method not only ensures sufficient space for the passage of air within the tube, but it also prevents the tongue from falling backwards, and therefore reduces the buildup of negative pressure in the back of hypopharynx [2]. Olson et al. [8] stated that with the cooperation of the parents, this method was worthy of greater use, because it allowed the patients to be discharged from the hospital earlier. Later investigation [8] showed that although this method was simple, it was difficult to keep the tube in an appropriate position in the throat because of constant movement of the patient’s head. Furthermore, coughing or swallowing actions could change the position of the tube end. If the tube moved upward, the base of tongue could fall back again, causing a renewed obstruction of the airway. If
the tube moved downward, it could irritate the larynx, and stimulate the gag reflex [5]. For these reasons, patients with severe airway obstruction who require a long-term intubation are not candidates for this treatment method. The authors feel that allowing the patient to go home with a nasopharyngeal tube in place, without proper monitoring of blood oxygen levels, is a potentially dangerous and inappropriate practice. Between the two patients who were fitted with nasopharyngeal tubes in this study, one obtained relief of symptoms after 3 weeks, and the tube was removed but later developed aspiration pneumonia; while the other patient was switched to an endotracheal tube after 4 weeks because of failure to obtain improvement of symptoms. Indeed, the authors feel that placement of the nasopharyngeal tubes should be limited to short periods among in-hospital patients. When the patient’s breathing improves, the tube can then be removed and the
Fig. 3. A nasopharyngeal tube inserted behind the base of the tongue to keep the airway patent.
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
49
Fig. 4. Robin sequence patient after tongue to lip adhesion.
patient kept under close observation. If there is no improvement of breathing or the breathing difficulty recurs, endotracheal intubation or tracheotomy may be required. The goal of the TLA procedure is to fix the tongue in a forward position on the lower jaw, to prevent it from falling backward and obstructing the airway. Although this is a simple method of treatment, it is not always successful. There are several reasons for this: (1). The muscles of the tongue are extremely strong, and often cause the wound to tear. (2). Robin sequence patients frequently present with other neuromuscular disorders in addition to a pure mechanical obstruction of the airway [9]. (3). When the airway of these patients are observed via a fiberscopic examination during sleep, the mechanism which causes the airway obstruction is found to be not only the dorsum of the tongue moving backwards (Type 1) but also the tongue becoming caught in the cleft of the palate (Type 2), the lateral pharyngeal wall moving medially (Type 3), and the pharyngeal wall contracting in a circular manner (Type 4)[10].
In addition, fixing the tongue in the appropriate position is extremely difficult. If the tongue is fixed too far backward, its dorsum may rise and cause renewed airway obstruction. If the tongue is fixed in a position too far forward, it may pull the
Fig. 5. Schematic diagram showing the results of the management of airway obstruction in Robin sequence patients. TLA: Tongue to lip adhesion.
50
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
epiglottis straight, thereby inhibiting its natural function in preventing foreign objects from entering the trachea during swallowing, and making children prone to aspiration pneumonia [5]. All these possibilities should be thoroughly discussed with the patient’s parents prior to surgery. The present study included seven patients who underwent the TLA procedure, three of which were successful. The remaining four did not show satisfactory results. Two patients who improved quickly had had fibroscopic examinations prior to surgery, which revealed that the cause of their airway obstruction was the backward and downward movement of the tongue (Type 1). Therefore, the authors feel that patients who do not respond to prone position therapy should first undergo fiberoptic examination before TLA is performed. If the examination shows that the airway obstruction is Type 1, TLA procedure could be attempted. If result of TLA is poor then a tracheotomy may need to be performed. Robin sequence patients with cyanosis, respiratory failure and sleep apnea require immediate endotracheal intubation to keep airway patent. It is often difficult to intubate these patients, as their tongues are far back in the throat where they block the larynx [11]. In our experience, choosing a straight anesthetic laryngoscope and inserting from the side of the tongue instead of directly from the middle is the best method. The endotracheal tube should have a stylet and no muscle relaxants should be used. An experienced pediatric anesthesiologist best performs this type of intubation. Among the patients in this study, the duration of endotracheal intubation did not exceed 3 weeks before definitive management. There were no serious consequences observed in this population. In the past, tracheotomy had not been considered as the ideal method of maintaining an open airway in Robin sequence patients [12] Previous reports suggested that the obstruction of the airway was caused solely by the backward and downward movement of the root of the tongue. Because tracheotomy did not address this problem and only created a detour, it was not considered to be a direct therapy. Higher mortality rate of tracheotomy in pediatric patients in the past
and the requirement for a longer hospital stay, usually several months until the tube was removed, as compared with the several weeks’ stay required for the TLA procedure, made the latter procedure more favorable [12]. In the recent years, however, many authors have discovered that the results obtained in using TLA procedure for the treatment of airway obstruction in Robin sequence were not as good as the original report [5]. Conversely, pediatric tracheotomy has benefited from improvements in surgical technique and clinical care in the recent years. This had caused the mortality rate to drop and allowed the patients to be discharged as soon as their condition stabilized so that they could be cared for at home. This had greatly reduced the required hospital stay. As the patients got older, their mandibles developed, and their ability to control the muscles of the tongue improved. A gradual enlargement in the size of the airway could be seen such that the tracheotomy tube could eventually be removed. In this series, the indications for tracheotomy were extubation failure or disruption of TLA causing airway compromise. The tubes were removed after an average of about 2 years. Generally, the tubes were kept in place even after the patient had undergone corrective surgery for cleft palate since the tube not only provided a route for general anesthesia during surgery but also helped to prevent possible deterioration in breathing following palatoplasty. At our institution, the Von Langenbeck palatoplasty was performed on Robin sequence patients at the age of 1 year unless they were first seen at an older age. Airway obstruction in these patients was always alleviated or controlled before palatal surgery. The severity of breathing difficulties did not influence the timing of surgery, and there was no postoperative airway compromise in this series. Nevertheless, the authors suggest that the oxygen saturation of the patients be monitored during the postoperative period in order to immediately detect the possible appearance of airway obstruction. Mandibular distraction osteogenesis has been performed in the treatment of upper airway obstruction in patients with craniofacial deformities in the recent years [13]. The results were excellent
H.-Y. Li et al. / Int. J. Pediatr. Otorhinolaryngol. 65 (2002) 45–51
in allowing the decannulation of patients with tracheostomies and the elimination of refractory sleep apnea [14]. The advantages of this new technique include the increase in airway size at the level of the tongue base, and cosmetic improvement of the facial contour. The disadvantages could include mandibular asymmetry, overcorrection of the length, and tooth injury [15]. Feeding problems are extremely common among patients with Robin sequence. The degree of feeding difficulty is usually related to the severity of the airway obstruction. Thus, treatment should primarily seek to improve the airway. Some specially designed assisting devices for infants including cleft palate bottles and nipples can allow patients to suckle without expending too much effort. Proper education of the parents is also important to guide them how to feed their children to avoid aspiration and choking. For patients who cannot feed, temporary placement of nasogastric tubes (or feeding tubes) is necessary. Once the clinical symptoms improve, bottle-feeding is begun as soon as possible. All the patients in this study could eat and drink or suckle normally at the time of follow-up and none of them required gastrostomy.
5. Conclusion In this 10-year retrospective study we found that airway obstruction in most Robin sequence patients manifested in noisy breathing. Three fourths of the subjects (82 out of 110) experienced relief of symptoms through conservative management, while one fourth (28 out of 110) required endotracheal intubation to maintain airway patency. Among those who required intubations, more than half of the patients (17 out of 28) were extubated without need for further airway treatment while the rest required definitive management. Among the interventions used to treat airway difficulties we found that TLA was not as successful as expected especially for patients with severe airway obstruction. Fibroscopic airway examination to determine the type of obstruction prior to TLA may be a useful tool. Nasopharyngeal tubes provided inadequate relief. Tra-
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cheotomy proved to a more reliable method than TLA in managing failed extubations. Acknowledgements The authors would like to thank Dr Kai-Fong Hung for assisting with this paper. References [1] J.D. Smith, Treatment of airway obstruction in Pierre Robin syndrome, Arch. Otolaryngol. 107 (1981) 419 – 421. [2] B. Benjamin, P. Walker, Management of airway obstruction in Pierre Robin, Intern. J. Pediatr. Otolaryngol. 22 (1991) 29 – 37. [3] U. Frohberg, R.T. Lange, Surgical treatment of Robin sequence and sleep apnea syndrome, J. Oral. Maxillofac. Surg. 51 (1993) 1274 – 1283. [4] D.P. Heaf, P.J. Helms, R. Dinwiddie, et al., Nasopharyngeal airways in Pierre Robin syndrome, J. Pediatr. 100 (1982) 670 – 689. [5] A. Augarten, M. Sagy, J. Yahav, Z. Barzilay, Management of upper airway obstruction in the Pierre Robin syndrome, Brit. J. Oral. Maxillofac. Surg. 28 (1990) 105 – 108. [6] A.P. Bath, P.D. Bull, Management of airway obstruction in Pierre Robin sequence, J. Laryngol. Otol. 111 (1997) 1155 – 1157. [7] S.M. Tomaski, G.H. Zalzal, H.M. Saal, Airway obstruction in Pierre Robin sequence, Laryngoscope 105 (1995) 111 – 114. [8] T.S. Olson, D.B. Kearn, S.M. Pransky, A. Seud, Early home management of patients with Pierre Robin sequence, Int. J. Otorhinolaryngol. 20 (1990) 45 – 49. [9] R.J. Shprintzen, The implication of the diagnosis of Robin sequence, Cleft Palate Craniofac. J. 29 (1992) 205 – 209. [10] A.E. Sher, R.J. Shprintzen, M.J. Thorpy, Endoscopic observations of obstructive sleep apnea in children with anomalous upper airway: Predictive and therapeutic value, Int. J. Pediatr. Otorhinolaryngol. 11 (1986) 1335 – 1346. [11] S.D. Handler, T.P. Keon, Difficult laryngoscopy/intubation: The child with mandibular hypoplasia, Ann. Otol. Laryngol. 92 (1983) 401 – 404. [12] W.S. Line, D.B. Haawkins, E.J. Kahistrom, et al., Tracheostomy in infants and young children: The changing perspective, Laryngoscope 96 (1986) 510 – 515. [13] J.D. Sidman, D. Sampson, B. Templeton, Distraction osteogenesis of the mandible for airway obstruction in children, Laryngoscope 111 (2001) 1137 – 1146. [14] S.R. Cohen, C. Simms, F.D. Burstein, Mandibular distraction osteogenesis in the treatment of upper airway obstruction in children with craniofacial deformities, Plast. Reconstr. Surg. 101 (1998) 312 – 318. [15] A.K. Gosain, Distraction osteogenesis of the craniofacial skeleton, Plast. Reconstr. Surg. 107 (2001) 278 – 280.