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Congenital nasal pyriform aperture stenosis: non-surgical management and long-term analysis
International Journal of Pediatric Otorhinolaryngology 60 (2001) 167– 171 www.elsevier.com/locate/ijporl
Case report
Congenital nasal pyriform aperture stenosis: non-surgical management and long-term analysis James J. Lee a,*, John P. Bent b, Robert F. Ward c a
Department of Otolaryngology, New York Uni6ersity School of Medicine, 550 First A6enue, New York, NY 10016, USA b Department of Otolaryngology, Albert Einstein School of Medicine, New York Otolaryngology Institute, New York, NY 10021, USA c Department of Otolaryngology, Cornell Uni6ersity Medical Center, New York Otolaryngology Institute, New York, NY, USA Received 28 November 2000; received in revised form 23 April 2001; accepted 24 April 2001
Abstract Congenital nasal pyriform aperture stenosis is a rare cause of airway obstruction in the newborn. Immediate recognition and therapy are essential for this potentially life-threatening condition. After initial management, which includes establishment of an oral airway, surgical repair of the stenotic bony inlet has been traditionally considered. We present a long-term follow-up of two patients with congenital pyriform aperture stenosis, who were managed expectantly. Pertinent embryology, clinical presentation, and general treatment strategies for these patients are also discussed. © 2001 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Maxillofacial abnormalities; Nasal cavity; Nasal obstruction; Nasal pyriform aperture stenosis
1. Introduction Nasal pyriform aperture stenosis has been recognized for many years [1]. However, congenital nasal pyriform aperture stenosis (CNPAS) has only been recently described as a cause of respiratory compromise in infants [2]. Neonates are obligate nasal breathers and therefore may present with various signs and symptoms related to respiratory compromise. * Corresponding author. Tel.: + 1-212-2636344; fax: + 1212-2638257. E-mail address: [email protected] (J.J. Lee).
Initial management of these infants includes establishment of an airway, usually with an oral airway or a McGovern nipple. When patients are able to breathe without an artificial airway and demonstrate adequate oral intake, they are discharged home with nasal decongestants and humidification. Surgical widening of the pyriform aperture stenosis is usually recommended to these infants, as many of them are expected to have difficulties with nasal congestion, feeding, and weight gain. Since the data on the long-term outcome of non-surgically managed patients with pyriform
0165-5876/01/$ - see front matter © 2001 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 1 ) 0 0 5 0 3 - 1
168
J.J. Lee et al. / Int. J. Pediatr. Otorhinolaryngol. 60 (2001) 167–171
aperture stenosis is limited, this paper will present the long-term follow-up of two expectantly managed patients.
2. Case report 1 A 34-week-old white male infant who had been born via normal vaginal delivery was noted to have respiratory distress, for which he was intubated. Further examination revealed a nasal obstruction, and the infant was extubated to a McGovern nipple. He also had a small ventricular septal defect without significant hemodynamic instability. An axial computed tomographic (CT) scan of the region established the diagnosis of nasal pyriform aperture stenosis (Fig. 1). He was able to breathe without an artificial airway by hospital day 6 and was discharged home 4 days later. On follow-up exam, the patient was noted as having adequate weight gain without breathing difficulties. He did not require the McGovern nipple after the first week and infrequently used topical nasal steroids. By 5 months of age, some nasal aperture growth had occurred, but stenosis remained significant enough for surgical repair to
be contemplated. At 6 months, the patient developed a central maxillary incisor and a mild midfacial hypoplasia that became more evident as hypotelorism and midface retrusion were noted 2 months later. This prompted further work-ups, including a brain magnetic resonance imaging (MRI), genetics consultation, and endocrine evaluation. The brain MRI did not reveal any midline brain dysgensis and the endocrine work-up showed intact hypothalamic–pituitary–thyroid– adrenal axis. By taking extra precautions to reduce upper respiratory infection (URI) episodes, the patient has been able to avoid significant respiratory compromise. Follow-up at 32 months of age reveals no feeding difficulties or growth delay. The patient’s height and weight were at the 40th percentile. We attribute mild obstructive symptoms during sleep to slight residual nasal vestibule narrowing as well as adenoid hypertrophy. For this reason an adenoidectomy is being contemplated, but the pyriform aperture has undergone enough growth that we no longer consider aperture enlargement surgery to be either likely or necessary.
3. Case report 2
Fig. 1. Case 1. Axial CT scan (bone window settings) at the level of the lower nasal cavity revealing bony stenosis at the pyriform aperture causing complete nasal obstruction.
An 8-week-old male infant, triplet with an estimated gestational age of 35 weeks, was referred to our office for evaluation of persistent nasal congestion existing since birth. This congestion led to difficulties with feeding and sleeping, and URI episodes that exacerbated the symptoms. His two triplet sisters bore minimal physical resemblance to the patient and had no respiratory or other medical concerns. On physical examination the patient manifested stertor with mild chest retractions. His nasal vestibular stenosis prevented passage of a 2-mm nasopharyngoscope through either nare. When he failed to improve with conservative measures including topical oxymetazoline and ranitidine, an imaging study was obtained. A non-contrast CT scan revealed the diagnosis of nasal pyriform aperture stenosis (Fig. 2). A surgical repair was offered at this time but was delayed because the father was overseas. Over the course of the next 2 months, the patient
J.J. Lee et al. / Int. J. Pediatr. Otorhinolaryngol. 60 (2001) 167–171
169
Fig. 2. Case 2. Axial CT scan demonstrating narrowing of the pyriform aperture by nasal process of the maxilla.
gradually improved until he could tolerate feeding without breathing difficulties. The anterior nasal airway was still narrow but did not cause discomfort. Surgery was never performed. The last follow-up at 1 year of age revealed an enlarged anterior nasal airway that permitted nasal breathing and a single upper maxillary incisor (Figs. 3 and 4). In addition, mild obstructive symptoms during sleep had essentially resolved; therefore, sleep study was not indicated. The patient’s height was at the 25th percentile, but both his head circumference and weight were below the 5th percentile. The patient will soon undergo further work-up to rule out other midline dysgenesis as well as endocrine and chromosomal abnormalities.
4. Discussion The pyriform aperture represents the most anterior bony opening that leads into the nasal cavity. Its boundaries include nasal bones superiorly and nasal processes of the maxilla laterally. Embryologically, nasal development occurs during the fifth to eighth gestational week. It begins with the formation of paired olfactory placodes under
which the nasal pits form. The medial nasal prominences fuse in the midline to become the primary palate. At about the same time the developing maxilla meets the lateral prominence to form the lateral nasal wall and the pyriform aperture. It is believed that overgrowth of maxillary ossification at the pyriform aperture may be responsible for the bony stenosis [2]. Clinically, congenital nasal pyriform aperture stenosis (CNPAS) presents with nasal obstruction that may cause respiratory distress in infants. In milder cases, cyclic apnea relieved by crying may be reported, as may breathing difficulty during feeding. Such findings are clinically indistinguishable from other conditions associated with nasal obstruction, such as bilateral choanal atresia or nasolacrimal duct mucoceles. The nasal airway is typically 1– 2 mm preventing the passage of the smallest 2-mm fiberoptic nasopharyngoscope. CAT scan with contiguous thin axial and coronal sections (1–3 mm) is the initial imaging study of choice. This would allow exclusion of choanal atresia. As mentioned previously, establishment of an airway is vital for those with respiratory compromise; otherwise, more conservative treatment for milder cases includes application of decongestants and nasal humidification.
170
J.J. Lee et al. / Int. J. Pediatr. Otorhinolaryngol. 60 (2001) 167–171
Fig. 3. Case 2. Axial CT scan at the level of the upper oral cavity demonstrating the single upper maxillary incisor.
Most investigators believe that surgical treatment of CNPAS is indicated when infants continue to have symptoms. An important indication for surgical intervention is nasal obstruction manifest by respiratory distress or feeding difficulties. If required, the surgical option consists of drilling out excess shelves of bone at the pyriform aperture, usually by a sublabial approach [2,3]. The experience of the two patients in this report suggests that if patients are able to tolerate conservative management, their nasal airway will improve with growth. Symptomatic improvement begins within 6 months of birth. Hui et al. has also included three non-surgically treated patients in their series with favorable outcomes [4]. They suggested that the ability to pass a No. 5 Fr catheter through the nasal cavity might predict success with conservative management. Both patients developed a central maxillary incisor, which has been reported as a possible manifestation of holoprosencephaly [5]. Furthermore, the first patient also had hypotelorism, which may be a facial anomaly associated with holoprosencephaly [6]. An MRI, however, did not reveal any midline brain dysgenesis. As there may be a genetic cause for holoprosencephaly, chromosomal analysis is also indicated with the eruption of a
single central maxillary incisor. In addition, an assessment of the hypothalamic– pituitary–thyroid–adrenal axis should be done, since an endocrine abnormality may be part of the developmental field defect [5,7,8]. The cases presented here demonstrate the feasibility of expectant management for CNPAS. With a stable airway and the absence of apnea and feeding difficulties, conservative treatment should be considered. The physician should prescribe
Fig. 4. Case 2. Facial photograph demonstrating the single upper maxillary incisor.
J.J. Lee et al. / Int. J. Pediatr. Otorhinolaryngol. 60 (2001) 167–171
careful follow-up to assess weight gain and respiratory status. Day care settings and other precipitants of URIs should be avoided. Central mega-incisors or hypotelorism should prompt further work-up to exclude midline brain dysgenesis and chromosomal or endocrine abnormalities.
[4]
[5]
[6]
References
[7]
[1] B. Douglas, The relief of vestibular nasal obstruction by partial resection of the nasal process of the superior maxilla, Plast. Reconstr. Surg. 9 (1952) 42 – 51. [2] O.E. Brown, C.M. Myer, S.C. Manning, Congenital nasal pyriform aperture stenosis, Laryngoscope 99 (1989) 86 –91. [3] H.H. Ramada, O. Ortiz, Congenital nasal pyriform aper-
[8]
.
171
ture (bony inlet) stenosis, Otolaryngol. Head Neck Surg. 113 (1995) 286 – 289. Y. Hui, J. Friedberg, W.S. Crysdale, Congenital nasal pyriform aperture stenosis as a presenting feature of holoprosencephaly, Int. J. Pediatr. Otorhinolaryngol. 31 (1995) 263 – 274. H. Arlis, R.F. Ward, Congenital nasal pyriform aperture stenosis: isolated abnormality vs. developmental field defect, Arch. Otolaryngol. Head Neck Surg. 118 (1992) 989 – 991. M.M. Cohen, Perspectives on holoprosencephaly, III: spectra, distinctions, continuities, and discontinuities, Am. J. Med. Genet. 34 (1989) 271 – 288. V.P. Johnson, Holoprosencephaly: a developmental field defect, Am. J. Med. Genet. 34 (1989) 258 – 264. M. Beregszaszi, J. Leger, C. Garel, D. Simon, M. Francois, M. Hassan, P. Czernichow, Nasal pyriform aperture stenosis and absence of the anterior pituitary gland: report of two cases, J. Pediatr. 128 (1996) 858 – 861.
Case report
Congenital nasal pyriform aperture stenosis: non-surgical management and long-term analysis James J. Lee a,*, John P. Bent b, Robert F. Ward c a
Department of Otolaryngology, New York Uni6ersity School of Medicine, 550 First A6enue, New York, NY 10016, USA b Department of Otolaryngology, Albert Einstein School of Medicine, New York Otolaryngology Institute, New York, NY 10021, USA c Department of Otolaryngology, Cornell Uni6ersity Medical Center, New York Otolaryngology Institute, New York, NY, USA Received 28 November 2000; received in revised form 23 April 2001; accepted 24 April 2001
Abstract Congenital nasal pyriform aperture stenosis is a rare cause of airway obstruction in the newborn. Immediate recognition and therapy are essential for this potentially life-threatening condition. After initial management, which includes establishment of an oral airway, surgical repair of the stenotic bony inlet has been traditionally considered. We present a long-term follow-up of two patients with congenital pyriform aperture stenosis, who were managed expectantly. Pertinent embryology, clinical presentation, and general treatment strategies for these patients are also discussed. © 2001 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Maxillofacial abnormalities; Nasal cavity; Nasal obstruction; Nasal pyriform aperture stenosis
1. Introduction Nasal pyriform aperture stenosis has been recognized for many years [1]. However, congenital nasal pyriform aperture stenosis (CNPAS) has only been recently described as a cause of respiratory compromise in infants [2]. Neonates are obligate nasal breathers and therefore may present with various signs and symptoms related to respiratory compromise. * Corresponding author. Tel.: + 1-212-2636344; fax: + 1212-2638257. E-mail address: [email protected] (J.J. Lee).
Initial management of these infants includes establishment of an airway, usually with an oral airway or a McGovern nipple. When patients are able to breathe without an artificial airway and demonstrate adequate oral intake, they are discharged home with nasal decongestants and humidification. Surgical widening of the pyriform aperture stenosis is usually recommended to these infants, as many of them are expected to have difficulties with nasal congestion, feeding, and weight gain. Since the data on the long-term outcome of non-surgically managed patients with pyriform
0165-5876/01/$ - see front matter © 2001 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 1 ) 0 0 5 0 3 - 1
168
J.J. Lee et al. / Int. J. Pediatr. Otorhinolaryngol. 60 (2001) 167–171
aperture stenosis is limited, this paper will present the long-term follow-up of two expectantly managed patients.
2. Case report 1 A 34-week-old white male infant who had been born via normal vaginal delivery was noted to have respiratory distress, for which he was intubated. Further examination revealed a nasal obstruction, and the infant was extubated to a McGovern nipple. He also had a small ventricular septal defect without significant hemodynamic instability. An axial computed tomographic (CT) scan of the region established the diagnosis of nasal pyriform aperture stenosis (Fig. 1). He was able to breathe without an artificial airway by hospital day 6 and was discharged home 4 days later. On follow-up exam, the patient was noted as having adequate weight gain without breathing difficulties. He did not require the McGovern nipple after the first week and infrequently used topical nasal steroids. By 5 months of age, some nasal aperture growth had occurred, but stenosis remained significant enough for surgical repair to
be contemplated. At 6 months, the patient developed a central maxillary incisor and a mild midfacial hypoplasia that became more evident as hypotelorism and midface retrusion were noted 2 months later. This prompted further work-ups, including a brain magnetic resonance imaging (MRI), genetics consultation, and endocrine evaluation. The brain MRI did not reveal any midline brain dysgensis and the endocrine work-up showed intact hypothalamic–pituitary–thyroid– adrenal axis. By taking extra precautions to reduce upper respiratory infection (URI) episodes, the patient has been able to avoid significant respiratory compromise. Follow-up at 32 months of age reveals no feeding difficulties or growth delay. The patient’s height and weight were at the 40th percentile. We attribute mild obstructive symptoms during sleep to slight residual nasal vestibule narrowing as well as adenoid hypertrophy. For this reason an adenoidectomy is being contemplated, but the pyriform aperture has undergone enough growth that we no longer consider aperture enlargement surgery to be either likely or necessary.
3. Case report 2
Fig. 1. Case 1. Axial CT scan (bone window settings) at the level of the lower nasal cavity revealing bony stenosis at the pyriform aperture causing complete nasal obstruction.
An 8-week-old male infant, triplet with an estimated gestational age of 35 weeks, was referred to our office for evaluation of persistent nasal congestion existing since birth. This congestion led to difficulties with feeding and sleeping, and URI episodes that exacerbated the symptoms. His two triplet sisters bore minimal physical resemblance to the patient and had no respiratory or other medical concerns. On physical examination the patient manifested stertor with mild chest retractions. His nasal vestibular stenosis prevented passage of a 2-mm nasopharyngoscope through either nare. When he failed to improve with conservative measures including topical oxymetazoline and ranitidine, an imaging study was obtained. A non-contrast CT scan revealed the diagnosis of nasal pyriform aperture stenosis (Fig. 2). A surgical repair was offered at this time but was delayed because the father was overseas. Over the course of the next 2 months, the patient
J.J. Lee et al. / Int. J. Pediatr. Otorhinolaryngol. 60 (2001) 167–171
169
Fig. 2. Case 2. Axial CT scan demonstrating narrowing of the pyriform aperture by nasal process of the maxilla.
gradually improved until he could tolerate feeding without breathing difficulties. The anterior nasal airway was still narrow but did not cause discomfort. Surgery was never performed. The last follow-up at 1 year of age revealed an enlarged anterior nasal airway that permitted nasal breathing and a single upper maxillary incisor (Figs. 3 and 4). In addition, mild obstructive symptoms during sleep had essentially resolved; therefore, sleep study was not indicated. The patient’s height was at the 25th percentile, but both his head circumference and weight were below the 5th percentile. The patient will soon undergo further work-up to rule out other midline dysgenesis as well as endocrine and chromosomal abnormalities.
4. Discussion The pyriform aperture represents the most anterior bony opening that leads into the nasal cavity. Its boundaries include nasal bones superiorly and nasal processes of the maxilla laterally. Embryologically, nasal development occurs during the fifth to eighth gestational week. It begins with the formation of paired olfactory placodes under
which the nasal pits form. The medial nasal prominences fuse in the midline to become the primary palate. At about the same time the developing maxilla meets the lateral prominence to form the lateral nasal wall and the pyriform aperture. It is believed that overgrowth of maxillary ossification at the pyriform aperture may be responsible for the bony stenosis [2]. Clinically, congenital nasal pyriform aperture stenosis (CNPAS) presents with nasal obstruction that may cause respiratory distress in infants. In milder cases, cyclic apnea relieved by crying may be reported, as may breathing difficulty during feeding. Such findings are clinically indistinguishable from other conditions associated with nasal obstruction, such as bilateral choanal atresia or nasolacrimal duct mucoceles. The nasal airway is typically 1– 2 mm preventing the passage of the smallest 2-mm fiberoptic nasopharyngoscope. CAT scan with contiguous thin axial and coronal sections (1–3 mm) is the initial imaging study of choice. This would allow exclusion of choanal atresia. As mentioned previously, establishment of an airway is vital for those with respiratory compromise; otherwise, more conservative treatment for milder cases includes application of decongestants and nasal humidification.
170
J.J. Lee et al. / Int. J. Pediatr. Otorhinolaryngol. 60 (2001) 167–171
Fig. 3. Case 2. Axial CT scan at the level of the upper oral cavity demonstrating the single upper maxillary incisor.
Most investigators believe that surgical treatment of CNPAS is indicated when infants continue to have symptoms. An important indication for surgical intervention is nasal obstruction manifest by respiratory distress or feeding difficulties. If required, the surgical option consists of drilling out excess shelves of bone at the pyriform aperture, usually by a sublabial approach [2,3]. The experience of the two patients in this report suggests that if patients are able to tolerate conservative management, their nasal airway will improve with growth. Symptomatic improvement begins within 6 months of birth. Hui et al. has also included three non-surgically treated patients in their series with favorable outcomes [4]. They suggested that the ability to pass a No. 5 Fr catheter through the nasal cavity might predict success with conservative management. Both patients developed a central maxillary incisor, which has been reported as a possible manifestation of holoprosencephaly [5]. Furthermore, the first patient also had hypotelorism, which may be a facial anomaly associated with holoprosencephaly [6]. An MRI, however, did not reveal any midline brain dysgenesis. As there may be a genetic cause for holoprosencephaly, chromosomal analysis is also indicated with the eruption of a
single central maxillary incisor. In addition, an assessment of the hypothalamic– pituitary–thyroid–adrenal axis should be done, since an endocrine abnormality may be part of the developmental field defect [5,7,8]. The cases presented here demonstrate the feasibility of expectant management for CNPAS. With a stable airway and the absence of apnea and feeding difficulties, conservative treatment should be considered. The physician should prescribe
Fig. 4. Case 2. Facial photograph demonstrating the single upper maxillary incisor.
J.J. Lee et al. / Int. J. Pediatr. Otorhinolaryngol. 60 (2001) 167–171
careful follow-up to assess weight gain and respiratory status. Day care settings and other precipitants of URIs should be avoided. Central mega-incisors or hypotelorism should prompt further work-up to exclude midline brain dysgenesis and chromosomal or endocrine abnormalities.
[4]
[5]
[6]
References
[7]
[1] B. Douglas, The relief of vestibular nasal obstruction by partial resection of the nasal process of the superior maxilla, Plast. Reconstr. Surg. 9 (1952) 42 – 51. [2] O.E. Brown, C.M. Myer, S.C. Manning, Congenital nasal pyriform aperture stenosis, Laryngoscope 99 (1989) 86 –91. [3] H.H. Ramada, O. Ortiz, Congenital nasal pyriform aper-
[8]
.
171
ture (bony inlet) stenosis, Otolaryngol. Head Neck Surg. 113 (1995) 286 – 289. Y. Hui, J. Friedberg, W.S. Crysdale, Congenital nasal pyriform aperture stenosis as a presenting feature of holoprosencephaly, Int. J. Pediatr. Otorhinolaryngol. 31 (1995) 263 – 274. H. Arlis, R.F. Ward, Congenital nasal pyriform aperture stenosis: isolated abnormality vs. developmental field defect, Arch. Otolaryngol. Head Neck Surg. 118 (1992) 989 – 991. M.M. Cohen, Perspectives on holoprosencephaly, III: spectra, distinctions, continuities, and discontinuities, Am. J. Med. Genet. 34 (1989) 271 – 288. V.P. Johnson, Holoprosencephaly: a developmental field defect, Am. J. Med. Genet. 34 (1989) 258 – 264. M. Beregszaszi, J. Leger, C. Garel, D. Simon, M. Francois, M. Hassan, P. Czernichow, Nasal pyriform aperture stenosis and absence of the anterior pituitary gland: report of two cases, J. Pediatr. 128 (1996) 858 – 861.