- Email: [email protected]
Neonatal respiratory distress secondary to bilateral intranasal dacryocystocoeles
International Journal of Pediatric Otorhinolaryngology (2008) 72, 1873—1877
www.elsevier.com/locate/ijporl
CASE REPORT
Neonatal respiratory distress secondary to bilateral intranasal dacryocystocoeles David S. Leonard a,*, Michael O’Keefe b, Helena Rowley c, Joseph P. Hughes a a
Department of Otolaryngology, Midwestern Regional Hospital, Dooradoyle, Limerick, Ireland Department of Ophthalmology, Children’s University Hospital Temple Street, Dublin 1, Ireland c Department of Otolaryngology, Children’s University Hospital Temple Street, Dublin 1, Ireland b
Received 17 June 2008; received in revised form 23 August 2008; accepted 2 September 2008
KEYWORDS Neonatal; Respiratory distress; Nasal obstruction; Dacryocystocoele
Summary Obstruction of the nasolacrimal duct is a common congenital abnormality reported in up to 84% of neonatal patients [J. Yohendran, A.C. Wignall, E.J. Beckenham, Bilateral congenital dacryocystocoeles with concurrent intranasal mucocoeles causing respiratory distress in a neonate, Asian J. Surg. 29 (2) (2006) 109—111; M.J. Cunningham, J.J. Woog, Endonasal endoscopic dacryocystorhinostomy in children, Arch. Otolaryngol. Head Neck Surg. 124 (1998) 328—333; D. Guery, E.L. Kendig, Congenital impotency of the nasolacrimal duct, Arch. Ophthalmol. 97 (1979) 1656— 1658]. Rarely, obstruction results in the development of an intranasal lacrimal duct cyst, or dacryocystocoele, which arises inferolateral to the inferior turbinate [H.R. Jin, S.O. Shin, Endoscopic marsupialisation of bilateral lacrimal sac mucoceles with nasolacrimal duct cysts, Auris Nasus Larynx 26 (1999) 441—445]. These lesions can cause nasal obstruction and, when bilateral, significant respiratory compromise. We present the case of a 3-day-old infant with bilateral intranasal lacrimal duct cysts causing nasal obstruction and intermittent respiratory compromise. The diagnosis was suspected on clinical examination and confirmed on MRI. The patient was successfully managed by bilateral endoscopic marsupialisation and probing of the nasolacrimal ducts. We also present a review of the literature surrounding investigation and management of intranasal lacrimal duct cysts. # 2008 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Obstruction of the nasolacrimal duct is a common congenital abnormality reported to occur in approximately 30% of neonates [1], but with rates as low as 6% and as high as 84% have been reported * Corresponding author. E-mail address: [email protected] (D.S. Leonard).
[2,3]. The duct is most often obstructed distally, at the valve of Hasner [4], where the duct enters the nasal cavity lateral to the inferior turbinate. In most cases (94—98%) the obstruction is asymptomatic [5,6] and resolves spontaneously within the first year of life [7]. In cases where coexistent proximal obstruction exists at the valve of Rosenmuller, where the common canaliculus enters the nasolacrimal sac, a cystic swelling results, a nasolacrimal
0165-5876/$ — see front matter # 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2008.09.011
1874
D.S. Leonard et al.
duct cyst or dacryocystocoele [8,9]. These typically present as a facial swelling inferior to the medial canthus of the eye with associated epiphora [8,14]. Management is by gentle massage, probing or less commonly silastic stenting. Nasolacrimal duct cysts may also expand inferiorly, herniating into the nasal cavity and forming a cystic intranasal swelling, which arises from the inferior meatus and displaces the inferior turbinate medially and superiorly. Unilateral cysts may present as feeding difficulties and/or sleep disturbance in older neonates while bilateral cysts present in the early neonatal period with intermittent respiratory distress and episodes of desaturation [10]. We present a case of bilateral dacryocystocoele with intranasal extension presenting as respiratory distress and episodic desaturation in a 3-day-old male infant. The diagnosis was suspected clinically and confirmed by MRI scan. The patient was successfully managed by bilateral endoscopic marsupialisation of the intranasal cysts, and blunt probing of both nasolacrimal ducts.
2. Case history A 3-day-old male infant, born by elective caesarian section at 38 weeks, was referred from a peripheral obstetric unit to our tertiary airway clinic with a history of grunting, difficulty in feeding and five cyanotic episodes with associated bradycardia. Immediate post-natal course was uncomplicated with apgar scores of 8 at both 1 and 5 min. Prior to transfer flexible nasal suction had been successfully passed through both nostrils into the nasopharynx, ruling out choanal atresia. At presentation, following ambulance transfer in an incubator, the infant was sleeping comfortably with oxygen saturations above 97%. He was grossly phenotypically normal. On initial examination, a swelling was noted at the medial canthus of the right eye, and a thick discharge from the left medial canthus. Nasal airflow, as assessed by simple mirror condensation, was reduced bilaterally, but more so on than left than the right. Anterior rhinoscopy revealed pale masses arising from the inferior meatus bilaterally, lateral and inferior to the inferior turbinates (Fig. 1). They were boggy on palpation and apparently cystic. A presumptive diagnosis of a bilateral nasolacrimal duct abnormality was made. Nasendoscopy confirmed that the swellings were confined to the anterior nasal fossa, that they were laterally based and that both posterior choana were patent. The post-nasal space was also normal. Flexible laryngoscopy was performed as part of a full upper airway examination revealing a normal
Fig. 1 Endoscopic view of dacryocystocoeles arising from the inferior meatus bilaterally, lateral and inferior to the inferior turbinates.
supraglottis and glottis, with normal vocal cord movements. Following full examination an MRI was performed. This revealed dilated nasolacrimal ducts bilaterally with cystic swellings at the distal ends (Fig. 2). No other abnormality was seen. The diagnosis of bilateral dacryocystocoeles with intranasal lacrimal duct cysts was made. Following the MRI scan, the infant was taken to operating room and bilateral marsupialisation and probing of the nasolacrimal ducts was performed as a combined otolaryngology/ophthalmology procedure. Following induction of general anaesthesia, the nasal mucosa was decompressed using deoxymetazoline-soaked pledgets. Both nasolacrimal ducts were gently probed from above and transmitted movements were observed in the intranasal cysts. Under endoscopic visualisation with a 2.7 mm, 0degree nasal endoscope the cystic swellings were grasped, adjacent to the lateral nasal wall, and incised using a straight trucut forceps, releasing a
Neonatal respiratory distress
1875 and flushed with sterile saline until both flowed freely. Specimens of the mucoid secretion and the resected mucosa were sent from both nostrils. Histology revealed ciliated, pseudostratified columnar respiratory epithelium and a fibrin-rich cyst fluid containing numerous inflammatory cells. Post-operatively, the patient enjoyed immediate improvement in respiratory function and made an uneventful recovery. No further episodes of respiratory distress were reported. At 9 months of age he had a normal follow-up nasal endoscopy and was discharged from our care.
3. Discussion Infants are obligate nasal breathers for the first 2— 6 months of life. During that time nasal obstruction can cause significant respiratory embarrassment. Although unilateral obstruction may be asymptomatic, or present with feeding difficulties and sleeping disturbance in older infants, bilateral obstruction typically presents in the neonatal period with respiratory distress and intermittent desaturation events. Differential diagnosis includes a variety of congenital malformations, inflammatory conditions, tumours and hamartomas (Table 1). Although congenital nasolacrimal duct obstruction is common, intranasal extension represents a rare congenital abnormality that can be either unilateral or bilateral, and can, therefore, present with airway embarrassment of varying severity. The nasolacrimal duct originates as a cord of ectodermal tissue between the medial aspect of the maxillary process and the lateral aspect of the frontonasal process. Canalisation begins at approxiTable 1 Differential diagnosis of upper airway respiratory distress secondary to nasal pathologya. Congenital Choanal atresia Meningo-encephalocoele Dermoid cyst Thornwald’s cyst Haemangioma Pyriform aperture stenosis
Fig. 2 Sequential MRI images demonstrating dilated lacrimal ducts bilaterally terminating in dilated intranasal mucocoeles or dacryocystocoeles.
thick mucoid secretion. The cyst was excised and the residual cuff of mucosa trimmed, revealing patent nasolacrimal duct orifices bilaterally. Both were then gently probed again from above, confirming patency,
Neoplastic processes Glioma Craniopharyngioma Trauma Infective Dacryocystitis Syphilis Oedema
1876 mately 3 months gestation, extending both cranially and caudally, and is complete by 6 months. Incomplete canalisation occurs in approximately 30% [1,8,10] of term infants, however rates as low as 6% and as high as 84% have been reported [2,3]. Distal obstruction occurs at the valve of Hasner, the distal opening of the duct beneath the inferior turbinate. The valve is normally ruptured by neonatal respiratory efforts at birth [11]. Obstruction can also occur proximally, at the valve of Rosenmuller, the junction between the common canaliculus and the lacrimal sac, or at both sites. The majority of patients with nasolacrimal duct obstruction (94—98%) are asymptomatic [5,7]. Cases of either proximal or distal obstruction may present with epiphora, which is generally self-resolving. Obstruction both proximally and distally results in the formation of a dacryocystocoele, the majority of which present as a unilateral fluctuant mass at the medial canthus, often with blue discoloration and associated epiphora [12,13]. Some authors believe that the valve of Rosenmuller may not have to be completely obstructed, but may behave as a unidirectional valve, preventing the escape of secretions collected against an imperforate valve of Hasner inferiorly [5,11]. Approximately 14% of congenital dacryocystocoeles are bilateral [8,14]. Although there is no published incidence, a small minority extend into the nasal cavity, presenting as cystic swellings arising from below the inferior turbinate. To date less than 100 cases have been reported in the English language literature. Bilateral intranasal extension, as seen in our patient is even less common, with fewer than 25 reported cases. Intranasal lacrimal duct cysts present with varying degrees of respiratory compromise. Patients with unilateral cysts may present with feeding difficulties, sleep disturbance or intermittent acute respiratory distress due to vascular congestion of the contralateral side associated with the physiological nasal cycle. Presentation is generally in the post-neonatal period, as the normal contralateral nasal airway is sufficient for respiration during the neonatal period. Bilateral cysts present early in the neonatal period with respiratory distress and intermittent episodes of desaturation [18]. The severity of obstruction is variable. While our patient only suffered from intermittent episodes of desaturation with bradycardia and cynaosis, the most severe cases may require emergency intubation for airway support [11]. In the stable patient a full ENT examination should be performed at a tertiary airway clinic. Investigation should include a detailed examination
D.S. Leonard et al. of the external nasal anatomy and anterior rhinoscopy, nasendoscopy and radiological investigations. Prior to more invasive investigations is essential to determine that the anterior nasal anatomy is normal: that both nostrils and pyriform apertures are patent and of normal diameter, and that the nasal septum is approximately central and not obstructing either nostrils. On nasendoscopy the anatomy, relations and nature of an intranasal mass or posterior obstruction can be defined. Specifically, an intranasal lacrimal duct cyst can be differentiated from other congenital intranasal masses: it is possible to pass a nasendoscope medial to a nasolacrimal duct cyst but not to either an intranasal encephalocoele or meningocoele [11,15]. Recent advances in ultrasonography have allowed prenatal diagnosis of nasolacrimal duct cysts at 30 weeks gestation, over half of which resolve spontaneously prior to delivery [16]. Post-natally, computerised tomography (CT) has been the radiologic investigation of choice for nasolacrimal duct abnormalities due to the excellent bony definition [17]; however, we feel that in the neonatal population use of MRI confers several advantages. The enhanced soft tissue definition provided by MRI scanning facilitates differentiation of the intranasal lacrimal duct cyst from surrounding soft tissue structures, rules out other congenital soft tissue abnormalities and excludes intracranial communication or the involvement of neural tissue in the intranasal mass. Simple dacryostocoeles can be managed conservatively with massage and warm compresses. Intranasal lacrimal duct cysts are best managed surgically [5]. Three surgical options have been reported: (1) Blind probing of the nasolacrimal duct [18]. (2) Nasendoscopy and marsupialisation of the intranasal cyst [11]. (3) Combined endoscopic marsupialisation and nasolacrimal duct probing [19,20]. Probing the nasolacrimal duct disrupts the valve of Rosenmuller and allows proximal decompression of the nasolacrimal apparatus. Endoscopic marsupialisation decompresses the system distally, allowing drainage of retained mucopurulent secretions. Combined probing and marsupialisation, as performed in our patient, re-establishes continuity of the nasolacrimal system, facilitating a return to normal, physiological function
4. Conclusion Nasal obstruction causes respiratory distress and compromise in neonatal patients. Causes include
Neonatal respiratory distress
1877
inflammatory conditions, tumours and hamartomas and congenital malformations. Nasolacrimal duct cysts with intranasal extension represent a rare congenital cause of neonatal respiratory distress. These rare congenital abnormalities are caused by obstruction of the nasolacrimal duct at both the valves of Hasner and of Rosenmuller. They can be either unilateral or bilateral, and mode of presentation is determined by the severity of the nasal obstruction. Acute airway stabilisation may be necessary prior to investigation by anterior rhinoscopy, nasendoscopy and either CT or MRI. Definitive management is surgical. Our preferred technique is endoscopic marsupialisation in conjunction with probing and irrigation of the lacrimal ducts to ensure patency of the nasolacrimal apparatus.
References [1] J. Yohendran, A.C. Wignall, E.J. Beckenham, Bilateral congenital dacryocystocoeles with concurrent intranasal mucocoeles causing respiratory distress in a neonate, Asian J. Surg. 29 (2) (2006) 109—111. [2] M.J. Cunningham, J.J. Woog, Endonasal endoscopic dacryocystorhinostomy in children, Arch. Otolaryngol. Head Neck Surg. 124 (1998) 328—333. [3] D. Guery, E.L. Kendig, Congenital impotency of the nasolacrimal duct, Arch. Ophthalmol. 97 (1979) 1656—1658. [4] R.G. Shashy, V. Durairaj, J.M. Holmes, G.G. Hohberger, D.M. Thompson, J.L. Kasperbauer, Congenital dacryocystocele associated with intranasal cysts: diagnosis and management, Laryngoscope 113 (2003) 37—40. [5] H.R. Jin, S.O. Shin, Endoscopic marsupialisation of bilateral lacrimal sac mucoceles with nasolacrimal duct cysts, Auris Nasus Larynx 26 (1999) 441—445.
[6] E.A. Paysse, D.K. Coats, J.M. Bernstein, et al., Management and complications of congenital dacryocele with concurrent intranasal mucocele, JAAPOS 4 (2000) 46—53. [7] R.A. Peterson, R.M. Robb, The natural course of congenital obstruction of the nasolacrimal duct, J. Pediatr. Ophthalmol. Strabismus 15 (1978) 246—250. [8] R.D. Devine, R.L. Anderson, R.M. Bumstead, Bilateral congenital lacrimal sac mucoceles with nasal extension and drainage, Arch. Ophthalmol. 101 (1983) 246—248. [9] J.R. Boynton, D. Drucker, Distension of the lacimal sac in neonates, Ophthalmic Surg. 20 (1989) 103—107. [10] J.C. Edmond, R.V. Keech, Congenital lacrimal sac mucocoele associated with respiratory distress, J. Pediatr. Ophthalmol. Strabismus 28 (1991) 287—289. [11] D. Roy, N. Guevara, J. Santini, L. Castillo, Endoscopic marsupialization of congenital nasolacrimal duct cyst with dacryocele, Clin. Otolaryngol. 27 (2002) 167—170. [12] A.B. Silva, A. Hotaling, W. Rasian, Pathologic quiz case #2–— nasolacrimal duct cyst, Arch. Otolaryngol. Head Neck Surg. 120 (1994) 888—892. [13] K.M. Hepler, G.E. Woodson, D.B. Keams, Respiratory distress on the neonate: sequelae of a congenital dacryocytocele, Arch. Otolaryngol. Head Neck Surg. 121 (1995) 1423—1425. [14] A.M. Mansour, K.P. Cheng, J.V. Mumma, et al., Congenital dacryocele. A collaborative review, Ophthalmology 98 (1991) 1744—1751. [15] B.F. Jaffe, Classification and management of anomalies of the nose, Otolaryngol. Clin. North Am. 14 (1981) 989—1004. [16] W. Supulveda, A.B. Wojakowski, D. Elias, et al., Congenital dacryocystocele: prenatal 2- and 3-dimensional sonographic findings, J. Ultrasound Med. 24 (2) (2005) 225—230. [17] P.K. Rand, W.S. Ball, D.R. Kulwin, Congenital nasolacrimal mucoceles: CTevaluation, Radiology 173 (3) (1989) 691—694. [18] S.W. Yee, R.W. Seibert, C.M. Bower, C.M. Glasier, Congenital nasolacrimal duct mucocoele: a cause of respiratory distress, Int. J. Pediatr. Otorhinol. 29 (1994) 151—158. [19] T.R. Grin, J.S. Mertz, M. Stass-Isern, Congenital nasolacrimal duct cysts in dacryocystocele, Ophthalmology 98 (1991) 1238—1242. [20] G.T. Lueder, Neonatal dacryocystitis associated with nasolacrimal duct cysts, J. Pediatr. Ophthalmol. Strabismus 32 (1995) 102—106.
Available online at www.sciencedirect.com
www.elsevier.com/locate/ijporl
CASE REPORT
Neonatal respiratory distress secondary to bilateral intranasal dacryocystocoeles David S. Leonard a,*, Michael O’Keefe b, Helena Rowley c, Joseph P. Hughes a a
Department of Otolaryngology, Midwestern Regional Hospital, Dooradoyle, Limerick, Ireland Department of Ophthalmology, Children’s University Hospital Temple Street, Dublin 1, Ireland c Department of Otolaryngology, Children’s University Hospital Temple Street, Dublin 1, Ireland b
Received 17 June 2008; received in revised form 23 August 2008; accepted 2 September 2008
KEYWORDS Neonatal; Respiratory distress; Nasal obstruction; Dacryocystocoele
Summary Obstruction of the nasolacrimal duct is a common congenital abnormality reported in up to 84% of neonatal patients [J. Yohendran, A.C. Wignall, E.J. Beckenham, Bilateral congenital dacryocystocoeles with concurrent intranasal mucocoeles causing respiratory distress in a neonate, Asian J. Surg. 29 (2) (2006) 109—111; M.J. Cunningham, J.J. Woog, Endonasal endoscopic dacryocystorhinostomy in children, Arch. Otolaryngol. Head Neck Surg. 124 (1998) 328—333; D. Guery, E.L. Kendig, Congenital impotency of the nasolacrimal duct, Arch. Ophthalmol. 97 (1979) 1656— 1658]. Rarely, obstruction results in the development of an intranasal lacrimal duct cyst, or dacryocystocoele, which arises inferolateral to the inferior turbinate [H.R. Jin, S.O. Shin, Endoscopic marsupialisation of bilateral lacrimal sac mucoceles with nasolacrimal duct cysts, Auris Nasus Larynx 26 (1999) 441—445]. These lesions can cause nasal obstruction and, when bilateral, significant respiratory compromise. We present the case of a 3-day-old infant with bilateral intranasal lacrimal duct cysts causing nasal obstruction and intermittent respiratory compromise. The diagnosis was suspected on clinical examination and confirmed on MRI. The patient was successfully managed by bilateral endoscopic marsupialisation and probing of the nasolacrimal ducts. We also present a review of the literature surrounding investigation and management of intranasal lacrimal duct cysts. # 2008 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Obstruction of the nasolacrimal duct is a common congenital abnormality reported to occur in approximately 30% of neonates [1], but with rates as low as 6% and as high as 84% have been reported * Corresponding author. E-mail address: [email protected] (D.S. Leonard).
[2,3]. The duct is most often obstructed distally, at the valve of Hasner [4], where the duct enters the nasal cavity lateral to the inferior turbinate. In most cases (94—98%) the obstruction is asymptomatic [5,6] and resolves spontaneously within the first year of life [7]. In cases where coexistent proximal obstruction exists at the valve of Rosenmuller, where the common canaliculus enters the nasolacrimal sac, a cystic swelling results, a nasolacrimal
0165-5876/$ — see front matter # 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2008.09.011
1874
D.S. Leonard et al.
duct cyst or dacryocystocoele [8,9]. These typically present as a facial swelling inferior to the medial canthus of the eye with associated epiphora [8,14]. Management is by gentle massage, probing or less commonly silastic stenting. Nasolacrimal duct cysts may also expand inferiorly, herniating into the nasal cavity and forming a cystic intranasal swelling, which arises from the inferior meatus and displaces the inferior turbinate medially and superiorly. Unilateral cysts may present as feeding difficulties and/or sleep disturbance in older neonates while bilateral cysts present in the early neonatal period with intermittent respiratory distress and episodes of desaturation [10]. We present a case of bilateral dacryocystocoele with intranasal extension presenting as respiratory distress and episodic desaturation in a 3-day-old male infant. The diagnosis was suspected clinically and confirmed by MRI scan. The patient was successfully managed by bilateral endoscopic marsupialisation of the intranasal cysts, and blunt probing of both nasolacrimal ducts.
2. Case history A 3-day-old male infant, born by elective caesarian section at 38 weeks, was referred from a peripheral obstetric unit to our tertiary airway clinic with a history of grunting, difficulty in feeding and five cyanotic episodes with associated bradycardia. Immediate post-natal course was uncomplicated with apgar scores of 8 at both 1 and 5 min. Prior to transfer flexible nasal suction had been successfully passed through both nostrils into the nasopharynx, ruling out choanal atresia. At presentation, following ambulance transfer in an incubator, the infant was sleeping comfortably with oxygen saturations above 97%. He was grossly phenotypically normal. On initial examination, a swelling was noted at the medial canthus of the right eye, and a thick discharge from the left medial canthus. Nasal airflow, as assessed by simple mirror condensation, was reduced bilaterally, but more so on than left than the right. Anterior rhinoscopy revealed pale masses arising from the inferior meatus bilaterally, lateral and inferior to the inferior turbinates (Fig. 1). They were boggy on palpation and apparently cystic. A presumptive diagnosis of a bilateral nasolacrimal duct abnormality was made. Nasendoscopy confirmed that the swellings were confined to the anterior nasal fossa, that they were laterally based and that both posterior choana were patent. The post-nasal space was also normal. Flexible laryngoscopy was performed as part of a full upper airway examination revealing a normal
Fig. 1 Endoscopic view of dacryocystocoeles arising from the inferior meatus bilaterally, lateral and inferior to the inferior turbinates.
supraglottis and glottis, with normal vocal cord movements. Following full examination an MRI was performed. This revealed dilated nasolacrimal ducts bilaterally with cystic swellings at the distal ends (Fig. 2). No other abnormality was seen. The diagnosis of bilateral dacryocystocoeles with intranasal lacrimal duct cysts was made. Following the MRI scan, the infant was taken to operating room and bilateral marsupialisation and probing of the nasolacrimal ducts was performed as a combined otolaryngology/ophthalmology procedure. Following induction of general anaesthesia, the nasal mucosa was decompressed using deoxymetazoline-soaked pledgets. Both nasolacrimal ducts were gently probed from above and transmitted movements were observed in the intranasal cysts. Under endoscopic visualisation with a 2.7 mm, 0degree nasal endoscope the cystic swellings were grasped, adjacent to the lateral nasal wall, and incised using a straight trucut forceps, releasing a
Neonatal respiratory distress
1875 and flushed with sterile saline until both flowed freely. Specimens of the mucoid secretion and the resected mucosa were sent from both nostrils. Histology revealed ciliated, pseudostratified columnar respiratory epithelium and a fibrin-rich cyst fluid containing numerous inflammatory cells. Post-operatively, the patient enjoyed immediate improvement in respiratory function and made an uneventful recovery. No further episodes of respiratory distress were reported. At 9 months of age he had a normal follow-up nasal endoscopy and was discharged from our care.
3. Discussion Infants are obligate nasal breathers for the first 2— 6 months of life. During that time nasal obstruction can cause significant respiratory embarrassment. Although unilateral obstruction may be asymptomatic, or present with feeding difficulties and sleeping disturbance in older infants, bilateral obstruction typically presents in the neonatal period with respiratory distress and intermittent desaturation events. Differential diagnosis includes a variety of congenital malformations, inflammatory conditions, tumours and hamartomas (Table 1). Although congenital nasolacrimal duct obstruction is common, intranasal extension represents a rare congenital abnormality that can be either unilateral or bilateral, and can, therefore, present with airway embarrassment of varying severity. The nasolacrimal duct originates as a cord of ectodermal tissue between the medial aspect of the maxillary process and the lateral aspect of the frontonasal process. Canalisation begins at approxiTable 1 Differential diagnosis of upper airway respiratory distress secondary to nasal pathologya. Congenital Choanal atresia Meningo-encephalocoele Dermoid cyst Thornwald’s cyst Haemangioma Pyriform aperture stenosis
Fig. 2 Sequential MRI images demonstrating dilated lacrimal ducts bilaterally terminating in dilated intranasal mucocoeles or dacryocystocoeles.
thick mucoid secretion. The cyst was excised and the residual cuff of mucosa trimmed, revealing patent nasolacrimal duct orifices bilaterally. Both were then gently probed again from above, confirming patency,
Neoplastic processes Glioma Craniopharyngioma Trauma Infective Dacryocystitis Syphilis Oedema
1876 mately 3 months gestation, extending both cranially and caudally, and is complete by 6 months. Incomplete canalisation occurs in approximately 30% [1,8,10] of term infants, however rates as low as 6% and as high as 84% have been reported [2,3]. Distal obstruction occurs at the valve of Hasner, the distal opening of the duct beneath the inferior turbinate. The valve is normally ruptured by neonatal respiratory efforts at birth [11]. Obstruction can also occur proximally, at the valve of Rosenmuller, the junction between the common canaliculus and the lacrimal sac, or at both sites. The majority of patients with nasolacrimal duct obstruction (94—98%) are asymptomatic [5,7]. Cases of either proximal or distal obstruction may present with epiphora, which is generally self-resolving. Obstruction both proximally and distally results in the formation of a dacryocystocoele, the majority of which present as a unilateral fluctuant mass at the medial canthus, often with blue discoloration and associated epiphora [12,13]. Some authors believe that the valve of Rosenmuller may not have to be completely obstructed, but may behave as a unidirectional valve, preventing the escape of secretions collected against an imperforate valve of Hasner inferiorly [5,11]. Approximately 14% of congenital dacryocystocoeles are bilateral [8,14]. Although there is no published incidence, a small minority extend into the nasal cavity, presenting as cystic swellings arising from below the inferior turbinate. To date less than 100 cases have been reported in the English language literature. Bilateral intranasal extension, as seen in our patient is even less common, with fewer than 25 reported cases. Intranasal lacrimal duct cysts present with varying degrees of respiratory compromise. Patients with unilateral cysts may present with feeding difficulties, sleep disturbance or intermittent acute respiratory distress due to vascular congestion of the contralateral side associated with the physiological nasal cycle. Presentation is generally in the post-neonatal period, as the normal contralateral nasal airway is sufficient for respiration during the neonatal period. Bilateral cysts present early in the neonatal period with respiratory distress and intermittent episodes of desaturation [18]. The severity of obstruction is variable. While our patient only suffered from intermittent episodes of desaturation with bradycardia and cynaosis, the most severe cases may require emergency intubation for airway support [11]. In the stable patient a full ENT examination should be performed at a tertiary airway clinic. Investigation should include a detailed examination
D.S. Leonard et al. of the external nasal anatomy and anterior rhinoscopy, nasendoscopy and radiological investigations. Prior to more invasive investigations is essential to determine that the anterior nasal anatomy is normal: that both nostrils and pyriform apertures are patent and of normal diameter, and that the nasal septum is approximately central and not obstructing either nostrils. On nasendoscopy the anatomy, relations and nature of an intranasal mass or posterior obstruction can be defined. Specifically, an intranasal lacrimal duct cyst can be differentiated from other congenital intranasal masses: it is possible to pass a nasendoscope medial to a nasolacrimal duct cyst but not to either an intranasal encephalocoele or meningocoele [11,15]. Recent advances in ultrasonography have allowed prenatal diagnosis of nasolacrimal duct cysts at 30 weeks gestation, over half of which resolve spontaneously prior to delivery [16]. Post-natally, computerised tomography (CT) has been the radiologic investigation of choice for nasolacrimal duct abnormalities due to the excellent bony definition [17]; however, we feel that in the neonatal population use of MRI confers several advantages. The enhanced soft tissue definition provided by MRI scanning facilitates differentiation of the intranasal lacrimal duct cyst from surrounding soft tissue structures, rules out other congenital soft tissue abnormalities and excludes intracranial communication or the involvement of neural tissue in the intranasal mass. Simple dacryostocoeles can be managed conservatively with massage and warm compresses. Intranasal lacrimal duct cysts are best managed surgically [5]. Three surgical options have been reported: (1) Blind probing of the nasolacrimal duct [18]. (2) Nasendoscopy and marsupialisation of the intranasal cyst [11]. (3) Combined endoscopic marsupialisation and nasolacrimal duct probing [19,20]. Probing the nasolacrimal duct disrupts the valve of Rosenmuller and allows proximal decompression of the nasolacrimal apparatus. Endoscopic marsupialisation decompresses the system distally, allowing drainage of retained mucopurulent secretions. Combined probing and marsupialisation, as performed in our patient, re-establishes continuity of the nasolacrimal system, facilitating a return to normal, physiological function
4. Conclusion Nasal obstruction causes respiratory distress and compromise in neonatal patients. Causes include
Neonatal respiratory distress
1877
inflammatory conditions, tumours and hamartomas and congenital malformations. Nasolacrimal duct cysts with intranasal extension represent a rare congenital cause of neonatal respiratory distress. These rare congenital abnormalities are caused by obstruction of the nasolacrimal duct at both the valves of Hasner and of Rosenmuller. They can be either unilateral or bilateral, and mode of presentation is determined by the severity of the nasal obstruction. Acute airway stabilisation may be necessary prior to investigation by anterior rhinoscopy, nasendoscopy and either CT or MRI. Definitive management is surgical. Our preferred technique is endoscopic marsupialisation in conjunction with probing and irrigation of the lacrimal ducts to ensure patency of the nasolacrimal apparatus.
References [1] J. Yohendran, A.C. Wignall, E.J. Beckenham, Bilateral congenital dacryocystocoeles with concurrent intranasal mucocoeles causing respiratory distress in a neonate, Asian J. Surg. 29 (2) (2006) 109—111. [2] M.J. Cunningham, J.J. Woog, Endonasal endoscopic dacryocystorhinostomy in children, Arch. Otolaryngol. Head Neck Surg. 124 (1998) 328—333. [3] D. Guery, E.L. Kendig, Congenital impotency of the nasolacrimal duct, Arch. Ophthalmol. 97 (1979) 1656—1658. [4] R.G. Shashy, V. Durairaj, J.M. Holmes, G.G. Hohberger, D.M. Thompson, J.L. Kasperbauer, Congenital dacryocystocele associated with intranasal cysts: diagnosis and management, Laryngoscope 113 (2003) 37—40. [5] H.R. Jin, S.O. Shin, Endoscopic marsupialisation of bilateral lacrimal sac mucoceles with nasolacrimal duct cysts, Auris Nasus Larynx 26 (1999) 441—445.
[6] E.A. Paysse, D.K. Coats, J.M. Bernstein, et al., Management and complications of congenital dacryocele with concurrent intranasal mucocele, JAAPOS 4 (2000) 46—53. [7] R.A. Peterson, R.M. Robb, The natural course of congenital obstruction of the nasolacrimal duct, J. Pediatr. Ophthalmol. Strabismus 15 (1978) 246—250. [8] R.D. Devine, R.L. Anderson, R.M. Bumstead, Bilateral congenital lacrimal sac mucoceles with nasal extension and drainage, Arch. Ophthalmol. 101 (1983) 246—248. [9] J.R. Boynton, D. Drucker, Distension of the lacimal sac in neonates, Ophthalmic Surg. 20 (1989) 103—107. [10] J.C. Edmond, R.V. Keech, Congenital lacrimal sac mucocoele associated with respiratory distress, J. Pediatr. Ophthalmol. Strabismus 28 (1991) 287—289. [11] D. Roy, N. Guevara, J. Santini, L. Castillo, Endoscopic marsupialization of congenital nasolacrimal duct cyst with dacryocele, Clin. Otolaryngol. 27 (2002) 167—170. [12] A.B. Silva, A. Hotaling, W. Rasian, Pathologic quiz case #2–— nasolacrimal duct cyst, Arch. Otolaryngol. Head Neck Surg. 120 (1994) 888—892. [13] K.M. Hepler, G.E. Woodson, D.B. Keams, Respiratory distress on the neonate: sequelae of a congenital dacryocytocele, Arch. Otolaryngol. Head Neck Surg. 121 (1995) 1423—1425. [14] A.M. Mansour, K.P. Cheng, J.V. Mumma, et al., Congenital dacryocele. A collaborative review, Ophthalmology 98 (1991) 1744—1751. [15] B.F. Jaffe, Classification and management of anomalies of the nose, Otolaryngol. Clin. North Am. 14 (1981) 989—1004. [16] W. Supulveda, A.B. Wojakowski, D. Elias, et al., Congenital dacryocystocele: prenatal 2- and 3-dimensional sonographic findings, J. Ultrasound Med. 24 (2) (2005) 225—230. [17] P.K. Rand, W.S. Ball, D.R. Kulwin, Congenital nasolacrimal mucoceles: CTevaluation, Radiology 173 (3) (1989) 691—694. [18] S.W. Yee, R.W. Seibert, C.M. Bower, C.M. Glasier, Congenital nasolacrimal duct mucocoele: a cause of respiratory distress, Int. J. Pediatr. Otorhinol. 29 (1994) 151—158. [19] T.R. Grin, J.S. Mertz, M. Stass-Isern, Congenital nasolacrimal duct cysts in dacryocystocele, Ophthalmology 98 (1991) 1238—1242. [20] G.T. Lueder, Neonatal dacryocystitis associated with nasolacrimal duct cysts, J. Pediatr. Ophthalmol. Strabismus 32 (1995) 102—106.
Available online at www.sciencedirect.com