B-ENT , 2008, 4, 141-145 Congenital dacryocystocele: five clinical cases

C. Hupin *, N. Lévèque *, Ph. Eloy ** , B. Bertrand ** and Ph. Rombaux * *Department of and Head and Neck , University of Louvain, Cliniques Saint Luc, Brussels, Belgium; **Department of Otorhinolaryngology and Head and Neck Surgery, University of Louvain, Cliniques de Mont- Godinne, Yvoir, Belgium

Key-words. Dacryocystocele; newborn; endoscopic endonasal marsupialisation

Abstract. Congenital dacryocystocele: five clinical cases. Congenital dacryocystocele (CDC) is recognised as a cause of nasal airway obstruction or respiratory distress in newborns. CDC is caused by the distal obstruction of the lachrymal duct and presents as a cystic formation in the inferior meatus. We discuss five cases of dacryocystocele, together with surgical management and outcome. Endoscopic endonasal marsupialisation and appropriate postoperative care resulted in definitive recovery for all patients. In newborns or infants with nasal obstruction, CDC should be considered in the differential diagnosis, and prompt endoscopic endonasal marsupialisation is mandatory.

Introduction and a cystic formation in the infe - parents had stopped the local care rior meatus. 24 hours previously. Physical Congenital dacryocystocele (CDC) In each case, we performed examination showed he was is one of the causes of nasal endoscopic endonasal marsupiali - tachypnoeic and using accessory obstruction in newborns. It presents sation, followed by appropriate muscles of respiration. Bilateral as a nasolachrymal or postoperative care, resulting in sibilant rales and purulent nasal obstruction of the nasolachrymal recovery for all patients. No recur - discharge were noted, without duct (NLD) with cystic extension rence was observed during follow- fever. Nasal fibroscopy showed a into the nasal fossa. When tears up. cystic mass filling the right inferi - and secretions accumulate in the or meatus. lachrymal sac as a result of an The patient was taken to the Case Report upper or lower obstruction in the operating room with the diagnosis lachrymal drainage apparatus, of right dacryocystocele. Nasal Case 1 there is dilatation of the lachrymal under general anaes - sac and often protrusion of the A full-term male infant presented thesia confirmed the diagnosis and cyst into the nasal lumen. 1 with dyspnoea, with evidence of marsupialisation was performed, Dacryocystocele, also called nasal obstruction, immediately in conjunction with probing of the dacryocele, lachrymal sac cyst, after birth. Choanal atresia and lachrymonasal duct. A peropera - nasolachrymal duct mucocele, pyriform aperture stenosis were tive CT scan showed a distended amniotocele or amniocele is a excluded after clinical exami- lachrymal sac and NLD. rare disease. 2 Nevertheless, this nation. Purulent was Nasal breathing improved condition must be included in the diagnosed, with Streptococcus immediately after surgery and the differential diagnosis of any new - Viridans. Local care was pre - patient remained asymptomatic born or infant with respiratory scribed: saline solution rinses, during the three years of follow- distress and/or nasal obstruction. oxymetazoline, bacitracine- up. We describe five cases of neomycine. The infant was dis - neonates with CDC. Patients pre - charged after four days of life and Case 2 sented either with nasal obstruc - evolution was excellent. Two tion, or a bluish swelling weeks later, he was admitted to An 8-day-old male infant was in the region of the medial canthus A&E for respiratory distress. The referred to our department for the 142 C. Hupin et al. evaluation and treatment of a right lent material were expressed. No up (16 months at present), there periorbital abscess. organisms grew in any cultures has been no evidence of recur - Urinoma due to a junction syn - of this discharge. Evolution was rence. drome was diagnosed during preg - rapidly positive, with reduction nancy, with good spontaneous of the periorbital swelling and Case 4 evolution. Biometrics were normal. normalisation of ocular motility. A two-month-old male infant was Birth at term via spontaneous vagi - Postoperative lachrymal sac mas - referred to our ENT outpatient nal delivery was uncomplicated. sages as well as saline rinses were clinic for the evaluation of left The Apgar score was normal. performed. At present, no recur - epiphora. Since birth, the patient Over the course of the next day, a rence has been observed. had presented a left ocular dis - bilateral purulent ocular discharge charge. Pressure on the medial was seen. Topical antibiotics Case 3 canthus showed pus in the lachry - (ofloxacine) were administered. mal points. Probing had been per - Two days later, periorbital erythe - A 2-week-old male infant whose formed previously under local ma and upper lid swelling parents and neonatologists had anaesthesia without success. appeared on the right side. The noted slow progressive respiratory Clinical examination showed a patient was given oral antibiotics distress associated with nasal bluish-grey cystic swelling arising (amoxicillin-clavulanic acid + obstruction was transferred to our beneath the left inferior turbinate. erythromycin) and the symptoms hospital. The medical history Diagnosis of left CDC was con - improved for 48 hours, after revealed an uncomplicated preg - firmed. which the swelling increased nancy, gastro-oesophageal reflux Endonasal endoscopic marsupi - again, as did the ocular discharge. since birth and neonatal jaundice alisation resulted in complete IV antibiotics were started treated with five days of pho - resolution of the mucocele and (amoxicillin-clavulanic acid + totherapy. Clinical examination improvement in symptoms. netilmicine ), and oral erythromy - upon admission showed right con - Microbiology revealed Staphylo - cin and local ofloxacine were junctivitis related to an infected coccus (coagulase-negative). The maintained. The day after, exoph - homolateral lachrymal cyst. patient remained asymptomatic thalmia was noted and so a CT Purulent discharge was present, during the 6 months of follow- scan was performed. It showed a which grew Streptococcus up. right periorbital abscess. No fever Viridans and Staphylococcus was observed throughout this peri - (coagulase-negative) on culture. Case 5 od. Nasal endoscopy was performed Six days after the onset of the and bilateral lachrymal with A 12-day-old female infant with first symptoms, the infant was right were found uneventful birth and pregnancy transferred to our hospital. (Figures 1a,1b). The patient was was referred to our ENT outpa - Clinical examination showed right given intravenous antibiotics with tient clinic with a diagnosis of exophthalmia, major palpebral anti-reflux treatment and local bilateral dacryocystocele. The swelling, purulent ocular dis - care (topical antibiotics and vaso - infant was born via spontaneous charge and decreased ocular hori - constrictor nasal drops). Forty- vaginal delivery. At birth, facial zontal motility. The diagnosis of eight hours later, the lachrymal swelling was noted by parents and right NLD obstruction with dacry - cysts were marsupialised under nurses. The swelling worsened ocystitis, periorbital cellulitis and endoscopic control. Following progressively, especially at the subperiosteal abscess was made. surgery, a marked improvement internal angle of the orbit. IV antibiotics were changed to IV was seen in the patency of the Respiratory distress was noted, cefotaxime + oral erythromycin. nasal airways and respiratory dif - especially during feeding. Surgery was scheduled for the ficulties improved immediately. Choanal atresia had been excluded same day. Endoscopy-guided mar - Two days later, the patient was by passing an aspiration sonde supialisation was performed, as discharged, with oral amoxicillin- through both nostrils. The infant well as ethmoidectomy and clavulanic acid, oxymetazoline, had been examined by an ENT drainage of the subperiosteal saline rinses and massage of the specialist who had diagnosed abscess. Three millilitres of puru - internal canthus. During follow- bilateral CDC and referred her to Congenital dacryocystocele 143

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Figure 1 Endoscopic endonasal view of the inferior meatus, showing a cyst beneath the inferior turbinate. a: right nasal fossa; b: left nasal fossa

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Figure 2 Endoscopic endonasal view of the right inferior meatus with cyst formation. a: before marsupialisation; b: after marsupialisation our hospital. Clinical examination tage of the general anaesthesia to after, patient was sent home, with revealed a bluish cutaneous mass perform a CT scan of the head and oral amoxicillin-clavulanic acid, inferior and lateral to the lachry - sinus (to exclude other causes for local oxymetazoline, saline rinses mal sac, with hypertelorism, the hypertelorism), which showed and internal canthus massages. At caused by the extra soft tissue bilateral cystic dilatation of the present, no recurrence has been medial to the eye. Nasal lachrymal sac and nasolachrymal noted. endoscopy showed cystic masses duct, forming masses that The summary of the clinical filling the inferior meatus of both obstructed both inferior meatus characteristics of these five nasal cavities, confirming the (Figures 3a,3b,3c,3d). No other patients is presented in Table 1. diagnosis. pathologic findings were detected. At 20 days of life, under direct A histological section of the cyst Discussion endoscopic visualisation, the cyst showed normal nasal mucosa. walls were marsupialised and a Immediately after surgery, perior - CDC is an uncommon disease thick discharge was evacuated bital and facial swelling decreased which is mainly caused by an (Figures 2a,2b). Just before and respiratory symptoms obstruction at the distal end of the starting surgery, we took advan - improved significantly. The day nasolachrymal duct. 3 144 C. Hupin et al.

Table 1 Clinical characteristics of CDC Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Gender Male Male Male Male Female Age at diagnosis 18 days 8 days 14 days 2 months 2 days Laterality Right Right Bilateral Left Bilateral Nasal obstruction +++++ Respiratory distress +- +/- -- Complications None Periorbital Dacryocystitis None None cellulitis Other congenital anomalies None None None None None Follow-up 38 months 45 months 16 months 6 months 2 months

one of our patients was female and three presented with unilateral dis - ease. The disease usually presents with an intranasal cystic mass in the inferior meatus, frequently associated with epiphora and/or a painless bluish swelling below the medial canthus. 5 Complications such as dacryocystitis, preseptal cellulitis, and respiratory distress ab have been described. 4,6 In newborns with respiratory distress and/or nasal obstruction, differential diagnosis should con - sider several diseases: malforma - tions include choanal atresia, pyri - form aperture stenosis, craniofa - cial dysmorphia and CDC. 7,8 It should be noted that upper airway obstruction secondary to CDC did not usually lead to severe respira - cd tory distress requiring intubation or intensive care. Tumours should Figure 3 be excluded (teratoma, nasal CT scan in horizontal planes, showing a cystic formation in the inferior meatus (right glioma, , meningo - and left) and increased volume of lachrymal sac due to obstruction of the lachrymal cele, hemangioma) as well as pathways. Arrows indicate dacryocystocele in the inferior meatus. 3a,b,c,d: from below to upper part of the lachrymal pathways. infectious (neonatal rhinitis) or traumatic aetiologies (septal injury during birth). 9 The diagnosis of CDC is made by clinical observation. A com - Symptoms of CDC can be This is more frequent in plete clinical examination is there - present at birth or appear a few fe males (65 to 80%) and can be fore essential for a correct diagno - days later, as in two of our cases. either uni- or bilateral. 1,2,4 Only sis and to provide appropriate Congenital dacryocystocele 145 treatment. Facial examination, firming diagnosis, the CDC was 2. Mansour AM, Cheng KP, Mumma JV, anterior rhinoscopy with palpation opened using a straight Blakesley et al . Congenital dacryocele. A col - of the cyst and meticulous nasal forceps. Resection of the inferior laborative review. . 1991;98:1744-1751. endoscopy are mandatory. If mucosa helped to marsupialise the 3. Tardiff A, Cole P, Wesley RE, necessary, further investigations CDC and the contained secretions Jeanty P. Dacryocystocele. 1991. may include facial computed were suctioned. Care was taken to Available at: http://www.TheFetus.net. tomography scan or MRI. ensure a patent opening of the Accessed July 15, 2008. Imaging may help in the evalua - lachrymal pathway. No nasal 4. Duval M, Alsabah BH, Carpineta L, Daniel SJ. Respiratory distress sec - tion of other craniofacial abnor - packing was required except in the ondary to bilateral nasolacrimal duct malities. patient (2) where an ethmoidecto - mucoceles in a newborn. Otolaryngol Management of CDC requires my was also performed. Head Neck Surg. 2007;137:353-354. conservative and surgical manage - Oral and topical antibiotics, 5. Jin HR, Shin SO. Endoscopic marsu - ment. 10 normal saline solution in the nasal pialization of bilateral lacrimal sac Conservative treatment may be fossae and repeated massage of the mucoceles with nasolacrimal duct cysts. Auris Nasus Larynx. 1999;26: 2,6 advised in uncomplicated cases lachrymal sac should be adminis - 441-445. and includes nasal decongestion, trated after surgical procedure. 6. Becker BB. The treatment of congen - saline rinses of nasal fossae and ital dacryocystocele. Am J Ophtalmol. lachrymal ducts, topical antibi - 2006;142:835-838. Conclusion otics and lachrymal sac massage. 7. Jonniaux E, Brihaye P, Bernheim N, Mansbach AL. Apertura pyriform This may lead to a spontaneous Congenital dacryocystocele is a stenosis in the newborn. B-ENT . opening of the inferior meatus of rare clinical entity but all neonates 2006;2:31-33. the lachrymal duct but is not suc - with respiratory distress and/ 8. Gordts F, Clement PA, Rombaux P, cessful in the vast majority of the Claes J, Daele J. Endoscopic or nasal obstruction should be cases. endonasal surgery in choanal atresia. examined for this diagnosis. We recommend the early con - Acta Otorhinolaryngol Belg. 2000;54: Anterior rhinoscopy and endo - 191-200. sideration of surgical management nasal endoscopy with visualisa - 9. Rombaux P, Hamoir M, Eloy P, to avoid complications such as tion of the inferior nasal meatus Bertrand B. L’obstruction nasale chez infection (dacryocystitis, cellulitis) l’enfant. Louvain Med. 1998;117: are therefore required. or respiratory difficulties. Typi - S402-S409. To facilitate nasal breathing cally, under general anaesthesia, 10. Shashy RG, Durairaj VD, Holmes JM, and to avoid complications, we Hohberger GG, Thompson DM, drainage of the cystic mass is per - recommend prompt endoscopic Kasperbauer JL. Congenital dacry - formed, followed by marsupialisa - endonasal marsupialisation of the ocystocele associated with intranasal tion of the cyst itself through an cyst in CDC. cysts: diagnosis and management. endoscopic endonasal approach. Laryngoscope. 2003;113:37-40. The current results indicate that The introduction of a bicanalicu - surgical treatment is successful in lonasal probe in the lachrymal duct all neonates/infants with early is not mandatory in primary cases. endonasal endoscopic surgery. Surgical procedure starts with Ph. Rombaux, M.D., Ph.D. vasoconstrictive agents (cottonoid Department of Otorhinolaryngology and pledgets soaked in a solution of References Head and Neck Surgery University of Louvain epinephrine: one gamma/kg). The Cliniques Saint Luc 1. Teymoortash A, Hesse L, Werner J, endoscopes used were 2.7 mm avenue Hippocrate 10 Lippert BM. Bilateral congenital and 4 mm in diameter, 0° or B-1200 Brussels, Belgium dacryocystocele as a cause of respira - Tel.: +32.2 764 19 30 30° (Storz-Hopkins telescopes) tory distress in a newborn. Rhinology. Fax: +32.2 764 89 35 clipped to a camera. After con - 2004;42:41-44. E-mail: [email protected]