Lacrimal drainage anomalies in Tessier cleft 3 with unilateral anophthalmos.

Bilateral Tessier cleft types 3 and 4 are rare and commonly involve the lacrimal drainage system owing to their anatomical location. Such clefts commonly present with associated ocular anomalies and include colobomatous eyelids, hypertelorism, microphthalmia, punctal or canalicular agenesis, and nasolacrimal duct obstruction or exstrophy. The current report presents an 18-month-old baby with bilateral Tessier cleft 3 with a unilateral anophthalmos, symmetrical eyelid colobomas, and lacrimal drainage anomalies. The lacrimal anomalies noted include small lacrimal sac with inferior canaliculus on the right side and upper and lower punctal and canalicular agenesis on the left side. Computed tomographic dacryocystography demonstrated unilateral lacrimal sac and bilateral maldevelopment of the bony nasolacrimal duct.

Congenital canaliculops with punctal agenesis: is there a possibility to establish patency?

Canaliculops is a non-inflammatory dilatation of the canaliculus which can be secondary to trauma, punctal agenesis, herpetic infections or chronic drug use. This condition can affect either canaliculi and has shown female preponderance. Treatment described so far has consisted of cyst marsupialization with no literature on the post-operative epiphora in these cases. We describe two cases of canaliculops with punctal agenesis where we have tried to establish lacrimal patency.

Conservative management of congenital dacryocystocele: resolution and complications.

OBJECTIVE: To investigate the clinical outcomes of initial conservative management in infants with congenital dacryocystocele. DESIGN: Retrospective case series. PARTICIPANTS: A total of 30 congenital dacryocystoceles of 28 Korean infants treated between January 2006 and December 2015. METHODS: All patients were managed conservatively with lacrimal sac massage. Clinical courses and outcomes of the patients were retrospectively reviewed, and rates of resolution of dacryocystoceles and development of dacryocystitis were analyzed. RESULTS: In 27 cases of dacryocystoceles without infection, spontaneous resolution was achieved without any complication in 20 of 27 (74.1%) cases after conservative treatment. The mean duration of treatment was 27.5 days. Lacrimal probing was needed in 5 (18.5%) dacryocystoceles that persisted despite lacrimal sac massage for more than 1 month. Infectious dacryocystitis developed in 2 of 27 (7.4%) cases. Three dacryocystoceles were infected at presentation. Overall, 5 dacryocystoceles were complicated with infection and those cases received prompt systemic antibiotics treatment. External incision and drainage of the lacrimal sac was needed in 3 dacryocystoceles, and all cases were finally resolved without any additional procedures. CONCLUSIONS: In this case series, most of the uninfected dacryocystoceles could be successfully managed with conservative treatment alone. In cases with infection, systemic antibiotics were essential and external drainage was sometimes required, but these cases could be conservatively managed after the remission of infection.

Congenital Dacryocystocele: A Major Review.

PURPOSE: To provide a systematic review of the literature on congenital dacryocystoceles (CDCs) and summarize their presentations, investigations, management, and outcomes. METHODS: The authors performed a PubMed search of all articles published in English on CDCs. Data captured include demographics, clinical presentations, investigations, management modalities, complications, and outcomes. Fourteen major series (10 or more than 10 cases) and 89 isolated case reports/series on CDCs with a collective patient pool of 1,063 were studied in detail. Specific emphasis was laid on addressing the controversial issues including initial conservative versus surgical management and the role of endoscopic evaluation. RESULTS: Numerous terminologies have been used to describe CDC. Congenital dacryocystoceles are rare variants of congenital nasolacrimal duct obstructions and comprise of 0.1% to 0.3% of all such cases. There is a female predilection (64.2%, 683/1,063) and the mean age at presentation is at 7 days of birth. Initial conservative treatment can be a viable option in the absence of an acute dacryocystitis or a respiratory distress. Endoscopy-assisted probing appears to have better outcomes as compared with the in-office probing. Congenital dacryocystoceles with acute dacryocystitis are preferably managed with intravenous antibiotics and an early probing under endoscopy guidance to avoid missing intranasal cysts. Marsupialization is the preferred technique in the management of intranasal cysts. Silicone intubation was rarely used and has no definitive indications. Dacryocystorhinostomy is very rarely needed in the management of CDC. CONCLUSIONS: Congenital dacryocystocele is a commonly accepted term and its use should be advocated to enhance uniformity in reporting. Endoscopic evaluation of CDC is useful in the diagnosis and treatment of associated intranasal cysts and enhances the rates of successful outcomes.

Selection of surgical intervention for congenital dacryocystocele.

PURPOSE:: To evaluate the surgical intervention and its effect on congenital dacryocystocele. METHODS:: A total of 531 children with congenital dacryocystocele admitted to the Department of Ophthalmology of Beijing Children's Hospital, Shanghai Aier Eye Hospital, Nanjing Aier Eye Hospital, and the First Affiliated Hospital of Jinzhou Medical University between January 2007 and January 2017 were retrospectively analyzed. RESULTS:: A total of 531 patients were followed up for 3-24 months, with an average of 13.3 months. No serious intraoperative complications (such as bleeding and tissue damage) and postoperative complications (bleeding, infection, and hole atresia) were found. We classified the outcomes into three categories based on the signs (overflowing tears and empyema) and objective routine follow-up by endoscopy. Cure indicated that mass, overflow of tears, and breathing difficulties disappeared, and 81.5% cases (433/531) were postoperatively cured. Improvement indicated disappearance of mass, overflow empyema, and the presence of residual tears (due to trocar and tears puncture), and 18.5% cases (98/531) were postoperatively improved. Unhealed indicated overflowing pus and tears, and 0% cases did not heal after surgery. CONCLUSIONS:: Nasal endoscopic surgery is relatively safe, with high success rate for treatment of congenital dacryocystocele. Systematic training is required to promote the application of nasal endoscopy, so that more ophthalmologists can learn this technique.

Congenital bilateral dacryocystocele.

INTRODUCTION: Newborns are obligatory nasal breathers. Therefore, nasal obstruction can lead to cyanosis and desaturation. In spite of being very rare, congenital bilateral dacryocystocele is a possible etiology for neonatal respiratory distress. CASE SUMMARY: Case report of a male newborn with respiratory distress caused by a bilateral polypoid and bluish lesion occupying almost the entire inferior nasal meatus. Imaging confirmed bilateral dacryocystocele. Treatment was conservative. There was spontaneous drainage, with relief of respiratory distress. Discussion The diagnosis of congenital dacryocystocele is clinical, although imaging exams may be requested to confirm it. Treatment is controversial, because the natural history is variable. An initial conservative management may be recommended, but, if there is a permanent respiratory obstruction without improvement, surgical management is mandatory.

Dacryoendoscopy-assisted nasal endoscopic marsupialization for congenital dacryocystocele.

OBJECTIVE: To examine the surgical outcome of dacryoendoscopy-assisted nasal endoscopic marsupialization for congenital dacryocystocele. METHODS: This is a retrospective, observational study that included 7 sides of 6 patients with congenital dacryocystocele. The surgical indication in 5 of the patients was acute dacryocystitis while one of the patients had no spontaneous resolution after long-term observation. Dacryoendoscopy was used for visualisation of the lacrimal cavity, direct probing of the obstruction at the common canaliculus, and localisation of a deflated intranasal cyst after an initial incision of the cyst. Surgical success was defined when the following postoperative conditions were satisfied: no epiphora, normal tear meniscus height, and complete clearance of the dye 5 min after instilling a drop of 2% fluorescein solution into the inferior conjunctional fornix. RESULTS: Out of 6 patients, 2 patients were noted to have membranous common canalicular obstruction. Postoperative follow-up ranged from 11 days to 3 months. Surgical success was achieved in all patients without any complication. CONCLUSIONS: Dacryoendoscopy-assisted nasal endoscopic marsupialization is an effective surgical management for congenital dacryocystocele.

Congenital dacryocystocele: sonographic evaluation of 11 cases.

PURPOSE: To report prenatal sonographic findings of congenital dacryocystocele. METHODS: The medical records of all cases diagnosed with congenital dacryocystocele at a tertiary care center from 2003 to 2015 were reviewed retrospectively. RESULTS: Eleven cases of congenital dacryocystocele were analyzed. No accompanying fetal anatomic anomalies where detected. Mean maternal age at evaluation was 22 years of age (range, 17-32 years). Four of 10 cases were primigravidas. The mean gestational age at evaluation was 32.6 weeks' gestational age. Ten out of 11 cases occurred in female fetuses (91%). Of the 11, 10 cases were unilateral and 1 was bilateral. The mean diameter at evaluation was 5.1 mm. Spontaneous resolution occurred in 2 cases (18%). In the remaining 9 fetuses, postnatal diagnosis of dacryocystocele were confirmed by an ophthalmologist evaluation. CONCLUSIONS: Prenatally diagnosed congenital dacryocystocele may undergo spontaneous resolution before birth. However, a better understanding of the prenatal sonographic findings can help to appropriately orient parents of potentially affected fetuses. Referral to a pediatrician and pediatric ophthalmologist may be considered for complete evaluation and postnatal management.

[Periorbital, blue tumour in newborn twins]. / Periorbitale, blauwe zwelling bij pasgeboren tweeling.

Newborn twins both had a blue, smooth tumour in the inner angle of the orbit; one of them had two such tumours. They were diagnosed with congenital dacryocystoceles. If decompression into the nose is not effective, patients should undergo probing early in life to reduce the incidence of dacryocystitis and orbital cellulitis.

Syndromic and Nonsyndromic Systemic Associations of Congenital Lacrimal Drainage Anomalies: A Major Review.

PURPOSE: To review and summarize the syndromic, nonsyndromic, and systemic associations of congenital lacrimal drainage anomalies. METHODS: The authors performed a PubMed search of all articles published in English on congenital lacrimal anomalies (1933-2016). Patients of these articles were reviewed along with the literature of direct references to syndromes and other systemic associations. Data reviewed included syndromic descriptions, systemic details, demographics, lacrimal presentations, management, and outcomes. RESULTS: Syndromic and systemic associations with congenital lacrimal drainage disorders are not known to be common. Although familial presentations have been reported, the inheritance patterns are unclear for most anomalies. There is an increasingly growing evidence of a genetic basis to many lacrimal anomalies. However, few syndromes have either widespread or are frequently associated with lacrimal anomalies. Few sequences of distinct lacrimal presentations and intraoperative findings are seen. Surgical challenges in these patients are distinct and a thorough pre and intraoperative anatomical assessment, detailed imaging when indicated, and assessment and correction of associated periocular and facial abnormalities may facilitate good outcomes. CONCLUSIONS: Lacrimal drainage anomalies associated with syndromic and nonsyndromic systemic conditions have certain unique features of their own and their surgical and anesthesia challenges are distinct. Diagnosis of congenital lacrimal drainage anomalies should prompt consideration of the possible presence of associated systemic abnormalities.

Long-term outcomes of cruciate marsupialization of intra-nasal cysts in patients with congenital dacryocele.

OBJECTIVES: To evaluate the long-term outcomes of cruciate marsupialization of intranasal cysts associated with congenital dacryocele. METHODS: This is a retrospective review of 15 patients of dacryoceles with intranasal cysts who underwent cruciate marsupialization of the medial wall of the cyst. Post-operative follow up for a minimum of 9 months was an inclusion criterion for analysis. Data analyzed include demographics, clinical presentations, previous interventions, associated systemic abnormalities, clinical course following marsupialization and recurrences. Primary outcome measures were resolution of the dacryocele and symptomatic relief from epiphora. RESULTS: The mean age at presentation was 1.6 months with a female preponderance (60%). History of prematurity (pre-term) was noted in 20% (3/15). Swelling in the lacrimal sac area (dacryocele) was the universal presentation. Five patients (33.3%) had a prior conservative treatment. Four patients (26.6%) had respiratory distress with feeding. Acute dacryocystitis was noted in 26.6% (4/15). At a mean follow up of 19.1 months, successful outcomes were achieved in 86.6%. CONCLUSION: Cruciate marsupialization of intranasal cysts achieves good results in patients with congenital dacryocele, which are maintained over a long period of time. Early diagnosis and management may prevent acute dacryocystitis in these patients.

Bilateral dacryocystocele with an intranasal cyst as the cause of respiratory distress in a newborn.

A congenital dacryocystocele with an intranasal cyst is an uncommon lesion that is usually treated by ophthalmologists, although sometimes an otorhinolaryngologist is consulted first because of nasal obstruction. The nasal cavity is narrow in newborns and can easily be obstructed, even by small lesions. Prolapse or expansion of the cyst into the nose may lead to respiratory distress and difficulty in feeding, since newborns are obligate nose breathers. Here we report a case of bilatera dacryocystocele with intranasal extension in a 3-day-old female infant. The infant presented with respiratory distress and episodic desaturation and was managed successfully by bilateral endoscopic marsupialization of the intranasal cysts. This case report discusses the diagnosis and management and reviews the relevant literature. These finding suggest tha congenital dacryocystocele with an intranasal cyst must be considered in the differential diagnosis of newborns suffering from nasal respiratory difficulty.

Management of congenital dacryocystocele: report of 3 clinical cases.

This case study describes three newborns referred to our otolaryngologic service for investigating and treating a cystic dilatation of the lacrimal duct. These dilatations corresponded to unilateral or bilateral dacryocystoceles, with or without complications. The first newborn exhibited respiratory distress at birth and received early surgery and endoscopic marsupialization of intranasal and bilateral cysts. The second newborn did not show any signs of complications, and after conservative treatment for a week, the cyst spontaneously resolved. The third newborn was diagnosed in utero with ultrasonography, and the cyst resolved spontaneously during childbirth. These cases provided an opportunity to review the pathophysiology of this rare congenital lacrimal anomaly and to note responses to different therapeutic approaches. Indeed, these three cases illustrated three different management approaches, and allowed us to address the issue of prenatal diagnosis.