25 Lacrimal System

289 CONGENITAL ANOMALIES OF

THE LACRIMAL SYSTEM 743.65

(Congenital Nasolacrimal Duct

Obstruction, Dacryocystitis,

Dacryocystocele, Accessary [Anlage]

Punctum, Punctal Stenosis,

Canalicular Stenosis)

David K. Coats, MD

Houston, Texas

Nasolacrimal outflow obstruction will resolve spontaneously during the first year of life in most patients. More than half of patients will be asymptomatic by 6–8 months of age and approximately 90% will be asymptomatic by 1 year of age. Dacryocystitis occasionally develops and can be life threatening in young infants. Dacryocystitis commonly occurs in association with a dacryocystocele. Respiratory distress can occur in infants with a unilateral or bilateral dacryocystocele because of the presence of cystic dilation of the nasal mucosa at the distal end of the nasolacrimal canal, which blocks the nasal passage.

Symptoms that are notably absent in infants with lacrimal outflow obstruction include photophobia and corneal clouding. When present, a more ominous condition, such as congenital glaucoma, should be considered.

ETIOLOGY/INCIDENCE

The most common congenital anomaly of the lacrimal drainage system is outflow obstruction, which may be present in as many as 5–6% of infants. The most common site of obstruction is at the distal portion of the nasolacrimal duct near its entry into the inferior meatus. Here, a membrane known as Hasner’s membrane, fails to regress, producing symptomatic outflow obstruction.

Less common clinical anomalies include punctal atresia or stenosis, canalicular atresia or stenosis, and accessory lacrimal puncta. A dacryocystocele is a congenital anomaly that may develop when there exists an obstruction at the valve of Hasner distally and a proximal obstruction at the valve of Rosenmuller. A dacryocystocele is typically obvious in the first few weeks of life and can be associated with dacryocystitis and/or respiratory distress as described below.

COURSE/PROGNOSIS

When the obstruction of the lacrimal drainage system is distal to the nasolacrimal sac, the symptoms are overflow tearing and recurrent mucopurulent discharge, as bacteria and debris sequestered within the lacrimal sac reflux onto the ocular surface. When the obstruction is proximal to the lacrimal sac, the symptoms are typically tearing only, with little or no mucopurulent discharge. Tearing is rarely present at birth in patients with lacrimal outflow obstruction, but usually develops in the first weeks of life. Among the most prominent symptoms are mucopurulent debris on the lashes upon awakening that may result in adhesion of the upper and lower lids together. Pressure on the nasolacrimal sac will often result in obvious reflux of tears and/or mucupurulent material from the punctum.

DIAGNOSIS

Clinical testing

Though children are inherently difficult to evaluate, office testing can be of value in selected cases when the clinical diagnosis is not obvious. An increased tear lake is almost always present. A dye disappearance test can be performed by placing fluorescein drops in the inferior cul-de-sac. In a child with an intact lacrimal drainage system, the dye will typically egress through the lacrimal drainage system within 3 to 5 minutes, with minimal or no overflow onto the cheeks. In a child with lacrimal outflow obstruction, in contrast, the fluorescein dye remains in the tear meniscus for a prolonged period of time and often overflows onto the cheek. Reflux of tears and/or mucopurulent material from the punctum with pressure on the nasolacrimal sac is probably the simplest confirmatory test in a young child.

DIFFERENTIAL DIAGNOSIS

The majority of children who present with persistent tearing and/or discharge, will be diagnosed with a nasolacrimal duct obstruction. The diagnosis is generally obvious. A number of more serious problems can masquerade as a nasolacrimal duct obstruction, and include congenital glaucoma, keratitis, uveitis, trichiasis, and corneal foreign body. These more serious problems can usually be readily distinguished from a simple nasolacrimal outflow obstruction by the presence of photophobia, corneal clouding, corneal enlargement, and/or persistent ocular redness, none of which occur in the child with isolated nasolacrimal duct obstruction.

System Lacrimal • 25 SECTION

TREATMENT

Nasolacrimal outflow obstruction

Because nasolacrimal outflow obstruction spontaneously resolves during the first year of life in most children, conservative therapy is typically warranted. Nasolacrimal sac massage is often recommended several times per day, both to empty the nasolacrimal sac and to potentially hasten resolution of the obstruction. Topical antibiotics are usually prescribed when mucopurulent discharge is present throughout the day. Topical antibiotics typically need not be used when discharge is present only upon awakening. Systemic antibiotics are indicated in the presence of an associated dacryocystitis and/or preseptal cellulitis.

Surgical

Probing of the nasolacrimal drainage system can be done in an office or operating room setting. Office probing is limited to smaller children, typically those less than 1 year of age. The recommended age at initial probing varies among practitioners, with some probing as early as 4 to 6 months of age, while others wait until a year or more of age. Probing in the office is done under topical anesthesia after wrapping the child in a blanket or restraining the child in a papoose board.

There are a number of surgical treatment options available for children undergoing treatment in the operating room. Many surgeons prefer probing and irrigation alone as the initial treatment for all children, reserving adjunctive therapies, such as silicone tube intubation and balloon dilation, for treatment failures. Other surgeons prefer to use adjunctive measures at the time of initial probing when treatement is performed under general anesthesia. If probing is performed alone, without adjunctive measures, irrigation of the lacrimal drainage system with fluorescein dyed saline solution and suction retrieval from the nose should be used to confirm patency of the lacrimal drainage system.

Silicone tubes can be placed in the lacrimal drainage system as a stint to reduce the tendency for the newly opened duct to close. A variety of tube delivery systems are available, including monocanalicular and bicanalicular systems. Tubes may be removed in the office weeks to months after placement, depending on the preference of the surgeon.

Balloon catheter dilation has been reported to be an effective primary surgical treatment for nasolacrimal duct obstruction. A collapsed balloon is placed through the punctum and into the nasolacrimal canal. It is then inflated to dilate the lacrimal drainage system. Because of the high cost of balloon catheters relative to other treatment options, many surgeons reserve balloon catheters for special situations, including treatment failures.

Controversy exists regarding the timing and method of treatment for children with dacryocystoceles. Certainly the child with respiratory distress should be treated urgently. For those without respiratory distress, some authors recommend massage and systemic antibiotics, with observation to allow time for spontaneous resolution. Others, citing a high incidence of dacryocystitis, recommend probing as soon as possible after the diagnosis is made. Initial probing can be performed in the office. Treatment failures are common and repeat treatment in the operating room is sometimes needed. Surgical removal of an associated intranasal mucocele, when present, may be needed to affect resolution. Identification and treatment of intranasal mucoceles can be facilitated by use of nasal endoscopy.

COMPLICATIONS

Complications of surgical treatment for lacrimal outflow obstruction are uncommon. Recurrence or persistence of symptoms may occur in a small number of patients. Damage to the mucosa lining the lacrimal outflow system, or creation of a false passage can occur and may render future treatment more difficult. Damage to the nasal mucosa and adjacent turbinates can occur, but is infrequent.

COMMENTS

Punctal stenosis can be overcome by dilation and/or a snip punctalplasty to enlarge the punctal opening. Puncture of a membranous covering over the puncta will relieve obstructions from this cause. Canalicular stenosis can be overcome by passing probes of gradually increasing diameter, followed by stenting to promote maintenance of the newly enlarged canaliculus.

Dacryocystorhinostomy and conjunctivodacryocystorhinostomy are occasionally indicated in children who fail standard treatment for lacrimal outflow obstruction. These procedures are generally deferred until children are 5 to 6 years of age, and are needed infrequently.

Ectopic (anlage) lacrimal ducts do not require treatment unless symptomatic. Tearing and/or discharge from an anlage duct may require treatment. The anomalous duct is removed intact by ligating it near the lacrimal sac followed by excision of the ectopia duct and closure of the overlying skin.

Nasolacrimal outflow obstruction tends to be more complex in patients with mid-face anomalies, including children with Down syndrome. In children with Down syndrome, abnormalities of the lacrimal drainage system proximal to the lacrimal sac predominate, including canalicular stenosis and canalicular atresia. Adjunctive therapies such as silicone tube intubation and balloon dilation are often considered during initial treatment.

Symptomatic nasolacrimal duct obstruction is common in infants, affecting up to 6% of newborn infants. Conservative treatment is typically recommended because spontaneous resolution will occur in most patients. Some surgeons recommend probing in the office as early as 4 to 6 months of age, while others wait until the child is a year or more of age. When performed in the operating room, probing and irrigation alone can be utilized as the initial treatment or probing can be combined with an adjunctive measure such as silicone tube intubation or balloon catheter dilation initially.

Complications of lacrimal outflow obstruction are uncommon and primarily involve infection of involved and surrounding tissues, and respiratory obstruction in the case of dacrycystoceles. Complications associated with treatment are also uncommon and can be minimized by proper selection of probes, adjunctive measures, and optimal technique. Failure of standard probing with or without adjunctive measures is uncommon, though repeat treatment is sometimes needed. Dacryocystorhinostomy and conjunctivodacryocystorhinostomy are rarely required.

REFERENCES

Lueder GT: Balloon catheter dilation for treatment of older children with nasolacrimal duct obstruction. Arch Ophthalmol 120:1685–1688, 2002.

290 CHAPTERDacryoadenitis •

System Lacrimal • 25 SECTION

Mafee MF, Edward DP, Koeller KK, Dorodi S: Lacrimal gland tumors and simulating lesions. Clinicopathologic and MR features. Radiol Clin North Am 37(1):213–239, 1999.

Jakobiec FA, Jones IS: Orbital inflammations. In: Duane TD, ed: Clinical ophthalmology. Haggerstown, Harper & Row, 1982:II:64–69.

291 DACRYOCYSTITIS AND

DACRYOLITH 375.30

Robert N. Tower, MD

Seattle, Washington

ETIOLOGY/INCIDENCE

Dacryocystitis is an inflammation in the lacrimal sac resulting from stasis of tears in the lacrimal drainage system and secondary infection by bacteria or fungus. It may be present in infancy (congenital nasolacrimal duct obstruction), affecting 2% to 6% of live births, or maybe an acquired condition. It most frequently presents in middle age, but it is not restricted to any age group.

Dacryolith is a concretion of material in the canaliculi, lacrimal sac, or nasolacrimal duct. It partially or completely obstructs the drainage of tears. Dacryoliths are frequently present in fungal canaliculitis, and as a distinct entity it may be present in many patients with chronic dacryocystitis and a patent nasolacrimal duct.

Infantile dacryocystitis

Causes include incomplete canalization of the nasolacrimal duct, with an obstructing membranous remnant; nasolacrimal duct atresia; facial cleft; and dacryocele.

Acquired dacryocystitis

●Chronic or acute infection is a common denominator.

●Trauma, nasal fracture or surgery, and laceration or blunt trauma to lacrimal sac are causes.

●In functional block, the nasolacrimal duct is patent, but lacrimal excretory function is compromised.

●Dacryolith is present in 15% to 20% of patients with functional block.

●Tumor is intrinsic in the lacrimal sac or extrinsic and impinging on the sac or duct.

●Granuloma, inflammatory or infectious disease are the causes.

●Retained foreign body, including the Silastic tubing from prior lacrimal intubation, can be a cause.

Canaliculitis

●The origin is usually fungal: Candida, Aspergillus, Nocardia, and Actinomyces spp., among others.

●A bacterial origin is unusual except after trauma.

COURSE/PROGNOSIS

Infantile dacryocystitis

Congenital nasolacrimal obstruction usually presents in the first 6 weeks of life with epiphora and chronic mucopurulent discharge in one or both eyes. Most infants will have

spontaneous resolution of the condition within the first 6 months of life, and more than 90% are reported to outgrow the problem by one year.

Acquired dacryocystitis

Acquired dacryocystitis may be acute or chronic. Acute dacryocystitis presents with painful, sometimes massive, enlargement of the lacrimal sac at or below the level of the medial canthal tendon and in the medial lower eyelid. If not treated, spontaneous rupture and drainage through the skin are common. A dacryocutaneous fistula may result. With proper therapy, the acute infections resolve in 7 to 14 days (Figure 291.1).

Chronic dacryocystitis, with or without dacryolith, is characterized by recurring episodes of epiphora or mucopurulent discharge, often but not always associated with a nontender fullness below the medial canthal tendon. Acute dacryocystitis may be superimposed on the chronic condition. Bloody tears may be a sign of lacrimal sac dacryolith or tumor.

Canaliculitis

Fungal canaliculitis is usually a unilateral disorder with thickening of the nasal eyelid and canthal erythema. It should be suspected in anyone with chronic unilateral nasal angle blepharoconjunctivitis that does not respond to typical antibiotic therapy. Compression of the canaliculus often will cause reflux onto the eye of a creamy pus containing ‘sulfur granules.’ Canaliculitis is found most commonly in the Midwest and other farming areas of the United States.

DIAGNOSIS

Infantile dacryocystitis

●Typical epiphora and discharge in the absence of conjunctival erythema are virtually pathognomonic.

●The fluorescein dye retention test will indicate poor lacrimal drainage in the involved eye.

●Digital pressure over the lacrimal sac may express mucopurulent material onto the globe.

Acquired dacryocystitis

Acute

●A typical clinical presentation is usually all that is needed for diagnosis.

FIGURE 291.1. Dacryocystitis.

Dacryolith291 CHAPTERand Dacryocystitis •

System Lacrimal • 25 SECTION

Lacerations and Contusions293 CHAPTERSystem Lacrimal •

System Lacrimal • 25 SECTION

canthal injuries with foreign bodies. Imaging with focus on extension into the anterior cranial fossa through the orbital roof should be obtained.

Clinical signs and symptoms

Epiphoria on the injured side and bleeding from the laceration site are the most common presenting signs and symptoms.

Differential diagnosis

Laceration of the periocular tissue without lacrimal system involvement.

PROPHYLAXIS

Following injury from a dog bite, the relationship of the animal to the victim and future risk should also be ascertained and appropriate steps taken to reduce the chance of subsequent injury as there is a significant risk of repeat attack.

TREATMENT

Systemic

In contaminated wounds from animal bites or vegetable matter, coverage for the most likely pathogens should be started immediately. Dogs habor a multitude of bacteria including p.multocidia and dysgenic fermenter 2. Antibiotic coverage should be broad spectrum. Frequently Keflex is used as a first choice. A tetanus booster should be given if it has been more than 5 years since the last booster in an immunized patient; tetanus immunoglobulin may be necessary in a non-immunized patient. Rabies prophylaxis is rarely needed in domestic pets but may need to be considered in wild animals and the immunization status of the animal should be noted.

Ocular

The status of the globe and visual function must first be determined. Any injury to the globe takes priority over the lacerated canaliculus or periocular tissue. Once the globe is determined to be stable, the injured eyelid tissue must be handled with delicate instruments and precision.

After carefully cleaning the tissue, the laceration should be replaced in its anatomic location. Rarely once the tissue is irrigated and replaced in its original location is there actual loss of lid tissue.

Medical

The wound should be gently irrigated with antibiotic solution such as 50,000 units Bacitracin in 500 cc normal saline. High pressures should be avoided as bacteria can be propelled further into the tissue. Depending on the degree of contamination both oral and topical antibiotics may be given for the first 10 days. An antibiotic-steroid ointment combination may help decrease both infection and inflammation. If the lacrimal drainage system has been repaired, an antibiotic-steroid drop should be given with slow taper over four weeks post-operatively.

Surgical

Numerous techniques exist to repair a lacerated canaliculus including bicanalicular stent placement through either a round eyed pigtail probe technique, or through stents placed through the nasal lacrimal duct, or a monocanalicular stent through a single disrupted canalicular system. The disrupted canaliculus

can be identified by the white cuff of tissue around the lumen of the canaliculus. An advantage to the pigtail probe technique is that the angle anatomy can be casted into position be securing the relationship through the lacrimal sac and opposing canaliculus. Passing the pigtail probe requires proper instruction in this technique as the probe must be carefully passed through the lacrimal system and gently guided through the medial canthal anatomy exiting through the proximal section the proximal segment and intact portion of the system can then be intubated with a 6-0 prolene suture. The probe can then be passed through the distal segment and this segment also intubated with the 6-0 prolene suture. A 24 mm piece of Crawford stent is then passed over the 6-0 prolene suture. Pericanalicular suture of 7-0 vicryl may be required to further oppose the severed ends of the canalicular system. At most 3 interrupted sutures are passed at the inferior, anterior and superior aspect of the pericanalicular tissue. If the medial canthal tendon is also severed, prior to securing these sutures, a 4-0 vicryl suture should be passed from the pretarsal orbicularis to the deep canthal periosteum to re-secure the posterior limb of the medial canthal tendon. The pericanalicular sutures can then be tied and the 6-0 prolene suture within the Crawford stent tied and the knot rotated into the intact segment of the lacrimal system.

COMPLICATIONS

Post-operative epiphoria or ectropion of the medial lower lid and or puncta are the most common complication encountered after these injuries.

COMMENTS

High speed or projectile injuries often allow for deep penetration of foreign bodies. Any trauma involving vegetable matter such as wood must be carefully evaluated to determine if any retained vegetable matter may be present. This will most commonly involve computed tomographic imaging and additional imaging with magnetic resonance imaging may be required. The retained wood must be removed as it will cause further soft tissue inflammation, infection and disruption of function. This type of trauma will sometimes cause fracture of the roof of the orbit as the foreign body penetrates the orbit and extends through the roof to the anterior cranial fossa. Neurosurgical consultation should be obtained.

REFERENCES

Burroughs J, Soparkar C, Patrinely J, et al: Periocular dog bite injuries and responsible care. Ophthal Plast Reconstr Surg 2002:18(6):416–419; discussion 419–420.

Herzum H, Holle P, Hintschich C. Eyelid injuries: epidemiological aspects. Ophthalmologe 98(11):1079–1082, 2001.

Jordan DR, Nerad JA, Tse DT: The pigtail probe, revisited. Ophthalmology 97(4):512–519, 1990.

Kersten RC, Kulwin DR. ‘One-stitch’ canalicular repair. A simplified approach for repair of canalicular laceration.Ophthalmology 103(5):785– 789, 1996.

Reifler DM: Management of canalicular laceration. Surv Ophthalmol 36:113–132, 1991.

Singer AJ, Thode HC, Hollander JE: National trends in ED lacerations between 1992–2002. Am J Emerg Med 24(2):183–188, 2006.

294 LACRIMAL GLAND TUMORS 224.2

Eric A. Steele, MD

Portland, Oregon

Roger A. Dailey, MD

Portland, Oregon

ETIOLOGY

Lacrimal gland tumors can arise from cells of epithelial origin, such as acinar or ductal elements, or from nonepithelial cells, such as inflammatory, neural, vascular, or fatty elements. More than half of all lacrimal gland tumors are nonepithelial in origin.

Epithelial tumors are evenly divided into benign and malignant lesions. The most common benign lesion is the benign mixed tumor (pleomorphic adenoma), whereas adenoid cystic carcinoma, adenocarcinoma, and malignant mixed cell tumor are typical epithelial malignancies. Of these, adenoid cystic carcinoma is the most common.

Nonepithelial tumors of the lacrimal gland include inflammatory and lymphoid lesions. Idiopathic orbital inflammation, which is also known as orbital pseudotumor, is the most common inflammatory process affecting the lacrimal gland. When the inflammation is limited to the lacrimal gland, this is also referred to as inflammatory dacryoadenitis. Lymphoid lesions occur along a disease spectrum from benign reactive lymphoid hyperplasia to malignant lymphoma.

COURSE/PROGNOSIS

●Benign mixed tumor presents with slowly progressive painless proptosis. The globe is displaced downward and medially. Prognosis is excellent if the tumor is completely excised with an intact capsule.

●Adenoid cystic carcinoma and adenocarcinoma are often associated with pain and rapid growth. Adenoid cystic carcinoma spreads via perineural invasion, whereas adenocarcinoma metastasizes to regional and distant lymph nodes. Both conditions have a poor prognosis.

●Malignant mixed tumor arises from benign mixed tumor that has been neglected or incompletely excised. The prognosis is poor.

●Idiopathic orbital inflammation typically presents acutely with pain, erythema, and tenderness. The lacrimal gland may be involved in isolation or in conjunction with other orbital structures.

●Lymphoid lesions tend to occur in older patients and to have a more insidious onset than does idiopathic orbital inflammation. Both benign and malignant diseases present with a slow onset of proptosis, downward displacement of the globe, ptosis, and, diplopia. A salmon-colored patch can be seen if conjunctival involvement is present. Reactive lymphoid hyperplasia has a good prognosis, although there can be malignant degeneration. Of patients with lacrimal gland lymphoma, 35% have or will develop systemic disease.

DIAGNOSIS

Laboratory findings

In addition to the history, radiologic studies play a key role in diagnosis. Computed tomography (CT) of the orbits is usually the preferred imaging modality since it provides excellent images of any associated bony changes.

●Benign epithelial lesions typically appear as a circumscribed mass with pressure remodeling but no destruction of the bony lacrimal fossa.

●Malignant epithelial lesions are typically ill defined, with lytic destruction of bone and intralesional calcification on CT scanning.

●Idiopathic orbital inflammation shows diffuse lacrimal gland enlargement, often with poor definition of gland margins due to inflammation in surrounding tissues. If a discrete mass is present, it typically conforms to the globe.

●Benign and malignant lymphoid lesions mold to the globe and other adjacent structures, without bony changes on CT. The margins often are quite well defined.

TREATMENT

Surgical

●Care must be taken to completely excise a benign mixed tumor, since residual tissue can degenerate into a malignant mixed tumor.

●The treatment of adenoid cystic carcinoma is controversial. Surgical options include wide local excision and orbital exenteration. Postoperative radiation therapy has been used with both surgical approaches. The prognosis has been poor regardless of the treatment modality, with few long-term survivors.

●Adenocarcinoma and malignant mixed tumor are also treated with tumor excision or orbital exenteration.

●Idiopathic orbital inflammation is treated initially with oral steroids and then with radiotherapy or antimetabolites if the patient becomes steroid dependent. Rapid improvement with steroid treatment supports the diagnosis of pseudotumor, although this must be interpreted cautiously because some orbital neoplasms transiently improve with steroid treatment. Atypical cases or lesions that do not respond to steroids may require incisional biopsy for diagnosis.

●Surgical treatment for lymphoid lesions is limited to incisional biopsy for diagnostic purposes. Reactive lymphoid hyperplasia is treated with low-dose radiation. A diagnosis of lymphoma warrants a systemic evaluation for extraorbital disease. If the disease is confined to the orbit, radiation is the treatment of choice. If extraorbital disease is present, chemotherapy, radiation, or both may be used.

REFERENCES

Kennerdell JS, Flores NE, Hartsock RJ: Low-dose radiotherapy for lymphoid lesions of the orbit and ocular adnexa. Ophthal Plast Reconstr Surg 15(2):129–133, 1999.

Tse DT, Neff AG, Onofrey CB: Recent developments in the evaluation and treatment of lacrimal gland tumors. Ophth Clin North Am 13 (4):663– 681, 2000.

294 TumorsCHAPTERGland Lacrimal •