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Paolo Nucci¹, Carlo de Conciliis², Matteo Sacchi¹, Massimiliano Serafino¹¹Eye Clinic, San Giuseppe Hospital, University of Milan, ²Eye Clinic, Istituto Auxologico Italiano, Milan, ItalyBackground: We present the first case of a congenital form of silent brain syndrome (SBS) in a young patient affected by Hallermann–Streiff syndrome (HSS) and the surgical management of the associated eyelid anomalies.Methods: HSS signs were evaluated according to the Francois criteria. Orbital computed tomography (CT) and genetic analysis were performed. An upper eyelid retractor-free recession was performed. Follow-up visits were performed at day 1, weeks 1 and 3, and months 3, 6, 9 (for both eyes), and 12 (for left eye) after surgery.Results: The patient exhibited six of the seven signs of HSS. Orbital CT showed bilateral enophthalmos and upward bowing of the orbital roof with air entrapment under the upper eyelid as previously described for SBS. Genetic analysis showed a 2q polymorphism. During follow-up, the cornea showed absence of epithelial damage and the upper eyelids were lowered symmetrically, with a regular contour.Conclusion: Our HSS patient shares features with SBS. We postulate that SBS could include more than one pattern, ie, an acquired form following ventriculoperitoneal shunting and this newly reported congenital form in our HSS patient in whom typical syndromic skull anomalies led to this condition. The surgical treatment has been effective in restoring an appropriate lid level, with good globe apposition and a good cosmetic result.Keywords: Hallermann–Streiff syndrome, silent brain syndrome, upper eyelid entropion

Background: An association between ocular adnexal MALT lymphoma (OAL) and Chlamydia psittaci (Cp) infection has been proposed, and recent reports suggest that doxycycline treatment causes tumor regression in patients with Cp-related OAL. The effectiveness of doxycycline treatment in Cp-negative OAL has not been tested., Methods: In a prospective trial, 27 OAL patients (15 newly diagnosed and 12 having experienced relapse) were given a 3-week course of doxycycline therapy. Objective lymphoma response was assessed by computerized tomography scans or magnetic resonance imaging at 1, 3, and 6 months after the conclusion of therapy and every 6 months during follow-up. Cp infection in patients was determined by touchdown enzyme time-release polymerase chain reaction (TETR-PCR). Statistical tests were two-sided., Results: Eleven patients were Cp DNA-positive and 16 were Cp DNA negative. Doxycycline was well tolerated. At a median follow-up of 14 months, lymphoma regression was complete in six patients, and a partial response (> or = 50% reduction of all measurable lesions) was observed in seven patients (overall response rate [complete and partial responses] = 48%). Lymphoma regression was observed in both Cp DNA-positive patients (seven of 11 experienced regression) and Cp DNA-negative patients (six of 16 experienced regression) (64% versus 38%; P = .25, Fisher's exact test). The three patients with regional lymphadenopathies and three of the five patients with bilateral disease achieved objective response. In relapsed patients, response was observed both in previously irradiated and nonirradiated patients. The 2-year failure-free survival rate among the doxycycline-treated patients was 66% (95% confidence interval = 54 to 78), and 20 of the 27 patients were progression free., Conclusions: Doxycycline is a fast, safe, and active therapy for Cp DNA-positive OAL that was effective even in patients with multiple failures involving previously irradiated areas or regional lymphadenopathies. The responses observed in PCR-negative OAL may suggest a need for development of more sensitive methods for Cp detection and investigation of the potential role of other doxycycline-sensitive bacteria.

Purpose: To describe a minimally invasive technique for conjunctivodacryocystorhinostomy with the Jones tube. This technique creates a direct communication between the conjunctiva and the middle meatus with the use of a 14-gauge angiocatheter. The glass tube is inserted under endoscopic or direct visualization., Methods: A retrospective review of consecutive patients who underwent the minimally invasive technique for conjunctivodacryocystorhinostomy for complete bicanalicular lacrimal obstruction was performed. The surgical time, intraoperative and postoperative complications, length of the tubes, long-term patency, tube displacement, and need for secondary revision were evaluated., Results: Fifty-five consecutive patients were included in the study. All surgical procedures were successfully performed without significant complications, in an operating time that averaged 16 minutes. In one early case, a patient had persistent postoperative bleeding that required cauterization of the middle turbinate. In 3 patients, late migration of the Jones tube into the nasal cavity required secondary intervention with successful Jones tube repositioning. Minor office tube cleaning was performed without removal of the tube. The patency of the Jones tube was regularly tested with demonstration of aspiration of 2% fluorescein solution from the tear meniscus in the tear lake opening of the tube at the slit lamp, the passage of the same solution in the nose with endoscopic view, and finally, with irrigation of saline solution in the tube., Conclusions: The minimally invasive technique for conjunctivodacryocystorhinostomy with the Jones tube can be successfully performed with a simple "poke-through" technique from the conjunctiva to the nose with direct or endoscopic control. This technique has proved to be time-effective and well tolerated by patients.

Background and Objective: Levator aponeurosis advancement is an effective technique that is routinely used to correct aponeurogenic ptosis. The standard technique involve a skin incision of the upper eyelid crease for the entire length of the eyelid, with or without associated blepharoplasty. We believe that, in a selected group of patients, a less invasive approach with an upper lid skin incision of only 0.8 cm is equally effective for the final result and offers several advantages compared to the traditional technique., Materials and Methods: We retrospectively reviewed the data of 48 patients affected by involutional ptosis with good levator function that underwent unilateral or bilateral levator advancement ptosis repair through a mini-invasive approach. Final outcome measures included postoperative eyelid height, contour, symmetry, periocular edema, surgical time and visibility of the incision site., Results: The mini-invasive approach for the correction of involutional ptosis resulted in our hands as effective and reliable as the traditional technique, required a shorter surgical time, offered an improved early post-operative course with minimal bruising and swelling and produced no visible scar., Conclusions: This mini-invasive ptosis correction technique replaced in our practice the traditional approach for the treatment of a selected subset of patients affected by aponeurogenic ptosis.

A 57-year-old white man presented with extensive bilateral, symmetric, confluent papules involving the upper and lower eyelids, causing visual impairment and cosmetic deformity. Surgical debulking of the papules was initially performed, but the lesion rapidly recurred and enlarged. Histopathologic examination revealed cutaneous amyloidosis. Six months later, extensive excision of the upper eyelid lesions was required to restore normal visual function.

We report the clinical features and surgical treatment of a 15-year-old girl with unilateral microblepharon. The anomaly was characterized by a vertical shortage of upper and lower eyelid skin, causing nocturnal lagophthalmos, corneal exposure, and cosmetic deformity. Treatment consisted in hard-palate grafting and lateral tarsal strip suspension of the lower eyelid of the affected side. The outcome was considered satisfactory by the surgeon and by the patient. No further surgery was required.

Purpose: To evaluate the appearance of the skin incision in external dacryocystorhinostomy 6 weeks and 6 months after surgery., Methods: A prospective, interventional, noncomparative case series of consecutive cases of external dacryocystorhinostomy was performed by 3 surgeons. At 6 weeks and 6 months after surgery, patients were asked to grade their incision, and standardized photographs were evaluated by 3 blinded observers., Results: Thirty-four consecutive patients were admitted and followed for 6 months. Six weeks after surgery, 9 of 34 patients could not see their incision site (26%), 13 of 34 graded it as minimally visible (38%), 9 of 34 (26%) graded it as moderately visible, and 3 of 34 patients (9%) graded it as very visible (grade 3). Two of 34 patients (6%) were not satisfied with the appearance of the incision. Six months after surgery, 15 of 34 patients (44%) could not see their incision site (grade 0), 16 of 34 (47%) graded it as minimally visible, 3 of 34 patients (9%) graded it as moderately visible, and no patient graded it as very visible. All patients were satisfied with the appearance of their incision. Photographic evaluation of patients 6 weeks after surgery by the 3 observers showed an average score of 1.12, 1.18, and 1.24. There was not a statistically significant difference between the observers (p = 0.95). At 6 months after surgery, the average scores were 0.56, 0.74, and 0.79. There was not a statistically significant difference between the observers (p = 0.43). The change in appearance of the incision at 6 weeks and at 6 months was statistically significant (p < 0.044), as evaluated by patients and observers (p < 0.001)., Conclusions: The skin incision in external dacryocystorhinostomy is satisfactory to most patients. Its appearance is improved with time; 86% of the incisions were graded invisible or minimally visible by observers and 91% by patients after 6 months.

Background: Ocular adnexal lymphomas may be antigen-driven disorders; however, the source of the putative antigen or antigens is still unknown. Hence, we assessed whether Chlamydiae infection is associated with the development of ocular adnexal lymphomas., Methods: The presence of Chlamydia psittaci, trachomatis, and pneumoniae DNA was investigated by polymerase chain reaction in 40 ocular adnexal lymphoma samples, 20 nonneoplastic orbital biopsies, 26 reactive lymphadenopathy samples, and peripheral blood mononuclear cells (PBMCs) from 21 lymphoma patients and 38 healthy individuals. Seven patients with chlamydia-positive PBMCs were treated with the antibiotic doxycycline, and objective response was assessed in four patients with measurable lymphoma lesions. Differences in Chlamydiae DNA detection between the case patients and the control subjects were analyzed using the Fisher exact test. All statistical tests were two-sided., Results: Thirty-two of the 40 (80%) ocular adnexal lymphoma samples carried C. psittaci DNA, whereas all lymphoma samples were negative for C. trachomatis and C. pneumoniae. In contrast, none of the 20 nonneoplastic orbital biopsies (0% versus 80%; P<.001) and only three of 26 (12%) reactive lymphadenopathy samples (12% versus 80%; P<.001) carried the C. psittaci DNA. Nine of 21 (43%) patients with chlamydia-positive lymphomas carried C. psittaci DNA in their PBMCs, whereas none (0%) of the healthy PBMC donors carried C. psittaci DNA in their PBMCs (43% versus 0%; P<.001). One month after doxycycline treatment, chlamydial DNA was no longer detectable in the PBMCs of all seven treated patients, and objective response was observed in two of the four evaluable patients., Conclusion: Patients with ocular adnexal lymphoma had a high prevalence of C. psittaci infection in both tumor tissue and PBMCs. Persistent C. psittaci infection may contribute to the development of these lymphomas, as was also supported by the clinical responses observed in this study with C. psittaci-eradicating antibiotic therapy.

Purpose: The real incidence of solitary fibrous tumor (SFT) of the orbit is unknown, but it seems that since it was first described in 1994, orbital SFT has been increasingly recognized. We believe that the orbital SFT is a relatively common tumor and that it should be considered in the differential diagnosis of any orbital tumor., Design: Interventional case series., Participants: Four new cases of orbital SFT., Methods: Four patients affected by solitary fibrous tumor of the orbit are described. One patient experienced a recurrent SFT shortly after initial surgical excision performed elsewhere. Thirty-eight cases have been reported in the literature in 7 years., Results: The number of orbital SFTs reported has been increasing, reaching an average of more than five tumors reported per year. Since the first orbital SFT was described in 1994, 37 cases have been reported in the literature. We add four new cases in our series, including a recurrent tumor. A total of 42 cases have now been described, eight with recurrences. Malignant transformation occurred in one case., Conclusions: We believe that before 1994, the diagnosis orbital SFT was confused with other benign orbital tumors, such as fibrous histiocytoma and hemangiopericytoma because of a lack of use of immunohistochemical techniques. This entity should now be considered relatively common and should be included in the differential diagnosis of orbital tumors in any age group. Local recurrences of SFT are possible and usually follow an incomplete initial excision. Recurrent tumors in the orbit have shown the tendency to infiltrate the surrounding tissues and the bone, rendering complete secondary excision more difficult. Recurrent orbital SFT also has the potential for malignant transformation. The treatment of choice of orbital SFT is complete surgical excision and careful follow-up. Considering the more aggressive course followed by recurrent tumor, correct diagnosis and management is essential.

The charts of 10 patients affected by myogenic ptosis who underwent surgical correction by means of a frontalis suspension sling using a silicone rod were reviewed. The patients included in the study were affected by ptosis secondary to myasthenia gravis (MG), chronic progressive external ophthalmoplegia (CPEO) or mitochondrial myopathy (MM). In every patient the ptosis was severe (MRD( 1) < 2 mm), with the eyelid partially or totally occluding the visual axis; levator function was poor (<5 mm), Bell's phenomenon was poor or absent and the orbicularis function was reduced. Final eyelid height, patient satisfaction and the presence of complications were our main outcome measures. Analysis of the results showed that the ptosis was corrected in every patient with a clear visual axis. One patient with absent Bell's and poor levator function had exposure keratopathy resistant to medical treatment and required surgical revision. We believe that the frontalis suspension sling is safe, effective and is the procedure of choice for patients affected by poor-function acquired ptosis. A silicone rod, because of its elasticity, is the material of choice in this selected category of patients.

A total of 145 patients with orbital dermoid cysts examined at the Orbital Clinic, Institute of Ophthalmology, School of Medicine, University of Naples "Federico II" over a period of 16 years were reviewed. The orbital cysts were classified as exophytic and endophytic, according to their site of attachment in relation to the orbital rims. This classification can explain the different natural history of these lesions. The exophytic cysts growing externally are discovered in childhood, whereas the endophytic ones are discovered later in life when they produce bone damage, with or without invasion of the adjacent structures.

Purpose: The authors studied the etiology of acquired ptosis in the young to middle-aged adult population with specific attention to the role of rigid contact lens use., Methods: The study consisted of all patients between the ages of 15 and 50 years with acquired ptosis who presented between April 1986 and May 1994. Potential factors responsible for acquired ptosis were investigated in all patients with specific attention directed to history and duration of contact lens wear., Results: In the consecutive series of 91 young to middle-aged adults with acquired ptosis, we found contact lens wear to be the only identifiable cause in 47% of patients. This was the most common cause for acquired ptosis in this age group. Trauma was a distant second cause, accounting for 19% of patients. Of the contact lens-induced ptosis, 58% were unilateral and 42% were bilateral. Of the 25 patients who wore contact lenses and had unilateral ptosis on examination, manual elevation of the ptotic lid showed an unsuspected contralateral ptosis to be manifest in seven patients due to Hering's law. Ptosis was overwhelmingly associated with rigid contact lens wear, and levator aponeurosis disinsertion was found in the large majority at the time of surgical repair., Conclusion: This study suggests that contact lens-induced ptosis is a much more common cause of acquired ptosis in young and middle-aged adults than has been suspected previously. The ptosis is primarily due to levator aponeurotic disinsertion, presumably due to recurrent traction on the aponeurosis during rigid contact lens removal.