L.M. Holbach

Parapapillary Atrophy: Histological Gamma Zone and Delta Zone

Background To examine histomorphometrically the parapapillary region in human eyes. Methodology/Principal Findings The histomorphometric study included 65 human globes (axial length:21–37 mm). On anterior-posterior histological sections, we measured the distance Bruchs membrane end (BME)-optic nerve margin (“Gamma zone”), BME-retinal pigment epithelium (RPE) (“Beta zone”), BME-beginning of non-occluded choriocapillaris, and BME-beginning of photoreceptor layer. “Delta zone” was defined as part of gamma zone in which blood vessels of at least 50 µm diameter were not present over a length of >300 µm. Beta zone (mean length:0.35±0.52 mm) was significantly (P = 0.01) larger in the glaucoma group than in the non-glaucomatous group. It was not significantly (P = 0.28) associated with axial length. Beta zone was significantly (P = 0.004) larger than the region with occluded choriocapillaris. Gamma zone (mean length:0.63±1.25 mm) was associated with axial length (P<0.001;r2 = 0.73) with an increase starting at an axial length of 26.5 mm. It was not significantly (P = 0.24) associated with glaucomatous optic neuropathy. Delta zone (present only in eyes with axial length of ≥27 mm) was associated with axial length (P = 0.001) and scleral flange length (P<0.001) but not with glaucoma (P = 0.73). Conclusions/Significance Parapapillary gamma zone (peripapillary sclera without overlying choroid, Bruchs membrane and deep retinal layers) was related with axial globe elongation and was independent of glaucoma. Delta zone (no blood vessels >50 µm diameter within gamma zone) was present only in highly axially elongated globes and was not related with glaucoma. Beta zone (Bruchs membrane without RPE) was correlated with glaucoma but not with globe elongation. Since the region with occluded choriocapillaris was smaller than beta zone, complete loss of RPE may have occurred before complete choriocapillaris closure.

Malignes Melanom des Tränensacks

A 68-year-old woman presented with a 10-month history of right-sided epiphora, bloody tears, and medial canthal mass. Computed tomography revealed a soft tissue mass of the right lacrimal sac with widening of the bony nasolacrimal canal. External dacryocystorhinostomy with incisional biopsy confirmed the diagnosis of malignant melanoma. After staging, further therapy included orbital exenteration, lateral rhinotomy with en bloc resection of the lacrimal drainage apparatus, and adjuvant radioimmunotherapy. One year after surgery, no evidence of local recurrence or metastatic disease could be detected.

Selektive Tränensackbiopsie bei externer Dakryozystorhinostomie

BACKGROUND Tumors of the lacrimal drainage system are rare but potentially life-threatening. The purpose of this study was to investigate the applicability of selective lacrimal sac biopsy in 500 cases of external dacryocystorhinostomy for acquired dacryostenosis. PATIENTS AND METHODS Lacrimal sac biopsy was obtained selectively for atypical clinical and/or intraoperative findings. The frequency and spectrum of biopsies were recorded and correlated with the follow-up results. RESULTS Lacrimal sac biopsy was performed in 19 external dacryocystorhinostomies (3.8%) including non-Hodgkin B-cell lymphoma (3), squamous carcinoma (2), mucoepidermoid carcinoma (1), malignant melanoma (1), oncocytoma (1), pyogenic granuloma (4), Wegeners granulomatosis (4) and sarcoidosis (3). Within 5 years none of the patients without biopsy developed systemic inflammatory diseases or lacrimal neoplasms. The 5-year survival was 87% in patients with significant lacrimal sac pathology and 96% in patients without significant pathology. CONCLUSION External dacryocystorhinostomy with selective biopsy for atypical clinical and intraoperative findings allows safe diagnosis and management of potentially life-threatening lacrimal lesions.

[Selective lacrimal sac biopsy for external dacryocystorhinostomy: a clinical pathological study].

BACKGROUND Tumors of the lacrimal drainage system are rare but potentially life-threatening. The purpose of this study was to investigate the applicability of selective lacrimal sac biopsy in 500 cases of external dacryocystorhinostomy for acquired dacryostenosis. PATIENTS AND METHODS Lacrimal sac biopsy was obtained selectively for atypical clinical and/or intraoperative findings. The frequency and spectrum of biopsies were recorded and correlated with the follow-up results. RESULTS Lacrimal sac biopsy was performed in 19 external dacryocystorhinostomies (3.8%) including non-Hodgkin B-cell lymphoma (3), squamous carcinoma (2), mucoepidermoid carcinoma (1), malignant melanoma (1), oncocytoma (1), pyogenic granuloma (4), Wegeners granulomatosis (4) and sarcoidosis (3). Within 5 years none of the patients without biopsy developed systemic inflammatory diseases or lacrimal neoplasms. The 5-year survival was 87% in patients with significant lacrimal sac pathology and 96% in patients without significant pathology. CONCLUSION External dacryocystorhinostomy with selective biopsy for atypical clinical and intraoperative findings allows safe diagnosis and management of potentially life-threatening lacrimal lesions.

Malignant melanoma of the lacrimal sac

A 68-year-old woman presented with a 10-month history of right-sided epiphora, bloody tears, and medial canthal mass. Computed tomography revealed a soft tissue mass of the right lacrimal sac with widening of the bony nasolacrimal canal. External dacryocystorhinostomy with incisional biopsy confirmed the diagnosis of malignant melanoma. After staging, further therapy included orbital exenteration, lateral rhinotomy with en bloc resection of the lacrimal drainage apparatus, and adjuvant radioimmunotherapy. One year after surgery, no evidence of local recurrence or metastatic disease could be detected.

Noduläre Fasziitis im Kindesalter im Bereich des Lids und der vorderen Orbita

An 8-year-old boy presented with a 6-week history of a rapidly progressive erythematous swelling of the right upper eyelid. Ultrasonography and magnetic resonance imaging revealed a subcutaneous nodular mass of the right upper eyelid medially with extension into the anterior orbit. The clinical differential diagnosis included rhabdomyosarcoma. A transcutaneous excisional biopsy was performed, and histopathologic examination confirmed the diagnosis of nodular fasciitis. Five years after surgery, there is no evidence of local recurrence, and the result is aesthetically satisfactory.

Micronodular thickening of eyelid margins. Initial findings of a general disease

A 9-year-old boy presented with bilateral chronic thickening of the upper and lower eyelid margins with bead-like papules. The voice was hoarse due to a previously diagnosed thickening of the vocal cords. There was a history of recurrent abscesses of the parotid gland and of attention deficit hyperactivity disorder (ADHD).The eyelid changes were recognized as monoliform blepharitis and the diagnosis of lipoid proteinosis (Urbach-Wiethe disease) was confirmed by eyelid biopsy. The diagnosis of this systemic disease explained the other signs and symptoms of the patient.ZusammenfassungEin 9-jähriger Junge wurde mit beidseitiger Verdickung und perlschnurartigen mikronodulären Veränderungen der Lidkanten vorgestellt. Die Stimme war heiser bei vorbeschriebenener Verdickung der Stimmlippen. Anamnestisch fanden sich Abszesse der Glandula parotis und ein Aufmerksamkeitsdefizit-Hyperaktivität-Syndrom (ADHS). Nach Einordnung des Lidbefunds konnte durch Biopsie und histopathologische Untersuchung eine Lipoidproteinose als systemische Grunderkrankung nachgewiesen werden. Die Veränderungen im HNO-Bereich und vermutlich auch die psychosozialen Auffälligkeiten können dadurch eingeordnet und verstanden werden.AbstractA 9-year-old boy presented with bilateral chronic thickening of the upper and lower eyelid margins with bead-like papules. The voice was hoarse due to a previously diagnosed thickening of the vocal cords. There was a history of recurrent abscesses of the parotid gland and of attention deficit hyperactivity disorder (ADHD).The eyelid changes were recognized as monoliform blepharitis and the diagnosis of lipoid proteinosis (Urbach-Wiethe disease) was confirmed by eyelid biopsy. The diagnosis of this systemic disease explained the other signs and symptoms of the patient.

[Nodular fasciitis of the eyelid and anterior orbit in children: case report and review of the literature].

An 8-year-old boy presented with a 6-week history of a rapidly progressive erythematous swelling of the right upper eyelid. Ultrasonography and magnetic resonance imaging revealed a subcutaneous nodular mass of the right upper eyelid medially with extension into the anterior orbit. The clinical differential diagnosis included rhabdomyosarcoma. A transcutaneous excisional biopsy was performed, and histopathologic examination confirmed the diagnosis of nodular fasciitis. Five years after surgery, there is no evidence of local recurrence, and the result is aesthetically satisfactory.

Mikronoduläre Verdickungen der Lidränder

A 9-year-old boy presented with bilateral chronic thickening of the upper and lower eyelid margins with bead-like papules. The voice was hoarse due to a previously diagnosed thickening of the vocal cords. There was a history of recurrent abscesses of the parotid gland and of attention deficit hyperactivity disorder (ADHD).The eyelid changes were recognized as monoliform blepharitis and the diagnosis of lipoid proteinosis (Urbach-Wiethe disease) was confirmed by eyelid biopsy. The diagnosis of this systemic disease explained the other signs and symptoms of the patient.ZusammenfassungEin 9-jähriger Junge wurde mit beidseitiger Verdickung und perlschnurartigen mikronodulären Veränderungen der Lidkanten vorgestellt. Die Stimme war heiser bei vorbeschriebenener Verdickung der Stimmlippen. Anamnestisch fanden sich Abszesse der Glandula parotis und ein Aufmerksamkeitsdefizit-Hyperaktivität-Syndrom (ADHS). Nach Einordnung des Lidbefunds konnte durch Biopsie und histopathologische Untersuchung eine Lipoidproteinose als systemische Grunderkrankung nachgewiesen werden. Die Veränderungen im HNO-Bereich und vermutlich auch die psychosozialen Auffälligkeiten können dadurch eingeordnet und verstanden werden.AbstractA 9-year-old boy presented with bilateral chronic thickening of the upper and lower eyelid margins with bead-like papules. The voice was hoarse due to a previously diagnosed thickening of the vocal cords. There was a history of recurrent abscesses of the parotid gland and of attention deficit hyperactivity disorder (ADHD).The eyelid changes were recognized as monoliform blepharitis and the diagnosis of lipoid proteinosis (Urbach-Wiethe disease) was confirmed by eyelid biopsy. The diagnosis of this systemic disease explained the other signs and symptoms of the patient.

Sympathische Ophthalmie nach zweimaliger Pars-plana-Vitrektomie

ZusammenfassungBei der sympathischen Ophthalmie handelt es sich um eine seltene bilaterale, granulomatöse Panuveitis, welche nach einem penetrierenden okulären Trauma auftreten kann. Bisher ist das Auftreten einer sympathischen Ophthalmie nach Vitrektomie nur vereinzelt in der Literatur beschrieben worden. Diese Kasuistik stellt eine Patientin mit einer sympathischen Ophthalmie nach zweimaliger Pars-plana-Vitrektomie anhand der klinischen Verlaufsbeobachtung, Fluoreszenzangiographie, Spectralis Domain OCT und Histopathologie vor.AbstractSympathetic ophthalmia is a rare form of bilateral granulomatous panuveitis, occurring after penetrating trauma. Hitherto, sympathetic ophthalmia after vitrectomy has only occasionally been described in the literature. This case report presents a female patient with sympathetic ophthalmia after repeated pars plana vitrectomy on the basis of clinical findings and follow-up with fluorescein angiography, spectral domain OCT, and histopathology.Sympathetic ophthalmia is a rare form of bilateral granulomatous panuveitis, occurring after penetrating trauma. Hitherto, sympathetic ophthalmia after vitrectomy has only occasionally been described in the literature. This case report presents a female patient with sympathetic ophthalmia after repeated pars plana vitrectomy on the basis of clinical findings and follow-up with fluorescein angiography, spectral domain OCT, and histopathology.