The Journal of Craniofacial Surgery
& Volume 25, Number 4, July 2014
Bilateral Lambdoid Dermoid Cyst Hwan Jun Choi, MD, PhD Abstract: Generally, dermoid and epidermoid cysts arise as a result of incomplete separation of the neuroectoderm from the superficial ectoderm with displacement of ectodermal elements along their lines of fusion during development. Dermoid cysts of the posterior head are rare lesions composed of epidermal and mesodermal elements. Furthermore, dermoid cysts in the occipital area are extremely rare. The presence of synchronous, bilateral occipital dermoids is quite rare and has not been reported previously. A unique case of lambdoidal dermoids in a 75-year-old woman is presented. Large right lesion was excised concurrently through direct skin incision, and a very small left mass was observed. And the etiology, natural history, management, and prognosis of dermoids are reviewed.
Brief Clinical Studies
scan was acquired because of the unusual presentation of bilateral posterior scalp masses. The CT scan confirmed that the subcutaneous masses were not in communication with related structures and, specifically, that there was no intracranial involvement. Upon exploration with the patient in a right semilateral position under local anesthesia, the elliptical skin was excised. The fatty tumor had well-defined cleavage planes and could be cleanly separated from the scalp centrally. Toward the peripheral, the tumor became more well defined and, although densely adherent to the periosteum and right lambdoidal suture, did not appear to invade it. A high-speed bur was then used to remove the adjacent bone and lambdoid suture, and the cyst was extracted without violating the inner table. This tumor was found just under the skin and was well encapsulated, so it was completely removed with adjacent periosteum. Histologically, specimen contained desquamated squamous epithelium and keratin in the lumen and was encapsulated and lined by keratinized stratified squamous epithelium and the presence of adnexal structures (Fig. 1). Her postoperative course was uncomplicated, and her incisions have healed well with an acceptable scar. Follow-up has been maintained for 2 years, and she has not shown any evidence of recurrence or untoward effects.
Key Words: Dermoid cyst, dermal sinus, bilateral, epidermoid, scalp
C
ongenital dermoid cysts develop during the fusion of the embryo when the ectodermal tissue gets trapped in the line of fusion.1 The natural history of dermoids is that of a slow, expanding lesion with possible acute inflammatory episodes and a mass effect on adjacent soft-tissue and bony structures. Only a few cases of dermoid cysts in the posterior scalp have been reported. Especially, a bilateral, synchronous presentation in this location has not been reported previously in the literatures. The following clinical report describes a unique presentation of bilateral occipital dermoids that were excised via direct skin incision. A brief review of the pertinent literature with reference to the etiology, diagnosis, and management of dermoid cysts is summarized.
CLINICAL REPORT In a 75-year-old woman undergoing, under local anesthesia, excision of a mass located in both occipital regions. Right-sided mass presented with a large (6 7 4 cm), soft, rubbery mass covering the posterior aspect of the skull. Left-sided mass presented with a small (1 1.5 1 cm), asymptomatic mass. The remainder of the head and neck examination was within normal limits. Because of the unsatisfactory appearance and sleeping discomfort of the right mass, the patient’s concern about further growth, and the potential for malignant change, the mass was excised. Right-sided case had a gradually enlarging mass of the posterolateral aspect of the scalp. But, incidental left-sided mass had a small size and no subjective symptoms, so it was observed. Both cysts were mobile, noncompressible, and nontender, without evidence of an associated sinus tract, skin dimpling, discoloration, or communication with adjacent structures. Enhanced computed tomographic (CT)
From the Department of Plastic and Reconstructive Surgery, College of Medicine, Soonchunhyang University, Cheonan, Korea. Received October 16, 2013. Accepted for publication November 14, 2013. Address correspondence and reprint requests to Dr. Hwan Jun Choi, Department of Plastic & Reconstructive Surgery, College of Medicine, Soonchunhyang University Hospital, 23-20, Bongmyung-Dong, Cheonan, 330-721, Korea; E-mail: [email protected] This work was supported by the Soonchunhyang University Research Fund. The author reports no conflicts of interest. Copyright * 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000585
DISCUSSION Dermoid cysts are thought to form when epidermal and dermal cells are trapped during neural tube closure. Dermal sinuses and cysts constitute abnormal communications between the skin and the underlying tissues.2 They show variable depths and can extend to the subcutaneous tissue, bone, extradural or subdural space, or to nervous tissue; in the latter case, those found in the posterior fossa may extend to the cerebellum or even into intravenous ventricle, ending either blindly or communicating with a dermoid or epidermoid cyst.2 It is generally accepted that dermoid cysts can be classified as either superficial or deep.3 Superficial dermoids manifest in a typical manner as a painless, firm, partially mobile subcutaneous mass.3 Although 10% to 45% of nasoglabellar dermoids present with sinus tracts and occasional intracranial extension, it is rare for frontotemporal dermoids to do so.4 Frontotemporal dermoids typically manifest as superficial, slow-growing masses that are treated by simple excision.4 If bony invasion exists, aggressive exposure and resection of at least the outer cranial table are necessary to prevent recurrence.4 Dermal sinuses are defects of closing of the ectoderm; they may extend deeply, through defects in tissue of mesodermal origin: skin, aponeurosis, bone, and dura mater.5 These defects are produced between the third and fifth week of intrauterine life; those cases in the posterior fossa may be produced by a failure of the anterior neuropore to close toward the end of the fourth week of gestation.5 The final route of these dermal sinuses and the definite location of their external orifice (posterior fossa, inion, supratentorial) depend on the relative movements between the nervous
FIGURE 1. A 75-year-old woman had cystic masses in both sides of occipital area. A, Preoperative finding of the dermoid cyst of the right occipital area. About 6 3.5 7-cm round and oval cystic mass seen in the right posteroinferior aspect of the head. B, Preoperative enhanced CT (bone setting). Showing cystic mass and mild bony erosion of both sides of the occipital bone. C, Intraoperative finding shows removed mass and periosteum. D, Excised mass. E, Histological finding of the mass. Cystic wall is lined by squamous epithelium and keratin materials. Squamous epithelium, sebaceous gland, hair follicle, and sweat gland are seen (hematoxylin-eosin stain, original magnification 100).
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
e321
The Journal of Craniofacial Surgery
Brief Clinical Studies
system and its cutaneous and osseous coverings, which take place after the closure of the ectoderm.5 Deep dermoids sinuses in the occipital region are often identified in children by recurrent bouts of meningitis or acute abscess formation.2 They may be also become apparent as a result of signs of increased intracranial pressure.2 When such a presentation occurs, a careful search should be made in the occipital area for a mass, telangiectasia, altered hair growth, or a pinpoint sinus.2 The CT scan may show the dermal sinus, the associated dermoid, or epidermoid cyst and reveal occasionally the bone defect if the level of the cut coincides with it.5 These cysts can easily be confused with other types of tumors or even with an abscess, with which it is frequently associated.5 Appropriate diagnosis requires not only an index of suspicion for this rare tumor but also a very careful history and search for skin changes. Especially, enhanced CT scan reveals the exact location of the cyst and its relationship with adjacent structures. Furthermore, if bony invasion is seen, aggressive exposure and resection of at least the outer table of the cranium are necessary to prevent recurrence. In addition, lambdoid dermoids represent a distinct entity from midline occipital dermoids, which require a separate classification. Also, the authors think that occipital dermoids divide into superficial and deep type. In this case, because they did not have intracranial involvement or fistula formation, they are the superficial type.
REFERENCES 1. Li WY, Reinisch JF. Cysts, pits, and tumors. Plast Reconstr Surg 2009;124S:106eY116e 2. Wiemer DR. An occipital dermoid tumor and sinus. Ann Plast Surg 1988;21:465Y467 3. Elahi MM, Glat PM. Bilateral frontozygomatic dermoid cysts. Ann Plast Surg 2003;51:509Y512 4. Lacey M, Gear AJL, Lee A. Temporal dermoids: three cases and a modified treatment algorithm. Ann Plast Surg 2003;51:103Y109 5. Schijman E, Monges J, Cragnaz R. Congenital dermal sinuses, dermoid and epidermoid cysts of the posterior fossa. Childs Nerv Syst 1986;2:83Y89
A Patient With Branch Retinal Vein Occlusion Accompanied by Superior Ophthalmic Vein Thrombosis Due to Severe Superior Ophthalmic Vein Enlargement in a Patient With Graves Ophthalmopathy Han Seok Park, MD, Hyo Jeong Gye, MD, Joon Mo Kim, MD, PhD, Young Ji Lee, MD Abstract: We report a patient with branch retinal vein occlusion (BRVO) accompanied by superior ophthalmic vein (SOV) thrombosis resulting from severe SOV enlargement in a patient with Graves ophthalmopathy (GO). A 78-year-old woman diagnosed with BRVO presented with reduced vision. She had a more than 20-year history of unilateral GO. Fluorescein angiography and spectralis optical coherence tomography revealed BRVO with cystoid macular edema. Approximately
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& Volume 25, Number 4, July 2014
5 months later, she complained of advanced left proptosis. A computed tomography scan at that time revealed dilated SOV with high internal attenuation due to SOV thrombosis of the left orbit. Because of the uncertain onset of SOV thrombosis and minimal signs of acute orbital congestion, we prescribed prophylactic daily aspirin to improve blood flow rather than a heparin-based treatment. In addition, she was treated with lubricants for exposure keratopathy and a dorzolamide/timolol eyedrops to prevent increases in intraocular pressure. This is the first clinical report of BRVO accompanied by SOV thrombosis due to SOV enlargement in chronic severe GO. It should be recognized that SOV thrombosis can lead to an increase in proptosis even in chronic GO. Key Words: Graves ophthalmopathy, branch retinal vein occlusion, superior ophthalmic vein enlargement, superior ophthalmic vein thrombosis
B
ranch retinal vein occlusion (BRVO) is considered to be one of the most common types of retinal vein occlusion, and it commonly leads to cystoid macular edema, a sight-threatening complication. It is thought that mechanical vein compression due to arteriovenous crossings, degenerative changes within venous walls, and hypercoagulability may cause this condition in a patient with various predisposing cardiovascular, thrombophilic, systemic, and ocular conditions correlated with the development of BRVO.1 We report a case of BRVO accompanied by superior ophthalmic vein (SOV) thrombosis due to severe SOV enlargement in a patient with chronic unilateral Graves ophthalmopathy (GO) and provide a discussion of pathophysiologic mechanisms of this condition.
CLINICAL REPORT A 78-year-old woman with unilateral GO visited our clinic for evaluation of diabetic retinopathy. She had more than 20-year history of Graves disease as well as hypertension and diabetes mellitus for the previous 10 years. She underwent cataract surgery on her left eye 5 years before presentation. Her best-corrected visual acuity (BCVA) was 20/25 OD and 20/30 OS. Her intraocular pressure (IOP) was 16 mm Hg OD and 20 mm Hg OS. Her left eye appeared to be protruding, and a difference of 6 mm between both eyes was measured using a Hertel exophthalmometer. There was no definite limitation of ocular motion or diplopia. Fundus examination showed flame-shaped retinal hemorrhages along the superior branch retinal arcade of the left eye (Fig. 1). After 1 week, her BCVA was reduced from 20/30 to 10/30 OS. On fluorescein angiography, there was no evidence of microaneurysms in either eye, but blocked fluorescence was seen due to retinal hemorrhage
From the Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea. Received October 17, 2013. Accepted for publication November 14, 2013. Address correspondence and reprint requests to Young Ji Lee, MD, Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, 108 Pyung-Dong, Jongno-Gu, Seoul 110-746, Republic of Korea; E-mail: [email protected] The authors report no conflicts of interest. Copyright * 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000586
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
& Volume 25, Number 4, July 2014
Bilateral Lambdoid Dermoid Cyst Hwan Jun Choi, MD, PhD Abstract: Generally, dermoid and epidermoid cysts arise as a result of incomplete separation of the neuroectoderm from the superficial ectoderm with displacement of ectodermal elements along their lines of fusion during development. Dermoid cysts of the posterior head are rare lesions composed of epidermal and mesodermal elements. Furthermore, dermoid cysts in the occipital area are extremely rare. The presence of synchronous, bilateral occipital dermoids is quite rare and has not been reported previously. A unique case of lambdoidal dermoids in a 75-year-old woman is presented. Large right lesion was excised concurrently through direct skin incision, and a very small left mass was observed. And the etiology, natural history, management, and prognosis of dermoids are reviewed.
Brief Clinical Studies
scan was acquired because of the unusual presentation of bilateral posterior scalp masses. The CT scan confirmed that the subcutaneous masses were not in communication with related structures and, specifically, that there was no intracranial involvement. Upon exploration with the patient in a right semilateral position under local anesthesia, the elliptical skin was excised. The fatty tumor had well-defined cleavage planes and could be cleanly separated from the scalp centrally. Toward the peripheral, the tumor became more well defined and, although densely adherent to the periosteum and right lambdoidal suture, did not appear to invade it. A high-speed bur was then used to remove the adjacent bone and lambdoid suture, and the cyst was extracted without violating the inner table. This tumor was found just under the skin and was well encapsulated, so it was completely removed with adjacent periosteum. Histologically, specimen contained desquamated squamous epithelium and keratin in the lumen and was encapsulated and lined by keratinized stratified squamous epithelium and the presence of adnexal structures (Fig. 1). Her postoperative course was uncomplicated, and her incisions have healed well with an acceptable scar. Follow-up has been maintained for 2 years, and she has not shown any evidence of recurrence or untoward effects.
Key Words: Dermoid cyst, dermal sinus, bilateral, epidermoid, scalp
C
ongenital dermoid cysts develop during the fusion of the embryo when the ectodermal tissue gets trapped in the line of fusion.1 The natural history of dermoids is that of a slow, expanding lesion with possible acute inflammatory episodes and a mass effect on adjacent soft-tissue and bony structures. Only a few cases of dermoid cysts in the posterior scalp have been reported. Especially, a bilateral, synchronous presentation in this location has not been reported previously in the literatures. The following clinical report describes a unique presentation of bilateral occipital dermoids that were excised via direct skin incision. A brief review of the pertinent literature with reference to the etiology, diagnosis, and management of dermoid cysts is summarized.
CLINICAL REPORT In a 75-year-old woman undergoing, under local anesthesia, excision of a mass located in both occipital regions. Right-sided mass presented with a large (6 7 4 cm), soft, rubbery mass covering the posterior aspect of the skull. Left-sided mass presented with a small (1 1.5 1 cm), asymptomatic mass. The remainder of the head and neck examination was within normal limits. Because of the unsatisfactory appearance and sleeping discomfort of the right mass, the patient’s concern about further growth, and the potential for malignant change, the mass was excised. Right-sided case had a gradually enlarging mass of the posterolateral aspect of the scalp. But, incidental left-sided mass had a small size and no subjective symptoms, so it was observed. Both cysts were mobile, noncompressible, and nontender, without evidence of an associated sinus tract, skin dimpling, discoloration, or communication with adjacent structures. Enhanced computed tomographic (CT)
From the Department of Plastic and Reconstructive Surgery, College of Medicine, Soonchunhyang University, Cheonan, Korea. Received October 16, 2013. Accepted for publication November 14, 2013. Address correspondence and reprint requests to Dr. Hwan Jun Choi, Department of Plastic & Reconstructive Surgery, College of Medicine, Soonchunhyang University Hospital, 23-20, Bongmyung-Dong, Cheonan, 330-721, Korea; E-mail: [email protected] This work was supported by the Soonchunhyang University Research Fund. The author reports no conflicts of interest. Copyright * 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000585
DISCUSSION Dermoid cysts are thought to form when epidermal and dermal cells are trapped during neural tube closure. Dermal sinuses and cysts constitute abnormal communications between the skin and the underlying tissues.2 They show variable depths and can extend to the subcutaneous tissue, bone, extradural or subdural space, or to nervous tissue; in the latter case, those found in the posterior fossa may extend to the cerebellum or even into intravenous ventricle, ending either blindly or communicating with a dermoid or epidermoid cyst.2 It is generally accepted that dermoid cysts can be classified as either superficial or deep.3 Superficial dermoids manifest in a typical manner as a painless, firm, partially mobile subcutaneous mass.3 Although 10% to 45% of nasoglabellar dermoids present with sinus tracts and occasional intracranial extension, it is rare for frontotemporal dermoids to do so.4 Frontotemporal dermoids typically manifest as superficial, slow-growing masses that are treated by simple excision.4 If bony invasion exists, aggressive exposure and resection of at least the outer cranial table are necessary to prevent recurrence.4 Dermal sinuses are defects of closing of the ectoderm; they may extend deeply, through defects in tissue of mesodermal origin: skin, aponeurosis, bone, and dura mater.5 These defects are produced between the third and fifth week of intrauterine life; those cases in the posterior fossa may be produced by a failure of the anterior neuropore to close toward the end of the fourth week of gestation.5 The final route of these dermal sinuses and the definite location of their external orifice (posterior fossa, inion, supratentorial) depend on the relative movements between the nervous
FIGURE 1. A 75-year-old woman had cystic masses in both sides of occipital area. A, Preoperative finding of the dermoid cyst of the right occipital area. About 6 3.5 7-cm round and oval cystic mass seen in the right posteroinferior aspect of the head. B, Preoperative enhanced CT (bone setting). Showing cystic mass and mild bony erosion of both sides of the occipital bone. C, Intraoperative finding shows removed mass and periosteum. D, Excised mass. E, Histological finding of the mass. Cystic wall is lined by squamous epithelium and keratin materials. Squamous epithelium, sebaceous gland, hair follicle, and sweat gland are seen (hematoxylin-eosin stain, original magnification 100).
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
e321
The Journal of Craniofacial Surgery
Brief Clinical Studies
system and its cutaneous and osseous coverings, which take place after the closure of the ectoderm.5 Deep dermoids sinuses in the occipital region are often identified in children by recurrent bouts of meningitis or acute abscess formation.2 They may be also become apparent as a result of signs of increased intracranial pressure.2 When such a presentation occurs, a careful search should be made in the occipital area for a mass, telangiectasia, altered hair growth, or a pinpoint sinus.2 The CT scan may show the dermal sinus, the associated dermoid, or epidermoid cyst and reveal occasionally the bone defect if the level of the cut coincides with it.5 These cysts can easily be confused with other types of tumors or even with an abscess, with which it is frequently associated.5 Appropriate diagnosis requires not only an index of suspicion for this rare tumor but also a very careful history and search for skin changes. Especially, enhanced CT scan reveals the exact location of the cyst and its relationship with adjacent structures. Furthermore, if bony invasion is seen, aggressive exposure and resection of at least the outer table of the cranium are necessary to prevent recurrence. In addition, lambdoid dermoids represent a distinct entity from midline occipital dermoids, which require a separate classification. Also, the authors think that occipital dermoids divide into superficial and deep type. In this case, because they did not have intracranial involvement or fistula formation, they are the superficial type.
REFERENCES 1. Li WY, Reinisch JF. Cysts, pits, and tumors. Plast Reconstr Surg 2009;124S:106eY116e 2. Wiemer DR. An occipital dermoid tumor and sinus. Ann Plast Surg 1988;21:465Y467 3. Elahi MM, Glat PM. Bilateral frontozygomatic dermoid cysts. Ann Plast Surg 2003;51:509Y512 4. Lacey M, Gear AJL, Lee A. Temporal dermoids: three cases and a modified treatment algorithm. Ann Plast Surg 2003;51:103Y109 5. Schijman E, Monges J, Cragnaz R. Congenital dermal sinuses, dermoid and epidermoid cysts of the posterior fossa. Childs Nerv Syst 1986;2:83Y89
A Patient With Branch Retinal Vein Occlusion Accompanied by Superior Ophthalmic Vein Thrombosis Due to Severe Superior Ophthalmic Vein Enlargement in a Patient With Graves Ophthalmopathy Han Seok Park, MD, Hyo Jeong Gye, MD, Joon Mo Kim, MD, PhD, Young Ji Lee, MD Abstract: We report a patient with branch retinal vein occlusion (BRVO) accompanied by superior ophthalmic vein (SOV) thrombosis resulting from severe SOV enlargement in a patient with Graves ophthalmopathy (GO). A 78-year-old woman diagnosed with BRVO presented with reduced vision. She had a more than 20-year history of unilateral GO. Fluorescein angiography and spectralis optical coherence tomography revealed BRVO with cystoid macular edema. Approximately
e322
& Volume 25, Number 4, July 2014
5 months later, she complained of advanced left proptosis. A computed tomography scan at that time revealed dilated SOV with high internal attenuation due to SOV thrombosis of the left orbit. Because of the uncertain onset of SOV thrombosis and minimal signs of acute orbital congestion, we prescribed prophylactic daily aspirin to improve blood flow rather than a heparin-based treatment. In addition, she was treated with lubricants for exposure keratopathy and a dorzolamide/timolol eyedrops to prevent increases in intraocular pressure. This is the first clinical report of BRVO accompanied by SOV thrombosis due to SOV enlargement in chronic severe GO. It should be recognized that SOV thrombosis can lead to an increase in proptosis even in chronic GO. Key Words: Graves ophthalmopathy, branch retinal vein occlusion, superior ophthalmic vein enlargement, superior ophthalmic vein thrombosis
B
ranch retinal vein occlusion (BRVO) is considered to be one of the most common types of retinal vein occlusion, and it commonly leads to cystoid macular edema, a sight-threatening complication. It is thought that mechanical vein compression due to arteriovenous crossings, degenerative changes within venous walls, and hypercoagulability may cause this condition in a patient with various predisposing cardiovascular, thrombophilic, systemic, and ocular conditions correlated with the development of BRVO.1 We report a case of BRVO accompanied by superior ophthalmic vein (SOV) thrombosis due to severe SOV enlargement in a patient with chronic unilateral Graves ophthalmopathy (GO) and provide a discussion of pathophysiologic mechanisms of this condition.
CLINICAL REPORT A 78-year-old woman with unilateral GO visited our clinic for evaluation of diabetic retinopathy. She had more than 20-year history of Graves disease as well as hypertension and diabetes mellitus for the previous 10 years. She underwent cataract surgery on her left eye 5 years before presentation. Her best-corrected visual acuity (BCVA) was 20/25 OD and 20/30 OS. Her intraocular pressure (IOP) was 16 mm Hg OD and 20 mm Hg OS. Her left eye appeared to be protruding, and a difference of 6 mm between both eyes was measured using a Hertel exophthalmometer. There was no definite limitation of ocular motion or diplopia. Fundus examination showed flame-shaped retinal hemorrhages along the superior branch retinal arcade of the left eye (Fig. 1). After 1 week, her BCVA was reduced from 20/30 to 10/30 OS. On fluorescein angiography, there was no evidence of microaneurysms in either eye, but blocked fluorescence was seen due to retinal hemorrhage
From the Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea. Received October 17, 2013. Accepted for publication November 14, 2013. Address correspondence and reprint requests to Young Ji Lee, MD, Department of Ophthalmology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, 108 Pyung-Dong, Jongno-Gu, Seoul 110-746, Republic of Korea; E-mail: [email protected] The authors report no conflicts of interest. Copyright * 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000586
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
