Studies of a pedigree with limbal dermoid cyst

AIM: To study clinical features and gene mutations within the paired-like homeodomain transcription factor 2 (PITX2) gene in a pedigree of bilateral limbal dermoids. METHODS: Complete eye examinations have been performed on each individual of the family. Exons of paired-like homeodomain transcription factor 2 ( PITX2)were amplified by polymerase chain reaction, sequenced, and compared with a reference database. RESULTS: We described the phenotype, clinic findings in a family with two affected members. The masses of the proband's eyes were excised surgically demonstrating a dermoid cyst by histopathological examination. No mutation was detected in the gene PITX2 in this pedigree. CONCLUSION: A family of limbal dermoid cyst was reported. In addition, no pathogenic sequence variations were found in PITX2, indicating that this phenotype in this family is a distinctive entity.

Dermoid Ovarian Cyst Complicated with Torsion in a Girl of 11 Years Old at the Teaching Hospital Sanou Souro of Bobo-Dioulasso (Burkina Faso): About an Observation and Review of the Literature

The dermoid ovarian is an organic mild tumor. It can be revealed by an adnexal torsion which constitutes a gynecological emergency that can bring into play in a short term after the functional prognosis. We are reporting an observed case in an 11-year-old teenager who is in times peri-pubertal. The patient was presenting a painful abdomino-pelvic syndrome and a pelvic mass at the physical examination. The ultrasound was showing a right cyst mass with a right moderated hydronephrosis. A right adnexectomy was realized. The histological test after excision of the adnexal was showing a mature cystic teratoma of the right ovary with a congestive and edematous tubal fragment.

Nasal Dermoid Cyst with Sinus Tract Intranasal Bone: A Case Report

Dermoid cysts are benign tumors originating from germ cells, which can form in various locations, including the nasal area in rare cases. They are of unknown exact etiology, but it is suggested that it is due to abnormal tissue migration during early embryonic development. Nasal dermoid cysts albeit rare, can present in various forms such as sinuses, fistulas, or intracranially extending tracts. They can be asymptomatic and incidentally discovered or present with a visible external mass or sinus that is either painful, infected or cosmetically concerning. If nasal dermoid cysts with an intra-nasal bone sinus tract are left untreated, they can lead to life-threatening complications. This report describes the case of a 6-year-old girl with a nasal dermoid cyst connected to a superficial punctum by an intra-nasal tract. She had undergone surgical excision of a nasal swelling previously diagnosed as a dermoid cyst. One year later, she returned to our clinic with a recurrence of the nasal swelling. Imaging tests revealed a nasal dermoid cyst with a tract extending to the nasal tip, without intracranial expansion. The cyst, along with the entire tract, was successfully removed surgically, and the postoperative follow-up indicated no complications. Histopathology confirmed the diagnosis of a dermoid cyst. This case underscores the significance of considering the dermoid tract in nasal cyst cases and the necessity of its complete removal to prevent recurrence.

Intra osseous dermoid cyst of mandible—A rare case report

The present article report a rare case of intraosseous dermoid cyst in mandible which clinically and radio-graphicaly presented as mandibular cyst. The diagnosis of dermoid cyst was confirmed histologically. A 20 year old patient was admitted to our Department with the complaint of swelling in left lower jaw with occasional pain. Radiographic examination revealed a radiolucent lesion extending from left 2nd molar to right premolar region. On surgical exploration the lesion was cystic in nature full of hair and keratinized tissues. The tissue was sent for the histopathological examination and macroscopic feature reveal numerous bits of soft tissue. The tissues were creamish brown in color, firm in consistency, with largest tissue measuring 2.5 × 1.5 × 0.5 cm in dimension. Few hair follicles were also evident within it. The histopathology reveal cyst lined by stratified squamous hyperor- thokeratinized epithelium supported by a fibrous connective tissue wall. The cystic epithelium is showing melanin pigmentation in the basal cell layer. Ab-undant onion skin keratin is seen within the cyst lumen. The epithelial lining as well as cyst wall shows numerous skin appendages such as lobules of seba-ceous glands. The underlying connective tissue shows mild chronic inflammatory infiltrate mainly composed of lymphocytes.

Dermoid cyst in a domestic shorthair cat

A S-year-old neutered male domestic shorthair cat was presented for examination of a subcutaneous mass in his tail.The mass was firm,non-painful,oval,and approximately 2.5×3.5 cm. Surgical exploration revealed a well-circumscribed,encapsulated mass.The mass was removed and sectioned for histopathological examination.In gross section,it was filled with numerous dark hairs.Histologically the mass was consisted of haired skin with dermal cystic structures lined by stratified squamous epithelium.The cyst lumen contained squamous debris and filled with keratinous material.Numerous hair shafts were extended from the wall of the cyst.The sebaceous and apocrine gland adnexal structures were also observed which confirmed the diagnosis of dermoid cyst.No tumor recurrence was observed after surgery in fallowing checkups.Cutaneous or subcutaneous cysts of all types are considered rare in cats and to our knowledge this is the third reported case of cutaneous dermoid cyst of cats in veterinary literature which is different from the other cases because it occurred in dorsal midline in tail area whereas other's occurred in flank area.

Rupture of a Posterior Fossa Dermoid Cyst Overlying the Torcular with Extracranial Extension: Technical Note

Intracranial dermoid cysts are congenital benign neoplasms mostly diagnosed in the pediatric?hood and usually involve the midline structures. They count approximately less than 1% of all intracranial neoplasms and are believed to arise from ectopic cell rests incorporated in the closing neural tube. These dermoid cysts, especially those involving the posterior fossa and overlying the torcular, are uncommon. We report perhaps the first case of this entity in a third-year-old boy and discuss the physiopathogenesis, the imaging features and the best technical note to manage this cyst in this location.

Challanging Management of a Giant Sublingual Dermoid Cyst Rapidly Enlarged Throughout Pregnancy and Influence of Hormonal Factors

The authors present a case with a sublingual giant dermoid cyst, rapidly enlarged during pregnancy, which showed itself with deglutition and mild respiratory problems. Dermoid cysts (DCs) are benign lesions arising from entrapment of epithelial rests during embryogenesis. They are most frequently located in the ovaries and testes. Up to 6.9% are found in the head and neck and 1.6% within the oral cavity, representing less than 0.01% of all oral cavity cysts. They usually present early in life as asymptomatic masses with slow and progressive growth. However, they may reach a large size with a potential impact on deglutition, speech, and respiration. It is suggested that the rapid growth of DCs during pregnancy is associated with the increase of plasma levels of estrogens and progesterone. In order to eliminate respiratory and deglutition issues, it was decided to perform the surgical excision after a cesarean delivery at 29th week.

Two sequential surgeries in infant with multiple floor of the mouth dermoid cysts: A case report

BACKGROUND Multiple intraoral dermoid cysts of large magnitude generally appear in the second or third decade of life.They are rare in infants and are usually solitary.In this case,a large mass was identified in utero during prenatal exams.CASE SUMMARY We introduce a rare case on multiple dermoid cysts in the floor of the mouth of an infant who underwent two surgeries for this.Preoperative magnetic resonance imaging confirmed a large well-circumscribed cystic lesion that originated at the former midline region in the floor of the mouth in which a suspicious lesion of minute size was likely compressed by the bulkier mass and overlooked.Therefore,the infant underwent two surgeries by an intraoral approach within 9 mo.At 5 mo after the second operation,a routine follow-up ultrasound showed evidence of an additional cyst.No further surgery was planned because the tumor had no immediate effect.CONCLUSION This report demonstrates the importance of carefully analyzing preoperative imaging to avoid multiple operations for a seemingly isolated oral cyst.

Asymptomatic traumatic rupture of an intracranial dermoid cyst:A case report

BACKGROUND Previous studies reported that most of the intracranial dermoid cyst ruptures were spontaneous,and only a few were traumatic,with asymptomatic much rarer than the symptomatic ruptures.Hence,how to deal with the asymptomatic traumatic rupture of intracranial dermoid cyst remains a challenge in the clinic.CASE SUMMARY A 59-year-old man was accidentally diagnosed with intracranial dermoid cyst through a cranial computed tomography(CT)scan due to a car accident.A mixed-density lesion with fat and a calcified margin was observed in the midline of the posterior fossa,accompanied with lipid droplet drifts in brain sulci,fissures,cisterns,and ventricles.After 1 wk of conservative observation,no change was observed on the updated cranial CT scan.After 2 wk of conservative observation,magnetic resonance imaging examination confirmed that the lesion was a traumatic rupture of a posterior fossa dermoid cyst with lipid droplet drifts.As the patient exhibited no adverse symptoms throughout the 2 wk,a 6-mo follow-up visit was arranged for him instead of aggressive treatment.Nonetheless,the patient did not show any abnormal neurological symptoms in the 6 mo of follow-up visits.CONCLUSION Asymptomatic traumatic rupture of intracranial dermoid cyst could be just followed or treated conservatively rather than treated aggressively.

An Intragaleal Frontal Dermoid Cyst: A Location Never Reported

All dermoid cysts arising at the cranial vault had been reported as being located subgaleally, i.e. under the galea aponeurotica or epicranium. We are presenting a case that we believe to be the first case of an intragaleal dermoid cyst ever reported. An eighteen years old girl presented at our consultation for a midline frontal swelling evolving since 2 years. Neurological examination was normal. The Computed Tomography (CT) scan showed a round shaped heterogeneous mass at the vertex with no intracranial extension. The lesion was excised surgically. It appeared to be located intragaleally (within the epicranium) and composed of hairs and liquefied fat evocative of a dermoid cyst. The diagnosis of dermoid cyst was confirmed on histology. Postoperative outcome was excellent and the patient is symptom-free 16 months after surgery.

Congenital Frontal Mature Dermoid Cyst: A Case Report

Dermoid cysts are uncommon congenital or acquired developmental cystic malformations that were usually seen in the midline of frontal region and the anterior fontanel. Congenital forms develop from the remnants of the epithelium or ectodermal membranous bone growth line. We report here a case of a congenital frontal dermoid cyst that should be considered in the differential diagnosis of lipoma and neurofibroma. Dermoid cysts should be considered in the differential diagnosis of all nodular cyst-like lesions in the head and neck region. Early recognition and accurate diagnosis by means of Computerized Tomography scan or Magnetic Resonance Imaging facilitate successful treatment.

A Girl with Life Threatening Meningitis Caused by an Infected Dermoid Cyst Derived from an Occult Dermal Sinus

Congenital dermal sinuses are a rare entity of spinal dysraphism. Most patients have cutaneous markers of their presence, including nevus or port wine stains, dimple, hypertrichosis and subcutaneous lipoma. Inclusion tumors, such as dermoid tumor, are associated to dermal sinus tract;they are congenital, benign and slow-growing lesions of midline. Their clinical relevance is due to the possibility of causing infection, spinal abscess, signs of corde or nerve root compression. Spinal Magnetic Resonance Imaging (MRI) is the diagnostic tool of choice. We present the case of a child with atypical meningitis, resistant to large spectrum antibiotic therapy, caused by an infected dermoid cyst, undiagnosed in the first months of life despite repeated MRI.

An Extremely Rare Case Report of Foramen Magnum Dermoid Cyst Presenting as an Acute Progressive Neurologic Deficit

Intracranial dermoid cysts are nonneoplastic lesions that present a rare clinical entity. They account for 0.04% to 0.6% of all intracranial tumors. Such cystic lesions usually contain different ectodermal derivatives including hairs, sebaceous glands, apocrine gland and teeth. Dermoid cysts of foramen magnum occur very rarely, with few cases reported in the literature. Computed tomography (CT) and magnetic resonance imaging (MRI) are two main diagnostic tools. Here in this paper, we described a case of intracranial (foramen magnum) dermoid cyst presented with neurologic deficits that completely removed surgically.

Nasal Dermoid Cyst: A Case Report

Nasal dermoid sinus cysts (NDSCs) are rare neoplasms in the median line of the nasofrontal area. Unlike other dermoid cysts, a NDSC can manifest as a cyst, sinus, or fistula, and may extend intracranially. Nasal dermoid cysts usually present at birth and are commonly diagnosed by 3 years of age. An incidentally detected nasal dermoid cyst in an adult patient is extremely rare. In this case, a 23-year-old female with a history of intermittent serous thick discharge from a pit in the nasal columella since early childhood. Examination of the nasal cavity showed mild septal deviation to the left side, dorsal hump, wide nasal bone, a small pit-sinus at the columella, over projected nose, and wide bulbous down rotated nasal tip. The findings were suggesting nasal dermoid cyst, so a magnetic resonance imaging (MRI) was done for the patient to confirm the diagnosis. The MRI findings showed that there are 3 small cysts at the nasal septum anteriorly with a small fistula connecting the anterior inferior 2 cysts with no involvement to the intracranial cerebrospinal fluid (CSF) space. During the surgery, an open rhinoplasty approach was used. The trans-columellar incision was made around the fistula, and the tract was identified using a lacrimal probe and methylene blue. The dissection around the tract was continued until the sac was identified in the area of the membranous septum, and it was removed and sent for histopathology. The surgery then continued in a regular full rhinoplasty fashion. In conclusion, dermoid cyst of the nasal septum is a rare congenital lesion, especially in adults. After a thorough history taking and physical examination, a preoperative high-resolution axial and coronal CT and/or MRI is essential for diagnostic and operative planning. The treatment of choice for nasal dermoid cysts is complete surgical excision with clear margins. Surgical strategies usually depend on the location and extent of the lesion. Recurrence is uncommon and often easily managed.

Dermoid Cysts of the Conus Medullaris Associated with Sacral Meningocele. A Case Report

Spinal dermoid cysts are gradually progressive benign tumors that may be congenital or acquired. They account for 0.8% to 1.1% of all primary spinal tumors. MRI is the gold standard for the radiological assessment. Management is difficult for tumors involving highly eloquent areas such as the conus medullaris. In this report, we present a rare case of an adult woman with dermoid cysts in the conus medullaris associated with a sacral meningocele which was treated with a midline myelotomy that drained yellowish keratinous fluid and decompressed the cyst. No aggressive attempt at complete resection of the cyst wall was undertaken. The patient fully recovered, and her condition considerably improved after surgery and remains good at a 6-month follow-up.

Clinicopathological analysis of 719 pediatric and adolescents’ ocular tumors and tumor-like lesions:a retrospective study from 2000 to 2018 in China

AIM:To describe the clinicopathologic features and classification of pediatric and adolescent ocular tumors and tumor-like lesionsMETHODS:A total of 719 cases of pathologically confirmed ocular tumors and tumor-like lesions in a pediatric population from two academic institutions over an 18-year period were retrospectively analyzed.The main outcome measures were the clinical and pathological features of the cases.RESULTS:Benign tumors accounted for 92.1%of all cases while malignant tumors accounted for 7.9%.The most common ocular benign tumors were(epi-)dermoid cysts(19.8%),nevi(15.2%),corneal dermoid tumors(9.8%),and calcified epitheliomas(8.8%).The most common ocular malignant tumors were retinoblastoma(80.8%),and rhabdomyosarcoma(3.9%).Eyelid and ocular surface tumors comprised 73.3%of benign tumors while intraocular and orbital cavity comprised 94.2%of malignant tumors.For tumor site,the upper eyelid was up to 1.79 times more than lower eyelid(P<0.05).Age at surgery and sex also had an association with different lesions(P=0.006,P=0.035,respectively).CONCLUSION:Most ocular tumors and tumor-like lesions in children and adolescents are benign.Pediatric ocular tumors are distinct from those in adults in terms of histological origin.(Epi-)dermoid cysts are the most common benign tumors while retinoblastomas the most common malignant tumors.

Review on 209 Congenital Cysts of the Floor of the Mouth

目的:比较1980～2005年在武汉大学口腔医院治疗的89位中国病人和在约旦医院治疗的120位病人三型口底囊肿的临床特点.方法:回顾性分析皮样囊肿、表皮样囊肿和畸胎样囊肿患者的年龄、性别、种族,以及比较两组患者的囊肿位置、大小、就诊时间、手术治疗方法和复发率等方面的差别.结果:209例口底囊肿中,93例(44.5%)男性,116例(55.5%)女性.皮样囊肿66例(31.6%)、表皮样囊肿115例(55%)、畸胎样囊肿28例(13.4%).皮样囊肿、表皮样囊肿多见于头3个10岁年龄组,20～29岁高发,而畸胎样囊肿高好发于10岁以前.约90%的患者无自觉症状.发病后6个月以内就诊者,第1组(43.8%)明显少于第2组(80%).这些先天性囊肿常发生在口底中部.就诊时第1组中囊肿直径大于3 cm 者达48.3%,而第2组中囊肿直径大于3 cm 者仅11.7%.第1组中经口外进路切除病变占58.4%,而第2组为41.7%.141例随访病例的术后复发25例,复发率是17.7%.结论:两组口底囊肿有类似的临床特点,其中表皮样囊肿较常见,畸胎样囊肿的发病年龄较小.手术切除不彻底可导致病变复发.

一例法斗犬眼角膜皮样囊肿切除及结膜瓣遮盖术

角膜皮样囊肿是一种长在人或动物的角膜、结膜、角巩膜等处被覆毛发的异常皮肤样组织。在人和动物均可见,动物中尤其是在牛和犬中较为常见。角膜皮样囊肿可以引发眼睑痉挛、眼分泌物增加、结膜炎、角膜炎和角膜溃疡等,严重者可导致角膜穿孔和眼球坏死。通过角膜切除术或结膜切除术,切除角膜皮样囊肿并对症治疗的方法来治疗眼部角膜皮样囊肿。以下是在2022年3月接诊的一例幼年法斗犬患有右侧眼角膜皮样囊肿,对患犬实施浅层角膜切除术切除右侧角膜皮样囊肿并用结膜瓣遮盖的手术方法进行治疗,术后恢复良好。

椎管内皮样囊肿破裂的MRI诊断(附13例报告)

目的 探讨椎管内皮样囊肿破裂 (RDIC)的MRI诊断价值。材料与方法 13例RDIC原发灶均经手术和病理证实 ,皮样囊肿均位于T12 以下椎管内 ,其中 9例位于脊髓内 ,4例位于髓外硬膜下。均行脑和全脊柱MRISE序列T1WI、T2 WI ,7例有脊柱脂肪抑制序列。 5例行脑CT检查。结果 4例髓外硬膜下皮样囊肿破裂MRI与CT均表现在脑池、脑沟或脑室内有脂肪滴。 9例脊髓内皮样囊肿破裂者均见脊髓中央管内分布有脂肪滴 ,其中 4例脑室内见脂肪滴。 5例脑MRI发现脑底及后颅窝脂肪滴者 ,CT仅显示 2例。脂肪滴MRISE序列T1WI呈显著高信号 ,T2 WI其信号略有衰减 ,脂肪抑制序列则呈极低信号 ;CT上脂肪滴则呈低密度 ,CT值 -2 0～ -15 0HU。结论 MRI是诊断RDIC的最佳影像学方法 ,其显示RDIC脂肪滴在脑室、蛛网膜下腔和脊髓中央管内分布优于CT。CT与MRI联合应用能有效地鉴别RDIC脂肪滴抑或积气、碘化油滞留和血块。

儿童眶周皮样囊肿114例临床分析

目的:探讨儿童眶周皮样囊肿的临床特点、彩色多普勒超声及CT影像学表现和诊疗经验。方法:回顾性分析114例经手术后病理组织学证实的眶周皮样囊肿患者的临床资料。所有患者均行彩色多普勒超声检查,部分患者行CT检查。根据肿物的部位设计不同的手术切口。结果:选取114例患儿中,右眼61例,左眼53例。肿物位于眶颞上缘68例(59.6%),眶上缘27例(23.7%),眶内上缘13例(11.4%),眶内下缘5例(4.4%),眶下缘1例(0.9%)。肿块与骨膜粘连紧密者49例。彩色多普勒检查示肿物边界清楚100例(87.7%),低回声者105例(92.1%),回声均匀者61例(53.5%),伴有液性暗区者4例,囊肿内部无血彩者107例(93.9%)。行CT检查者40例中,CT值范围-55~32Hu。肿物周边骨壁破坏7例。术中根据肿物的部位行不同的手术切口。眉弓切口72例,上睑双重睑切口10例,囊肿部位顺皮肤方向皮肤切口30例,穹隆切口2例。所有患者术后随访3mo^4a,均无明显手术并发症,无复发。结论:联合彩色多普勒及CT检查,可术前对儿童眶周皮样囊肿进行准确定性、定位诊断。巧妙利用肿块位置设计手术切口,完整摘除囊壁是达到术后更好的美容效果,避免复发的关键。