A case of recurrent, grade 3 corneal choriostoma after surgical excision.

We report a case of congenital grade 3 corneal choriostoma accompanied by anterior staphyloma. The tumor was excised by deep lamellar dissection, and the defect was filled with multilayer amniotic membranes. On histologic examination, the excised tumor contained demislike connective tissues, dermal adnexa (hair follicles and sebaceous gland), and adipose tissue, and was lined by keratinizing squamous epithelium. One month after surgery, the tumor recurred and progressively enlarged.

Conservative Surgical Management of a Grade III Corneal Dermoid.

PURPOSE: To present a rare, unique, grade III corneal dermoid treated with conservative surgical management. METHODS: An 8-month-old boy was admitted to our clinic with a lesion on the central cornea of the right eye and a corneal mass in the left eye. The mass had a skin-like surface and protruded to the outside of the palpebral fissure. MRI (magnetic resonance imaging) of the orbits showed a large cyst covering most of the cornea and the absence of the anterior chamber and lens. The dermoid was excised and sent for histopathological examination. The cornea was reconstructed using the partial thickness scleral graft. RESULTS: The histopathology report confirmed the diagnosis of a grade III corneal dermoid. On examination 1 year after the surgery, the orbital and globe volumes were the same, and the intraocular pressure was normal. CONCLUSIONS: This is likely the first report of an unusual case in which a grade III corneal dermoid with an anterior staphyloma was treated by anterior segment reconstruction using an autologous partial thickness scleral graft. We believe that this technique has the advantage that it stimulates orbital and facial development and has good cosmetic results.

[Developmental malformations of the cerebral cortex]. / Heterotopie, Polymikrogyrie, Lissenzephalie und Co - Malformationen der kortikalen Hirnentwicklung.

Migration disorders (MD) are increasingly recognized as an important cause of epilepsy and developmental delay. Up to 25 % of children with refractory epilepsy have a cortical malformation. MD encompass a wide spectrum with underlying genetic etiologies and clinical manifestations. Research regarding the delineation of the genetic and molecular basis of these disorders has provided greater insight into the pathogenesis of not only the malformation but also the process involved in normal cortical development. Diagnosis of MD is important since patients who fail three antiepileptic medications are less likely to have their seizures controlled with additional trials of medications and therefore epilepsy surgery should be considered. Recent improvements in neuroimaging have resulted in a significant increase in the recognition of MD. Findings can be subdivided in disorders due to abnormal neurogenesis, neuronal migration, neuronal migration arrest and neuronal organization resulting in different malformations like microcephaly, lissencephaly, schizencephaly and heterotopia. The examination protocol should include T 1-w and T 2-w sequences in adequate slice orientation. T 1-w turbo-inversion recovery sequences (TIR) can be helpful to diagnose heterotopia. Contrast agent is needed only to exclude other differential diagnoses.

Heterotopic gastric mucosa of the esophagus: literature-review and proposal of a clinicopathologic classification.

The prevalence of heterotopic gastric mucosa (HGM) in the cervical esophagus is frequently underestimated. Tiny microscopic foci have to be distinguished from a macroscopically visible patch, also called "inlet patch." Symptoms as well as morphologic changes associated with HGM are regarded as a result of the damaging effect of acid, produced by parietal cells in the mostly fundic type of HGM. We herein review the literature and propose a new clinicopathologic classification of esophageal HGM: Most of the carriers of esophageal HGM are asymptomatic (HGM I). Some individuals with HGM in the esophagus complain of dysphagia, odynophagia, or "extraesophageal manifestations" (hoarseness and coughing), without further morphologic findings (HGM II). Still fewer patients are symptomatic due to morphologic changes, i.e., esophageal strictures, webs, or esophagotracheal fistula (HGM III). Malignant transformation via dysplasia (intraepithelial neoplasia, HGM IV) to cervical esophageal adenocarcinoma (HGM V) is exceedingly rare (only 24 reported cases). In contrast to Barrett's esophagus, HGM should not be regarded as a precancerous lesion. Symptoms are more likely to occur in patients with inlet patch, whereas malignant transformation and adenocarcinogenesis can also occur in microscopic HGM foci. Asymptomatic HGM requires neither specific therapy nor endoscopic surveillance. Only in symptomatic cases treatment, i.e., dilatation for (benign) strictures or acid suppression for reflux symptoms, can be recommended. Patients with low-grade dysplasia in HGM might be candidates for surveillance strategies, whereas in cases of high-grade dysplasia and invasive adenocarcinoma oncological treatment strategies must be employed.

Quiste meníngeo intraorbitario asociado a tejido cerebeloso heterotópico / Cystic meningeal intraorbitary association tissue cerebrose heterotopy

Presentamos el caso de una niña de 14 meses con colomboma coroideo bilateral y proptosis paraaxial progresiva del ojo izquierdo, en la que se halló un quiste meníngeo en la órbita izquierda con tejido cerebeloso ectópico. La lesión se resolvió hallar otros dos casos similares en la literatura, describiendo tejido cerebeloso ectópico en la órbita

True ectopic ovary: a case and review.

INTRODUCTION: Ectopic ovarian tissue is a rare gynecologic problem. The nomenclature and classification systems are unclear and still debated. CASE REPORT: Herein we provide a case report and review the literature to help clarify the classification system of ectopic ovarian tissue.

[Complete supernumerary breast on the thigh in a male patient]. / Sein surnuméraire complet de la cuisse chez un homme.

BACKGROUND: Supernumerary nipples are not rare but the developement of a complete supernumerary breast is exceptional. CASE REPORT: A 59-year-old man presented a progressively increasing ancient right- groin-masse. The histopathologic examination of the lesion confirmed the diagnostic of polymastia. DISCUSSION: The interest of this observation results from the very unusual occurence of polymastia (less than 1 p. 100 of supernumerary nipples), especially for a caucasian man. The prevalence appears to be higher in women and oriental people. Diagnostic of supernumerary nipple is difficult because of its atypical appearance and ectopic location. However, this diagnostic is important because ectopic breast tissue is subject to the same pathologic changes that occur in normally positioned breasts and it can be a marker for associated diseases such as urologic malformations or urogenital malignancies.

Endoscopic ultrasonographic classification of gastric aberrant pancreas.

BACKGROUND: Gastric aberrant pancreas (GAP) can be demonstrated by its characteristic patterns on endoscopic ultrasonography (EUS). We tried to classify the EUS figures of GAP to provide a useful EUS pattern for diagnosing this disease. METHODS: Among 68 cases of GAP suspected by EUS, 13 patients who were proven to have GAP by histology from surgical specimens (4 cases), mucosectomy (6 cases), and biopsy (3 cases) were enrolled. EUS was performed under a standard procedure using an Olympus EU-M3 machine with a 7.5/12 MHz inter-changeable transducer. The essential histological finding of GAP is pancreatic tissue in specimens. RESULTS: Among 13 patients with GAP (size from 0.7 to 1.6 cm in diameter), 11 tumors were located in the antrum, and 2 were located at the lower body. All 13 tumors arose from the third layer of the gastric wall with an echogenicity higher than the fourth hypoechoic layer. We classified EUS figures of these 13 GAP into 3 types based on the change of the muscular (fourth) layer below the tumor: Type 1 (6 cases), the fourth layer is intact and has a normal thickness; Type 2 (4 cases), the fourth layer has thickened; and Type 3 (3 cases), the fourth layer has thickened, and there are some hyperechoic densities (tubular-like structures) within the fourth layer. Mucosectomy was performed without any complication in 6 cases (four type 1 and two type 2). CONCLUSION: EUS figures of our 13 cases of GAP were classified into 3 types which will be helpful in the EUS diagnosis of GAP and can provide information on selecting patients for mucosectomy.

Neuronal migration disorders in man.

A classification of neuronal migration disorders based on the gross morphology of the disorder with subclassifications according to the cause of the underlying disorder as far as that is known is presented. Emphasis is on disorders of the neocortex. The classical entities classical lissencephaly, cobblestone lissencephaly, polymicrogyria, neuronal heteropia within the white matter and leptomeningeal glial and glioneuronal heterotopia are described. Recent findings on the genetics of neuronal migration disorders are discussed. More subtle structural abnormalities such as cortical dysgenesis or dysplasia remain still more controversial as separate entities.

Retroperitoneal ectopic neural mass: "abdominal brain"--presentation of two cases and proposal of classification of paraneuraxial neural ectopia.

An encapsulated mass of brain tissue was found in the retroperitoneum of a fetus of gestational week 15 and a boy of age 3 years. The masses possessed fibrous tissue that bound them to the spine and intraspinal connective tissue, respectively, but there was no evidence of direct continuity of the ectopic brain tissue with the normal central nervous system. There was no dysraphism. In our fetal case, possible Foix-Alajouanine anomaly was additionally found. The ectopic neural tissue in the retroperitoneal region may be termed "abdominal brain." In the literature, an identical state has been described in the head (paracranial region) but there are no other records of the paraspinal region. Despite the different locations of the masses (head/paracranial or retroperitoneum/paraspinal), these ectopic brain masses should belong to the same disorder spectrum of the paraneuraxial neural ectopia, a new concept.

[Lipoma, multiple lipomas and lipomatosis]. / Lipomes, lipomes multiples et lipomatoses.

Lipomatous tumors are very frequent; simple lipoma is the most common variety. According to Enzinger, lipomatous tumors are classified into five different groups: simple lipomas, variant lipomas forms, heterotopic lipomas, infiltrating lipomas and lipomatosis, and finally hibernomas. Usually, lipomatous tumors are characterized by a slow, unpainful, growth. Classical treatment includes surgical resection. The clinical diagnosis is confirmed by histology. Liposucion can be a therapeutic option in certain cases.

Supernumerary breast tissue: a case of pseudomamma on the face.

Supernumerary breast tissue usually develops along the milk line. But occasionally, it may be seen outside the milk line. In this article, the authors describe a case of pseudomamma on the face, one of the rarest locations for supernumerary breast tissue. The lesion was removed for cosmetic reasons. There was no associated anomaly.

Tumores disontogénicos del globo ocular y sus anexos. II. Coristomas / Disontogenic tumors of the eye and ocular anexa. II. Coristomas

Los coristomas se dividen en simples y complejos. Los coristomas simples más frecuentes son: Dermoides sólidos y quísticos, quistes epidérmicos, osteoma epibulbar, osteoma coroideo, glándula lagrimal aberrante y coristoma facomatoso. Los coristomas complejos varían dependiendo de la diversificación de sus elementos constitutivos, se incluye en este grupo como entidad individual al glioneuroma. Se hace una revisión del tema y se presentan las imágenes clínico-patológicas más sobresalientes

Tumores disontogénicos del globo ocular y sus anexos. I. Introducción, aspectos básicos y hamartomas / Disontogenic tumors of the eye and ocular anexa. Introduction, basic principles and hamartomas

Los tumores disontogénicos son lesiones proliferativas que se originan como un trastorno del desarrollo e incluyen una variedad de neoformaciones: Hamartomas, coristomas y el complejo grupo de los teratomas. La mayoría de estas lesiones son benignas con excepción de los teratomas. Son entidades algunas de ellas frecuentes en la clínica y otras son excepcionales. El tratamiento es quirúrgico y el pronóstico es bueno en la mayoría de los casos. Es un grupo de lesiones poco conocidas en la clínica oftalmológica. Se hace una revisión completa del tema en tres partes. Los hamartomas pueden ser simples o complejos y las características histopatológicas y la taxonómicas dependen de sus componentes tisulares. Afectan a la órbita, párpados, conjuntiva, córnea y túnicas internas

Periventricular nodular heterotopia: epileptogenic findings.

PURPOSE: We studied 17 patients with periventricular nodular heterotopia (PNH) to further investigate the electroclinical pictures and semiology of the associated seizures. METHODS: PNH was diagnosed by means of magnetic resonance imaging (MRI). The patients' clinical and familial histories were carefully analyzed, and their electroclinical features and course of epilepsy followed for periods ranging from 10 months to 22 years. The electroclinical data were compared with those of previously reported PNH cases. RESULTS: The patients were subdivided into those with bilateral (7) and unilateral (10) PNH. The former were mainly characterized by structural abnormalities in the posterior cerebral fossa and multiple seizure types; the latter were characterized by the paratrigonal location of the malformation and, frequently, by elementary seizures with a visual or auditory onset. Focal seizures were drug resistant in most cases. The interictal EEG abnormalities were always focal and consistent with the location of the PNH. A previously unreported photic driving of posterior background activity was observed in all patients and was always consistent with the PNH location. CONCLUSIONS: Our present findings and previously reported data show that bilateral and unilateral PNH cases are different in their morphological and electroclinical features and may be determined by different etiologies. The female predominance, frequent familial occurrence, and positive family history for epilepsy suggest that genetic factors may be involved in the genesis of bilateral and symmetrical PNH, whereas the presence of prenatal risk factors and its location in the watershed paratrigonal area suggest that vascular mechanisms may determine unilateral PNH.

Pharyngeal dermoids ("hairy polyps") as accessory auricles.

The purpose of this study is to clarify the origin and nature of so-called hairy polyps or dermoids of the pharynx, which are often thought to be a variant of pharyngeal teratoma. For this purpose, a case is reported of a dermoid polyp involving the middle ear of an infant, the features of multiple examples of pharyngeal dermoid polyps and teratomas received for consultation by the Armed Forces Institute of Pathology are examined, and selected pertinent reports from the literature are reviewed. All three means are used to support the conclusion that these lesions are choristomatous developmental anomalies arising from the first branchial cleft area and that they essentially represent heterotopic accessory "ears" (auricles) without the growth potential of a teratoma.