Anterior segment epibulbar choristoma containing brain tissue and with aphakia

We report an unusual case of an epibulbar choristoma in a neonate born with a mass arising from the cornea. Radiologic examination showed focal corneal bulge with absence of the lens. Histologic study revealed the lesion was an epibulbar choristoma composed of only brain tissue.     

Glial choristoma of the tongue: report of a case and review of the literature

Glial choristoma of the tongue is an extremely rare developmental malformation. The authors report a case of a 5-month-old male baby with a congenital glial choristoma located on the posterior part of midline of the left dorsal tongue. Histological examination of the resected specimen revealed a poorly demarcated submucosal mass containing neuroglial tissue, scattered neuron, choroids plexus and ependyma. In addition to neuroglial tissue, a sheet of leptomeningeal tissue was observed more rarely in the case. The clinical and pathological characteristics of previous cases and their probable embryogenesis were also reviewed.     

Epibulbar complex choristoma involving the bulbar conjunctiva and cornea

A male infant was born with a fleshy mass between the temporal bulbar conjunctiva and cornea in the right eye. Four weeks after birth the patient underwent excision of the epibulbar tumor and a lamellar keratoplasty. Histopathologic examination of the excised tumor revealed cartilage, lobules of lacrimal gland, and adipose tissue, indicating an epibulbar complex choristoma. Although the graft remained almost completely transparent, right amblyopia ensued. Epibulbar complex choristoma involving the conjunctiva and cornea is a rare condition.     

Epibulbar osseous choristoma and ectopic lacrimal gland underlying a dermolipoma

A 6-month-old girl underwent surgical excision of an epibulbar mass which had the clinical appearance of a dermolipoma. Intraoperatively, a separate underlying mass was uncovered which was firmly adherent to the sclera. Histopathologic examination confirmed the superficial lesion to be a typical dermolipoma, and the underlying lesion to be an epibulbar osseous choristoma with associated adherent ectopic lacrimal gland tissue. The clinical features, management, and histopathology of this new association are reviewed.     

Atypical oculo-orbital complex choristoma in organoid nevus syndrome

We report organoid nevus with left oculo-orbital choristoma fixing the globe in hypotropia, precanthal skin tag, and upper eyelid coloboma and right limbal dermoid. Computed tomography revealed a dilated lateral ventricle and cerebral atrophy. No neurologic deficit existed. The coloboma was repaired, choristoma debulked, and precanthal tag excised.     

Neuromuscular hamartoma: imaging features of a rare paediatric craniofacial tumour

Neuromuscular hamartoma (also referred to as neuromuscular choristoma or benign triton tumour) has not previously been described in the radiological literature. It is a rare benign lesion composed of mature elements of striated muscle and neural tissue. We report a case of neuromuscular hamartoma involving the skull base, nasopharynx, orbit and maxilla in a 2.5-year-old child who presented with facial swelling. The CT and MRI appearances of this unusual soft-tissue tumour are emphasized, together with a discussion of the pathological findings, differential diagnosis and review of the literature.     

Bilateral ocular osseous choristomas

Choristoma is a benign tumour defined as normal tissue in an ectopic location. Osseous choristoma, one subtype of this entity, occurring within the orbit has rarely been reported in the world literature. We report a 6-year-old girl with bilateral ocular osseous choristomas who presented with palpable nodules protruding from both upper lids. The radiological and clinical findings are described and previous reports are reviewed.This work was presented as a poster at the 41st Annual Congress of the European Society of Paediatric Radiology, Heidelberg, Germany, June 2004An erratum to this article is available at     

Managing epibulbar choristoma with microphthalmos

A 3-year-old boy had a large epibulbar choristoma with microphthalmos of his left eye. He received simple partial tumor excision due to significant local mechanical erosion and disfiguring appearance. The cosmetic outcome was satisfactory.     

Phakomatous choristoma: clinical, histopathologic, and ultrastructural findings in a 4-month-old boy

A case of phakomatous choristoma of the lower eyelid is described. A survey of previously reported cases reveals a characteristic and consistent clinical and morphologic picture. Universally the patient presents in infancy with a rather small, firm, rubbery nodule attached to the lower edge of the inferior tarsal plate. Always present in the nasal aspect of the lower lid, the tumor is easily palpable. Recognition of this clinical picture may allow the ophthalmologist to suspect the appropriate diagnosis, but the final diagnosis should be based on the recognition of characteristic histopathology.     

Foregut choristoma of the ileum, (adenomyoma)--a case report

An ileal adenomyoma in a 7-year-old boy who presented with jejunoileal intussusception is described and the term "foregut choristoma" is proposed for this and related conditions, such as ectopic pancreas or heterotopic Brunner's glands in the gastrointestinal tract. The polypoid mass in the ileum of this patient was composed of cystically dilated glandular structures lined by a tall columnar epithelium resembling hepatobiliary or pancreatic ductal epithelium, hypertrophic and irregular smooth muscle bundles surrounding the glandular elements, and small amount of fibrous stroma. Reported cases of this disorder in the gastrointestinal tract are generally confined to the antrum of the stomach.     

Growing mammary choristoma masquerading as a lumbosacral lipomyelomeningocele in a pubertal girl

The authors report, to the best of their knowledge, the first case of lumbosacral choristoma of breast origin, presenting in a young girl with lumbosacral lipomyelomeningocele. Although choristomas are considered to be benign, the regrowth of this mass when the patient was 15 and 16 years of age, and its involvement in the conus medullaris and cauda equina, warranted 2 additional resections with spinal cordotomy resulting in cessation of any further growth. The authors describe the case and provide a review of pertinent literature and a discussion of the mechanisms involving the development and growth of this lesion.     

Tissue regeneration based on tissue engineering technology

Recent development of biomedical engineering as well as basic biology and medicine has enabled us to induce cell-based regeneration of body tissue to self-repair defective tissue or substitute biological functions of damaged organs. For successful tissue regeneration, it is indispensable to give cells an environment suitable for regeneration induction. Tissue engineering is a newly emerging biomedical technology for creating an environment for tissue regeneration with various biomaterials. The paper presented here overviews recent research data on tissue regeneration based on tissue engineering, and briefly explains the key technology of tissue engineering.     

Biomaterials for tissue engineering applications

With advancements in biological and engineering sciences, the definition of an ideal biomaterial has evolved over the past 50 years from a substance that is inert to one that has select bioinductive properties and integrates well with adjacent host tissue. Biomaterials are a fundamental component of tissue engineering, which aims to replace diseased, damaged, or missing tissue with reconstructed functional tissue. Most biomaterials are less than satisfactory for pediatric patients because the scaffold must adapt to the growth and development of the surrounding tissues and organs over time. The pediatric community, therefore, provides a distinct challenge for the tissue engineering community.     

Frequency and potential cause of bronchus-associated lymphoid tissue in fetal lungs

Bronchus-associated lymphoid tissue consists of lymphoid follicles with or without a germinal center within the bronchial wall. Bronchus-associated lymphoid tissue is part of the integrated mucosal immune system and present in about 50% of healthy infants. We examined a series of 141 fetal and neonatal lungs and detected bronchus-associated lymphoid tissue in 100% of cases with amniotic infection while postpartum perinatal pneumonia did not elicit bronchus-associated lymphoid tissue formation. Only rarely and in low density, bronchus-associated lymphoid tissue was present in non-infected fetuses. The in utero formation of bronchus-associated lymphoid tissue seems to be a reactive phenomenon and - as has been shown in another study - does not portend an adverse prognosis.     

Heterotopic Pancreas as Lead Point in Intussusception: New Variant of Vitellointestinal Tract Malformation

Two cases of intussusception are reported with heterotopic pancreatic tissue attached to and draining into the ileum. The first patient, a boy aged 16 months, presented with ileoileal intussusception. The diagnosis was confirmed on ultrasound scan. Laparotomy and resection were performed. A 12-mm nodule of heterotopic pancreatic tissue was identified in the ileal serosa at the apex of the intussusceptum, fully formed with acinar tissue, islets, and draining duct. The second patient, also a boy aged 16 months, presented with obstructed ileocolic intussusception in which the lead point at surgery resembled a Meckel's diverticulum. Histopathology revealed a similar 10-mm nodule of fully formed pancreatic tissue in the ileal serosal tissues, with some acinar tissue extending through the wall of the intestine alongside ductal structures. In both cases there was ectopic gastric mucosa either in the distal part of the draining duct or in the small intestine itself at the opening. Heterotopic pancreas is a rare cause of intussusception. We propose that this lesion is of vitellointestinal tract origin, conceptually similar to a Meckel's diverticulum but without a diverticulum as such. Heterotopic pancreatic tissue occurring alone is more common in the proximal small intestine, duodenum, and stomach than in the ileum, and it is often asymptomatic. Received April 3, 1998; accepted July 23, 1998.     

脂肪组织造血再生潜能的研究进展

脂肪组织来源于中胚层,由脂肪细胞及基质血管成分组成.基质血管成分是一个尚未完全确定的细胞群,其中包含多种干/祖细胞.近来研究表明脂肪组织含有一定数量的造血干/祖细胞以维持其造血再生潜能,但具体调节机制目前尚不明确.现就脂肪组织及与骨髓微环境相似性研究进展作一综述,为认识脂肪组织的造血再生潜能提供新思路.     

Temporal Soft Tissue Glioneuronal Heterotopia in a Child with a Seizure Disorder: Case Report and Review of the Literature

Tissue and cellular elements generally attributed to the central nervous system (CNS) are infrequently found in areas outside the CNS proper. Most of these lesions contain predominantly glial tissue. In rare instances, heterotopic CNS tissue is found in the scalp, many associated with an intracranial connection and overlying skin and hair anomalies. In follow-up of these patients, development is normal. We present a case of a temporal scalp mass in a 19-month-old girl with a seizure disorder beginning at about age 1 year. At the time of excision, the mass was not associated with intracranial connection or overlying cutaneous abnormalities and demonstrated abundant neuronal and glial elements with features of dysplastic CNS tissue. We speculate that, in certain circumstances, aberrantly localized CNS tissue may be related to abnormal CNS development.     

Raynaud syndrome in childhood

Twenty-seven patients with Raynaud syndrome (mean age at onset 11.7 years) were studied to determine the prevalence of primary Raynaud syndrome and to assess the predictive role of antinuclear antibody, nail-fold capillary microscopy, and photoelectric plethysmography in this population. Fourteen patients (52%) had a connective tissue disease, four (15%) had a probable connective tissue disease, and nine (33%) had primary Raynaud syndrome. In all patients with either a connective tissue disease or a probable connective tissue disease, there was a positive reaction to antinuclear antibody, in contrast to patients with primary Raynaud syndrome, in whom antinuclear antibody was not detected. Nail-fold capillary microscopy scores differed significantly between patients with either a connective tissue disease or a probable connective tissue disease and those with primary Raynaud syndrome for both enlarged loop score (p less than 0.025 and less than 0.05, respectively) and avascular score (p less than 0.005 and less than 0.01, respectively). Photoelectric plethysmography scores were reduced in all groups but did not differ significantly between groups. Our findings suggest that in children with Raynaud syndrome, the primary type is more common than was originally suspected, and that both antinuclear antibody and nail-fold capillary microscopy, but not photoelectric plethysmography, can distinguish patients with primary Raynaud syndrome from those with either a connective tissue disease or a probable connective tissue disease.     

Brainstem nitric oxide tissue levels correlate with anoxia-induced gasping activity in the developing rat

Gasping is an important mechanism for survival that appears to be developmentally modulated by the glutamate-nitric oxide (NO) pathway. However, the temporal characteristics of NO brain tissue levels during gasping are unknown. We hypothesized that during anoxia-induced gasping, the gasping frequency would be closely correlated with caudal brainstem tissue NO concentrations in developing rats. Brainstem and cortical tissue NO levels were measured during anoxia using a voltammetric electrode in adult rats and 5-day-old pups during control conditions and following pretreatment with the NMDA receptor antagonist MK-801 (1 mg/kg) or the neuronal NO synthase inhibitor 7-nitro-indazole (7-NI; 100 mg/kg). In young animals, NO tissue levels followed a triphasic trajectory coincident with gasp frequency which was markedly altered by MK-801 and 7-NI, albeit with preservation of gasp frequency-NO tissue level relationships. In adult rats, 40-fold higher NO tissue levels occurred and followed a monophasic trajectory coincident with gasp patterning. In the cortex, monophasic increases in NO levels occurred at all ages. We conclude that anoxia-induced gasping neurogenesis is modulated via NMDA-NO mechanisms in the developing rat. We postulate that higher NO brainstem concentrations may favor early autoresuscitation, but limit anoxic tolerance.     

Bilateral adrenal cysts and ectopic pancreatic tissue in Beckwith-Wiedemann syndrome: is a conservative approach acceptable?

Beckwith-Wiedemann syndrome is a common overgrowth syndrome associated with an increased risk of neoplasias which might be explained by the nature and localization of the genetic defect. While malignant tumors are often associated with hemihypertrophy, benign tumors are also found. We report a patient with the typical features of Beckwith-Wiedemann syndrome with two histologically different abdominal tumors, bilateral cystic adrenals and ectopic pancreatic tissue present at birth. In both tumors no malignancy could be detected. Ectopic pancreatic tissue is rarely seen and has been described in Beckwith-Wiedemann syndrome only once. After extirpation of the ectopic pancreatic tissue the cystic adrenals were left in situ since macroscopically no normal adrenal tissue could be identified and separated. Regular ultrasound examinations revealed complete resolution of the cystic adrenals within 24 months. Thus it seems that a conservative approach in selected tumors associated with the Beckwith-Wiedemann syndrome might be acceptable.