Rathke's cleft cyst in two girls with precocious puberty

Rathke's cleft cysts arise from remnants of Rathke's pouch and are usually found incidentally on MRI or autopsy. In childhood, the most common presenting symptoms of Rathke's cleft cysts are endocrine abnormalities, such as reduced growth hormone secretion, hyperprolactinemia, or diabetes insipidus. Non-specific symptoms, such as headache and visual disturbance, may also occur. Although precocious puberty has occasionally been described in association with suprasellar lesions, such as hamartomas, arachnoid cysts, and craniopharyngiomas, to our knowledge there have been no documented cases secondary to Rathke's cleft cysts. We report here two patients, both of whom presented with precocious puberty, and were found to have Rathke's cleft cysts.     

MRI in seven cases of Rathke's cleft cyst in infants and children

Seven MRI observations of presumed Rathke's cleft cysts in infants and children are reported. Age ranged between 6 months and 13 years. Size of the cysts ranged between 4 and 20 mm. While 3 small cysts were detected incidentally, 4 were large enough to cause symptoms secondary to compression of the pituitary gland and suprasellar components. The MRI appearance of the Rathke's cleft cysts varied according to their nature. MRI provides a major contribution to the diagnosis of these lesions; nevertheless, it often remains difficult to differentiate complex Rathke's cleft cysts from cystic craniopharyngiomas. The difficulties related to this differentiation are discussed.Presented at the 29th Congress of the European Society of Pediatric Radiology, 27 April to 1 May 1992, Budapest, Hungary and selected for publication by an International Group of the ESPR     

Rathke's cleft cysts in children and adolescents: association with female puberty

There are few pediatric data regarding manifestations and outcomes of Rathke's cleft cysts (RCC). We retrospectively reviewed 13 cases treated at Massachusetts General Hospital over 10 years. Age at presentation was 12-17 years, except for one 7-year-old who presented with sexual precocity. There was a female preponderance [11 females, 2 males, p = 0.01], and all were pubertal at diagnosis. Common features at presentation were headaches (11/13), endocrine abnormalities (5/13) and visual disturbances (2/13). Four patients underwent transsphenoidal surgery. Symptoms improved in all but one, in whom headaches persisted. Recurrent growth in one patient was treated successfully by excision. For conservatively treated patients, cyst size was unchanged over follow-up (6 months-5 years). Female preponderance and pubertal presentation suggest a possible link between sex hormones and RCC pathogenesis. Although estrogen and progesterone receptor immunostaining was negative in the cyst lining, estrogen receptor immunostaining was positive in adjacent pituitary cells. Further investigations regarding this issue are warranted.     

Spontaneous rupture of a choledochal cyst: clues to diagnosis and etiology.

A healthy 3-year-old black girl had acute onset of abdominal pain, vomiting, and elevated aminotransferase, amylase, and lipase levels. Sonographic evaluation suggested a choledochal cyst, but hepatobiliary scintigraphy clearly showed an extrahepatic biliary leak. Exploratory laparotomy confirmed bile peritonitis and a ruptured choledochal cyst. Other cases with this unusual presentation is reviewed in the literature. An anomalous insertion of the pancreatic duct into the common duct appears to facilitate reflux of pancreatic secretions into the biliary tree and may contribute to formation and even perforation of a choledochal cyst.     

The role of ocular torsion on the etiology of A and V patterns

Theoretically, the torsion of the globe produced by primary oblique overaction results in a vertical displacement of the insertions of the horizontal recti and a horizontal displacement of the insertions of the vertical recti. This should alter the vectors of the forces exerted on the globe by the rectus muscles so that the horizontal recti become partial elevators or depressors and the vertical recti become increasing abductors or adductors. This change in force vectors tends to enhance the A or V pattern that results from oblique dysfunction. I propose that the etiology of A and V patterns is the result of a complex interplay between all of the extraocular muscles and present a clinical series to suggest the validity of that theory.     

全垂体功能减低男性患儿性激素变化及治疗

目的探讨全垂体功能减低男性患儿性激素的改变特点,以适时进行性激素补充治疗(HRT),改善男性性征。方法测定1999-09—2006-03因生长迟缓在山东省医院就诊的44例全垂体功能减低的10岁以上男性患儿性激素,并与44例年龄匹配的对照组进行比较;观察两组儿童性激素水平差异。结果44例全垂体功能减低患儿与对照组儿童性激素比较显示,观察组促卵泡激素(FSH)、促黄体生成激素(LH)、睾酮(To)均显著低于对照组,且44例患儿(100%)均有此三项降低;雌激素(E2)、泌乳素(PRL)均显著高于对照组。其中26例(59%)有E2升高,27例(61%)有PRL升高。此可解释男性垂体功能减低患儿女性体态、外生殖器发育不良及乳房发育。结论全垂体功能减低患儿常伴有性发育不良,早期诊断、及时有效替代治疗非常必要。     

Persistent femoral neck cysts in healed Legg-Perthes' disease: a clue to the etiology of simple bone cyst?

Persistent femoral neck cysts in an adult with healed Legg-Perthes disease had the radiological features of which are rather like those of simple bone cyst. To the author's knowledge such persistent cysts have not been reported previously. Existence of such cysts in healed Legg-Perthes' disease may provide a clue to the etiology of simple bone cyst.     

Efficacy of OK-432 local injection for the treatment of a neonatal branchial cleft cyst: a case report

We herein present a case of a neonatal cervical cyst, which was diagnosed prenatally, and markedly decreased in size and disappeared after a local injection therapy of OK-432. A 0-day-old boy had an abnormal prenatal ultrasonography scan suggestive of rt. cervical cyst, measuring about 25 mm in diameter at 29 weeks’ gestation. At birth, an elastic soft mass, measuring about 30 mm in diameter, was found on the right side of his neck. Computed tomography (CT) scans showed a giant cyst, which extended from the upper level of epipharynx to the upper mediastinum, and the contents were air and fluid. At 20 days of age, ultrasonography (US)-guided needle aspiration was performed. The aspirated fluid contained no epithelial cells, but many lymphocytes and neutrophils based on a cytological analysis. After the local injection of OK-432 had been performed four times, the right neck cyst had almost completely disappeared on US scans. During the local injection therapy, we analyzed the other sample of the second aspiration fluid of the neck cyst. Several clusters of epithelial cells, columnar epithelium, squamous cells, and ciliated epithelium were thus cytologically observed. Therefore, a final diagnosis of a branchial cleft cyst was made. The local injection of OK-432 was thus found to be an effective treatment for branchial cleft cysts.     

Repair of cleft lip and simultaneous repair of cleft hard palate with vomer flap in unilateral complete cleft lip and palate: a comparative study

Abstract  

Repair of anterior palate after repairing the cleft lip in complete cleft lip and palate patient is sometimes very difficult. It needs wide, extensive and difficult dissection, and has later chances of wound infection, wound dehiscence, complete wound disruption, recurrent oronasal fistula formation and also maxillary hypoplasia. In this study, we compare the outcome of simultaneous repair of cleft lip and cleft hard palate with vomer flap against cleft lip repair alone in patients with unilateral complete cleft lip and palate (UCLP).     

Seizures in a 20-month-old native of Minnesota: a case of neurocysticercosis

A 20-month-old child, native of Minnesota, was diagnosed with neurocysticercosis. He had no history of international travel or pork consumption. This case and review of the literature emphasize the need to consider neurocysticercosis in any child with nonfebrile seizures in the United States because international travel and exposure to international travelers have become so common throughout the world.     

男性儿童乳房发育38例临床分析

目的：对男性儿童乳房发育症的病因、治疗经验进行总结。方法：回顾性分析38例2~14岁男性儿童乳房发育症的临床资料。结果：38例中17例为男性青春期乳腺增生症,2例可能与原发疾病有关,4例因误服药物或接触含“性激素”类护肤品所致,15例为男性儿童特发性乳房发育症。对3例B3期男性乳房发育患儿给予乳癖消片1.34 g口服,每日3次,连服1月;对16例年龄≥12岁的B2期伴有明显症状的男性乳房发育患儿给予乳癖消片1.34 g口服,每日3次,连服3~5 d;其余均未给予药物治疗,仅针对原发病因治疗。随访1月至1年,绝大多数病例增大的乳房均消退。结论：导致男性儿童乳房发育症的原因包括青春期乳腺增生、误服药物以及其他疾病的合并症等。绝大多数男性儿童乳房发育症是生理性的,仅临床症状明显患儿予药物治疗。     

小儿呼吸困难误诊漏诊临床分析

目的探讨误诊漏诊的小儿呼吸困难及其原因。方法回顾性分析我院儿科62例误诊漏诊呼吸困难患儿的临床资料。结果62例误诊漏诊的呼吸困难患儿中，呼吸道误吸19例。呼吸系统解剖异常14例，先天性心脏病12例，纵隔占位性疾病6例，糖尿病酮症酸中毒3例，吉兰-巴雷综合征2例，其它6例。其中单一病因（非肺炎疾患）所致呼吸困难14例，复合病因（肺炎＋基础疾病）48例。因疾病表现不典型或基础疾病被肺炎掩盖而误诊漏诊。结论小儿呼吸困难误诊漏诊的主要疾病是呼吸道误吸。对那些不能单独用呼吸系统疾病合理解释的呼吸困难，应尽早做相应检查以便确诊。     

小儿呼吸困难误诊漏诊临床分析

目的探讨误诊漏诊的小儿呼吸困难及其原因。方法回顾性分析我院儿科62例误诊漏诊呼吸困难患儿的临床资料。结果62例误诊漏诊的呼吸困难患儿中,呼吸道误吸19例,呼吸系统解剖异常14例,先天性心脏病12例,纵隔占位性疾病6例,糖尿病酮症酸中毒3例,吉兰-巴雷综合征2例,其它6例。其中单一病因(非肺炎疾患)所致呼吸困难14例,复合病因(肺炎+基础疾病)48例。因疾病表现不典型或基础疾病被肺炎掩盖而误诊漏诊。结论小儿呼吸困难误诊漏诊的主要疾病是呼吸道误吸。对那些不能单独用呼吸系统疾病合理解释的呼吸困难,应尽早做相应检查以便确诊。     

Basal meningoencephalocele, anomaly of optic disc and panhypopituitarism in association with moyamoya disease

Basal meningoencephalocele is frequently associated with midfacial anomaly, optic disc anomaly, brain anomaly, cerebrospinal fluid rhinorrhea, chiasma syndrome, and endocrinologic disturbance. The combination of basal meningoencephalocele and moyamoya disease is extremely rare. A 29-year-old man had basal meningoencephalocele (transsphenoidal type), anomaly of the optic disc (morning glory syndrome), panhypopituitarism and moyamoya disease. The patient was treated by hormone replacement, but surgical intervention was not required. Basal meningoencephalocele and moyamoya disease are a possible combination of the diseases.     

Transformation of corneal epithelial cyst into anterior chamber implantation cyst and scleral cyst: a rare occurrence

An 8-year-old boy underwent a penetrating keratoplasty for a corneal epithelial cyst. After surgery an anterior chamber implantation cyst appeared. Repeated Neodymium:YAG (Nd:YAG) laser treatments failed to eradicate this cyst; it later extended into the sclera together with the collapse of the anterior chamber cyst. The possible mechanism underlying the development of these cysts is discussed.