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骶尾部畸胎瘤:尼日利亚儿童的临床特征及长期预后

Sacrococcygeal teratoma: clinical characteristics and long-term outcome in Nigerian children.

作者信息

Chirdan Lohfa B, Uba Aba F, Pam Sunday D, Edino Stephen T, Mandong Barnabas M, Chirdan Oluwabunmi O

机构信息

Paediatric Surgery Unit, Department of Surgery, Jos University Teaching Hospital, Jos, Nigeria.

出版信息

Ann Afr Med. 2009 Apr-Jun;8(2):105-9. doi: 10.4103/1596-3519.56238.

Abstract

BACKGROUND/PURPOSE: The excision of sacrococcygeal teratoma (SCT) may be associated with significant long-term morbidity for the child. We reviewed our experience with SCT in a tertiary health care facility in a developing country with particular interest on the long-term sequelae.

METHODS

Between January 1990 and May 2008 inclusive, 38 consecutive children with the diagnosis of SCT were identified from the operation register and the Cancer Registry of the Jos University Teaching Hospital. Their clinical presentation, investigation, operative findings, histology report, and outcome were recorded and analyzed. The long-term follow-up of some of the patients were also recorded and analyzed.

RESULTS

There were 31 females and 7 males. Twenty-three patients presented during the neonatal period with a median age at presentation of 7 days (range 1-18 days) and a median weight at presentation of 2.8 kg (range 2.0-3.6kg), 10 presented between 1 month and 12 months, while 5 were older than 1 year at presentation. Most of the patients had significantly external tumors. Excision of the tumor was mainly by the sacral route, four had abdominal-sacral excision. Histology was mainly benign; four were malignant at presentation. Four children with malignant disease had chemotherapy in addition to excision of the tumor. Eight had immediate post-operative wound-related complications while three children died, two of the deaths were related to anesthesia, while one died of colostomy complications. Twenty-one (60%) were followed up for a median duration of 6 years (range 1 month-8 years). Two (9.5%) had recurrent disease after primary excision; five (23.8%) had some degree of functional impairment at the follow-up.

CONCLUSION

While SCT is usually benign, recurrence, malignant transformations in patients who present late and long-term functional sequelae are problems that must be tackled by the care givers. A multi-center study may be necessary to characterize this disease in developing countries and assess the long-term functional sequelae in survivors.

摘要

背景/目的:骶尾部畸胎瘤(SCT)切除术可能会给儿童带来严重的长期并发症。我们回顾了在一个发展中国家的三级医疗机构中治疗SCT的经验,特别关注其长期后遗症。

方法

在1990年1月至2008年5月期间,从乔斯大学教学医院的手术登记册和癌症登记册中确定了38例连续诊断为SCT的儿童。记录并分析了他们的临床表现、检查、手术发现、组织学报告及结果。还记录并分析了部分患者的长期随访情况。

结果

31例女性,7例男性。23例患者在新生儿期就诊,就诊时中位年龄为7天(范围1 - 18天),中位体重为2.8 kg(范围2.0 - 3.6 kg);10例在1个月至12个月之间就诊,5例就诊时年龄超过1岁。大多数患者有明显的外部肿瘤。肿瘤切除主要通过骶骨途径,4例采用腹骶联合切除。组织学检查主要为良性;4例就诊时为恶性。4例患有恶性疾病的儿童除肿瘤切除外还接受了化疗。8例术后立即出现与伤口相关的并发症,3例儿童死亡,2例死亡与麻醉有关,1例死于结肠造口并发症。21例(60%)接受了中位时长为6年(范围1个月 - 8年)的随访。2例(9.5%)在初次切除后出现疾病复发;5例(23.8%)在随访时有一定程度的功能障碍。

结论

虽然SCT通常为良性,但复发、晚期就诊患者的恶性转化以及长期功能后遗症是护理人员必须应对的问题。可能需要进行多中心研究来明确发展中国家这种疾病的特征,并评估幸存者的长期功能后遗症。

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