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

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.

Typhoid intestinal perforation in children: a continuing scourge in a developing country.

Typhoid intestinal perforation is a principal cause of morbidity and mortality affecting both adults and children. This study aims to evaluate the pattern of typhoid intestinal perforation and outcome of its management in Nigerian children. The records of consecutive children managed for typhoid intestinal perforation at Juth, between 1996 and 2005 have been reviewed. There were 184 children (M:F = 1.04:1), with a mean of 5.8 years (range 4 to 15 years). More than a half (62.5%) of the patients were in the 5-6 year age group. The incidence peaked in April-May, and November-December. Increased incidence of typhoid perforation was observed between 2002 and 2005. All patients presented with the classic features of typhoid enteric perforation. Hypokalaemia and anaemia were common at presentation. Only 75 (40.6%) patients had operation within 24 h of perforation. The types of surgery included simple excision of the edges of the peroration and closure (74.5%), wedge resection and closure in (14.5%), segmental resection with primary end-to-end anastomosis (3.6%) and right hemi-colectomy with ileo-colic or ileo-transverse anastomosis. Wound infection and dehiscence, anastomotic breakdown with faecal fistula, intra peritoneal abscesses and chest infections were the main post operative complications. The overall mortality rate was 22.8 (42). Excision and simple closure was associated with the least incidence of anastomotic breakdown and operation time. The incidence of typhoid intestinal perforation is on the increase at our institution. Early limited surgery provides optimal results.

Management of cystic lymphangioma in children: experience in Jos, Nigeria.

The management of cystic lymphangiomas (CL), especially in the head and neck region, presents challenges to the pediatric surgeon. This is a retrospective study of all children seen with lymphangioma at the Pediatric Surgical Unit of the Jos University Teaching Hospital from 1996 to 2004. There were 27 children, 14 (51.9%) were males and 13 (48.1%) were females (M:F = 1.2:1), with ages ranging from 2 days to 5 years (median 5 months). Majority (84.6%) of the patients were below 1 year. The head and neck region was the most frequent site of involvement. There were three cases of lymphangioma circumscriptum. Six (54.5%) of the 11 children with cervical CL presented with complications including infection in 4, respiratory obstruction in 3, ulcerated lesion in 1 and intracystic hemorrhage with rapid increase in cyst size in 1. Twenty-three children had either surgical excision or marsupialization. The main complications were wound infections and respiratory obstructions, which led to five deaths. Six children had recurrences, some of which occurred at sites which were normal at the initial or previous excision. The mortality rate was high (34.8%), mainly due to respiratory obstruction and asphyxia in the head and neck lesions. Head and neck region was the most commonly involved site by CL. The management of CL at this site is associated with significant morbidity and a high rate of mortality.

[Breast cancer: assessment of individual risk and possibilities for prevention]. / Mammakarzinom: Ermittlung des individuellen Erkrankungsrisikos und Möglichkeiten der Prävention.

Today, prevention of breast cancer (BC) is a great demand. The exact estimation of the individual BC-risk is a prerequisite for the participation on early cancer detection or the use of preventive medication or surgery. Various models for risk assessment of BC development or the presence of a predisposing mutation (i.e. BRCA1 or BRCA2) are used, but the statistical individual risk assessment still remains uncertain. Calculating an elevated risk or detection of a predisposing mutation leads to the recommendation of preventive measurements. After detailed assessment, prophylactic bilateral mastectomy is an option to consider for mutation carriers. For women with low BC-risk, chemoprevention can be discussed. Chemoprevention with tamoxifen (TAM)--indirectly supported by BC data from the raloxifen (RLX) prevention trial for osteoporosis and cardio-vascular disease--points to the right direction. Results from the three published TAM prevention trials are variable. Life time risk, age and life style have to be considered in the adapted individual risk-benefit assessment. The lack of long term risk data for chemoprevention and the effect on survival are arguments contra the routine use of TAM as a chemopreventive agent.

[Tumor risk consultation for predisposed women from high risk cancer families]. / Tumorrisikosprechstunde für prädisponierte Frauen aus Krebsrisikofamilien.

Germline mutations of the cancer susceptibility genes BRCA1 and BRCA2 seem to lead to a very high risk for breast and/or ovarian cancer. Therefore, genetic counselling and identification of high-risk families may be essential to offer the opportunity to participate in a specific early cancer detection program and to provide individualized psychological support. In a two year period (August 1994-August 1997) 304 consultees present for genetic counselling at the interdisciplinary cancer genetic clinic (Department of Obstetrics & Gynecology and Human Genetics, Heinrich-Heine-Universität, Düsseldorf). For genetic testing a BRCA1/2 mutation detection strategy including protein truncation test (PTT), single strand conformation polymorphism (SSCP), and direct DNA sequencing is used. 161 families fulfilled the inclusion criteria; at present, 72 families for whom complete analytical material is available are analyzed. Although genetic testing for BRCA1 and BRCA2 is technically challenging, women with a family history of multiple sporadic breast/ovarian cancers and those with a hereditary BRCA1 and BRCA2 gene defect may be distinguished. For the first group of consultees this may ease their concern, for the second group preventive measures including an early cancer detection or prevention program, psychological support or prophylactic surgery may be discussed.