PubMed Health⌕ Search

Biomedical subjects

R Wiersma

Publications and source records attributed to R Wiersma.

17 recordsLinked to original sources

Intersex conditions in children and adolescents: surgical, ethical, and legal considerations.

INTRODUCTION: Approximately one in 2000 children globally is born with an intersex condition. There is unfortunately a relative paucity of data on the choices and the surgical and psychosocial outcomes in patients who undergo genital surgery for intersex conditions and ambiguous genitalia, especially in developing countries. Specialists in these and other countries, where patient follow-up is generally poor, are faced with the daunting task of offering the appropriate medical and surgical management, in the absence of guidelines or recommendations. SURGICAL CONSIDERATIONS: A surgical procedure in these patients sometimes involves clitoral recession, reduction, vaginoplasty, and gonadectomy. The best surgical outcome is likely to be achieved with a multidisciplinary surgical team; however, the choice of surgery and appropriate timing remains controversial. Some authors have suggested delaying surgery until the child becomes competent to make his/her own decisions. LEGAL/ETHICAL CONSIDERATIONS: All procedures should conform to an ethical code of practice and be in the interest of the child. Exhaustive counseling of all parties and informed consent is of paramount importance, as is adherence to laws that protect the rights of the child as outlined in respective constitutions. RECOMMENDATIONS: Recommendations in this article, which have been put together from the combined input of three departments, are broad-based. They emphasize the need for extensive counseling, informed consent, adherence to ethical and legal norms, a multidisciplinary input and a shift away from a paternalistic approach.

Adolescent↗

True hermaphroditism in southern Africa: the clinical picture.

This is an 18-year (1985-2001) retrospective review of 85 patients with true hermaphroditism, with the aim of facilitating early recognition of this condition. Presentation of neonates and infants 6 months or younger, constituting 54% of this cohort, were different from the older children. The presentation, clinical features and investigative results of all patients diagnosed with true hermaphroditism at a single South African paediatric surgical unit were reviewed. This paper highlights the previously reported high incidence (51%) of this condition, as well as some of the unusual features of true hermaphroditism in this region. Diagnosis of true hermaphroditism requires a high index of suspicion for subtle anomalies of the genitalia. Although there were no pathognomonic clinical features, the true hermaphrodite presents as a patient of either gender with a congenital anomaly of the genitalia. The child is likely to have a normal male phallus, bifid labio-scrotal folds, a perineal hypospadias and in 53% of patients there was a palpable gonad. The method of investigation, together with the results and some of the management dilemmas associated with true hermaphroditism in a Third World population are presented.

Adolescent↗

Minimizing surgery in complicated intussusceptions in the Third World.

When presentation is delayed, intussusceptions may be difficult to reduce using standard enema regimens. Our endeavour to minimize the need for surgery in an environment where failed reductions are common has led to the development of an aggressive, non-operative method of reducing intussusceptions. One hundred and six patients with intussusception were reviewed with the aim of evaluating a new method of reducing intussusceptions suited to our Third World environment. In our cohort, delayed presentation was common, with 32% of patients presenting more than 48 h after the onset of the intussusception. On clinical grounds alone, 41% of patients required a primary laparotomy. Standard barium and air reductions for intussusception were rarely successful under these conditions i.e. 13% and 22%, respectively. By using an air enema under general anaesthesia in the operating theatre, the reduction rate has improved to 53%. This approach is suggested as a last attempt at reducing an intussusception prior to laparotomy following failed standard enema reduction, and as the first line of management in the attempted reduction in the patient with delayed presentation without symptoms of peritonitis.

Air↗

Neonatal gastric perforation.

BACKGROUND: Gastric perforation in neonates is a catastrophe associated with high morbidity. Most are due to underlying primary pathology. OBJECTIVES: To review the management of gastric perforation in neonates in Kwa Zulu-Natal, South Africa. DESIGN: Retrospective study of consecutive complete data sets of neonates presenting with gastric perforation. SETTING: Department of Paediatric Surgery, Nelson R. Mandela School of Medicine, University of Natal, Durban, South Africa. SUBJECTS: Eight neonates treated for gastric perforation between January 1998 and April 2003. MAIN OUTCOME MEASURES: Morbidity and mortality. RESULTS: There was an equal number of males and females. Median birth weight was 2.0 kg with a range of 1.4 to 3.2 kg. Five of the eight neonates were premature. Primary pathologies were associated with perforation in seven of the eight neonates. Prematurity, low birth weight and pneumonia were contributing factors to the poor outcome. Sepsis was a complication in seven of the eight neonates leading to their death (88% mortality). CONCLUSION: Active perinatal management, early treatment of primary pathologies, and protection of the stomach against distension in neonates at risk are essential in the management of neonatal gastric perforation.

Female↗

HIV-positive African children with rectal fistulae.

BACKGROUND/PURPOSE: Human immunodeficiency virus (HIV) disease is an increasingly common infection in children in sub-Sahara Africa. Rectal fistulation is one such condition with which these patients present to the paediatric surgeon. This appeared to be an exclusively female condition until 2 male patients were treated recently. METHODS: A 6-year (1996 through 2001) retrospective study found 39 children presenting with HIV-related rectal fistulae. Thirty-seven girls were seen with rectovaginal fistulae (RVF), and there is supportive documentation showing an increase in this condition throughout Southern Africa. Until now, boys have not been described with this condition. The author presents 2 boys who complete this spectrum of HIV-related acquired rectal fistulae. RESULTS: All patients were found to have rectal fistula at the dentate line. In girls it varied in size from pin-point to 5 mm diameter, tracking anteriorly into the vagina. When closure of the fistula was attempted, it broke down. The 2 boys had a large fistula, which tracked to the prostatic urethra on the right of the verumontanum. The first patient underwent a successful repair. The second patient had a "Y"-shaped fistula based at the dentate line, with the second limb passing into the bladder. The parents refused further treatment and took the child home. CONCLUSIONS: HIV disease affects increasing numbers of children. A spectrum of rectal fistulae now has been seen in both girls and boys. These acquired rectal fistulae arise at the dentate line in both genders. Girls with these fistulae are seen more commonly, presenting with RVF. The closure of a fistula has only been successful in one boy.

Child↗

Gastro-intestinal tract perforation in neonates.

BACKGROUND: Gastro-intestinal tract (GIT) perforation in neonates is a serious problem associated with high mortality due to resulting sepsis. Co-morbid factors, eg. prematurity, respiratory problems, low birth weight, and nutritional factors, negatively affect the outcome. OBJECTIVES: To review the management outcome of gastro-intestinal tract perforation in neonates in KwaZulu-Natal and identify factors that require attention for better survival of neonates with GIT perforation. DESIGN: Retrospective study of consecutive complete data sets of patients presenting with a diagnosis of GIT perforation. SETTING: Department of Paediatric Surgery, Nelson R. Mandela School of Medicine, University of Natal, Durban, South Africa. SUBJECTS: Fifty four neonates treated for gastro-intestinal tract perforation between January 1998 and January 2003. MAIN OUTCOME MEASURES: Morbidity as determined by complications and mortality. RESULTS: More males (69%) were affected than females (31%). The median birth weight was 2.3 kg and median age at presentation was four days. Eighty nine percent were referred from peripheral hospitals. Abdominal distension was the leading symptom and sign (74%). Co-morbid factors were present in 89%, with prematurity as the leading factor (52%). Necrotising enterocolitis (NEC) was the main cause of perforation (33%) and the terminal ileum was the most common site. Most (56%) were treated by excision and primary repair of perforations. Sepsis was the leading complication (44%) and major cause of death (72%). Mortality was highest (56%) in perforations due to other primary pathology followed by NEC (53%). Overall mortality was 46%. CONCLUSION: It is essential to prevent secondary perforations by early recognition and management of primary pathology. Management of pneumoperitoneum in neonates with respiratory difficulties should be included in resuscitation before transfer. Rectal temperature monitoring and herbal enemas should be strongly discouraged.

Anti-Infective Agents↗

Rectal gastric heterotopia in infancy.

Heterotopic gastric mucosa is rare in children with most cases presenting in association with Meckel's diverticulum. The authors present a 2-year-old boy with gastric heterotopia of the anorectum, who presented with painless, intermittent rectal bleeding.

Anus Diseases↗

Management of the African child with true hermaphroditism.

BACKGROUND/PURPOSE: A disproportionally high incidence of true hermaphroditism is seen among the South African black people, constituting 51% of children in local study on all intersex types. These patients were different from the commonly reported patients with true hermaphroditism in that the common gonad was a mixed type of ovotestis, making the management of such patients problematic. The aim of this study was to establish a protocol for the management for children with true hermaphroditism in Southern Africa. METHODS: Seventy-one children, over a 16-year period, were diagnosed with true hermaphroditism. After a decision on the child gender status, part of the management consisted of excision of all discordant and ovotesticular gonadal tissue. The histology of these gonads was then compared with the initial biopsy results. RESULTS: The histology of the initial gonadal biopsies showed that 55% of all gonads were ovotestes, 26% were ovaries, and 19% testes. Thirty-five patients had 44 gonads excised. Comparing the 2 sets of histology showed that the initial histology of the discordant gonads was insufficient to show all the ovotesticular tissue in 22% of gonads. Initial biopsies that showed testicular tissue only were misdiagnosed in 82% of cases. CONCLUSION: It is suggested that, in Southern African true hermaphrodites, all ovotestes, discordant gonads, and all gonads that show only testicular tissue be excised as part of the initial corrective management.

Adolescent↗

Lymph node biopsies in HIV-infected and non-infected children with persistent lung disease.

OBJECTIVE: The diagnosis of opportunistic infections in children with persistent lung disease (PLD) who are infected with the human immunodeficiency virus (HIV) is difficult to establish, especially in resource-poor countries. Lymphadenopathy is a frequent associated clinical finding among these children. We evaluated the usefulness of excision lymph node biopsies in determining an aetiological diagnosis in HIV-infected and non-infected children with PLD. METHOD: Forty-five children with PLD and significant lymphadenopathy were subjected to lymph node biopsy. Of these, 27 were HIV-infected. All subjects had excision biopsies; 39 (86.7%) of these cases also underwent fine needle aspiration cytodiagnosis (FNAC) and trucut needle biopsies. RESULTS: Tuberculosis was identified as the final diagnosis in 11 (40.7%) and 12 (66.7%) HIV-infected and noninfected children, respectively. Ancillary investigations (Mantoux, gastric washings) suggested a diagnosis of tuberculosis in eight (72.7%) and eight (66.7%) of the final diagnoses of tuberculosis among HIV-infected and non-infected children, respectively. Lymph node biopsies identified a further three (27.3%) and four (33.3%) more cases of tuberculosis as compared to ancillary investigations among HIV-infected and non-infected groups, respectively. Results of FNAC and trucut biopsy showed good correlation with excision biopsy: 96.4% and 97.4%, respectively. However, adequate samples were obtained in only 23 of 39 FNAC and 33 of 39 trucut biopsies. CONCLUSION: Excision lymph node biopsies form a useful adjunct investigation in children with PLD and generalised lymphadenopathy. The most common disease identified among HIV-infected and non-infected children in Durban, South Africa, is tuberculosis. FNAC and trucut biopsies may also be useful in the evaluation of lymphadenopathy when appropriate specimens are obtained.

AIDS-Related Opportunistic Infections↗

Surgical lessons learned from the Shigella dysenteriae type I epidemic.

An epidemic of Shigella dysenteriae type I is spreading through Africa. It is a particularly infectious and virulent form of dysentery which can cause clinical confusion with other endemic diseases and may present to the surgeon as a result of its complications. A total of 140 children with Shigella dysenteriae type I presented to the paediatricians at King Edward VIII Hospital in 1995; 35 were referred to the surgeons because of abdominal tenderness, distension, peritonitis or perforation. Ten children underwent laparotomy--four for peritonitis and six for perforation. Of the four children with peritonitis, three had transmural colitis. Therefore laparotomy was only performed for objective evidence of perforation. Of the subsequent non-operated group with the clinical features of peritonitis, none developed further surgical problems in the acute phase and none died. It is suggested that surgery in the acute phase should be avoided unless there is evidence of perforation.

Abdominal Pain↗

Membranous atresia of the body of the stomach.

Pyloric and prepyloric webs and atresias have long been recognized and frequently reported; however, membranous atresia of the body of the stomach has not been reported previously. The authors report such a case.

Female↗

Intralingual cysts of foregut origin.

Intralingual cysts lined by epithelium of foregut origin are rarely reported. We present 16 patients seen over an 8-year period (1983 to 1990) with such lesions. Respiratory and squamous epithelium were the most commonly encountered. These unusual cysts generally presented in the neonatal period with feeding difficulty. Two children had multiple cysts. Histological varieties of epithelia are described and the clinical features are discussed. From our experience we conclude that these cysts are adequately treated by complete surgical excision through a sagittal glossal split. This can be safely done electively in the neonatal period. Cyst aspiration was found to be inadequate definitive treatment.

Cysts↗

Infantile hypertrophic pyloric stenosis in a Third-World environment.

Sixty-two consecutive patients with hypertrophic pyloric stenosis, presenting over a 6-year period, are reported. The sequelae of late presentation and diagnostic delay are outlined. Clinical diagnosis may be difficult. Barium meal examination has proved a more useful single investigation than ultrasonography, although these methods of investigation are complementary. Management difficulties include severe biochemical derangement, correction of which delays surgery. In black communities pyloric stenosis remains a rare condition and heightened clinical awareness remains the most important means of achieving earlier diagnosis.

Humans↗

Membranous atresia of the intra-abdominal oesophagus. A case report.

A premature neonate who presented with inability to feed is reported. The unusual radiological appearances stimulated further studies to define the level of atresia in the intra-abdominal oesophagus. A membranous atresia was disrupted using a thoraco-abdominal approach. However, this rare variant of oesophageal atresia is correctable through an abdominal approach and awareness may prevent unnecessary thoracotomy. Recovery was uneventful. Our patient is only the second reported with intra-abdominal membranous atresia of the oesophagus, and is the first reported survivor.

Esophageal Atresia↗

Small bowel volvulus complicating intestinal ascariasis in children.

Twenty-nine patients in whom small bowel volvulus complicated intestinal ascariasis are reported. Attention is drawn to the varied clinical and radiological presentation and the relationship of volvulus to the use of vermifuge. The mortality of 17 per cent was contributed to by delay in diagnosis and failure of pre-operative resuscitation. Early diagnosis is dependent upon clinical vigilance and repeated radiological assessment of those patients with simple ascaris obstruction who fail to improve rapidly with conservative treatment. The instillation of intraluminal vermifuge intra-operatively has minimized postoperative worm migration through suture lines and anastomoses.

Ascariasis↗

Eventration of the diaphragm.

Diaphragmatic eventration is a large or small area of the diaphragm consisting of fibrous tissue only. If small it is termed partial; if large, complete. The condition is infrequently recognised since it is seldom suggested on referral. A high index of suspicion in the interpretation of chest radiographs is often all that is needed, but occasionally more complex investigative procedures may be necessary. The records of patients seen in this unit are reviewed and discussed. Induction of a pneumoperitoneum was a safe procedure and diagnostic in 8 of 9 patients. All patients with complete eventration were advised to have surgery; in those in whom partial eventration was confidently diagnosed surgery was not recommended, but it was undertaken in 4 patients in whom a confident diagnosis was not made.

Adolescent↗