Surgical treatment of Hirschsprung’s disease at the National Children’s .. Enfermedad de Hirschprung. Fantobal-Rojas A. Constipación crónica en el niño. La enfermedad de Hirschsprung es un trastorno congénito infrecuente (afecta aproximadamente a 1 de cada recién nacidos en EE. UU.) que provoca. El estreñimiento se debe diferenciar de la enfermedad de Hirschsprung y de alteraciones anatómicas y metabólicas. La evaluación clínica incluye una historia.
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Hirschsprung’s disease in Barbados a year review. To assess the surgical outcome of patients with Hirschsprung’s disease HD who were treated in Barbados, establish referral patterns and identify factors which can lead to better management. All patients with HD who had definitive surgery at the Queen Elizabeth Hospital, Barbados, over a year period between July and June were identified from the hospital records system. Data from patients notes were collected to establish demographics, including age at referral and definitive surgery, gender, presenting symptoms, surgical intervention s and definitive procedure.
Long term outcome was assessed by the use of a simple questionnaire which was completed by the authors after personal or telephone contact with patients, their parents or guardians or the referring physician.
Between July and June27 children were identified with HD. There were 20 males and seven females. Children who had definitive surgery for HD in Barbados using Martin’s modified Duhamelprocedure had an overall good long-term outcome comparable to international results.
Children referred from the surrounding Caribbean islands were referred at an older age, but their overall outcome was not worse than local Barbadian patients. Veinte eran varones y siete hembras.
Constipation is a common problem in children throughout the world, and the Caribbean is no exception. The challenge for healthcare providers throughout the islands of the Caribbean, especially in the smaller islands, is to identify children who do not have primary constipation, but are constipated from treatable underlying medical or surgical conditions, for example Hirschsprung’s disease HD.
It is then imperative that these children be referred as early as possible for specialist care, and once referred appropriately managed. This study outlines nioos referral patterns to ee centre in the Caribbean The Queen Elizabeth Hospital, Barbados which offers patients definitive surgery for HD and reports on their long term outcome. All patients with Hirschsprung’s disease HD who had definitive surgery at the said institution over a year period between July and June were identified from hospital records.
Data from patients’ notes were collected to establish demographics, including age at referral and at definitive surgery, gender, presenting symptoms, presence of any associated anomalies, family history, surgical intervention s and definitive procedure. The long-term outcome of all patients without total colonic HD who had the same definitive procedure Martin’s modified Duhamel procedure by the same surgeon was then assessed. Long term outcome was assessed by the use of a simple questionnaire completed by the junior author MVV after personal or telephone contact with patients, their parents or guardians or the referring physician.
None of the questionnaires were completed by the nos surgeon SUJ. The questionnaire sought to assess the following: Over a year period, between July and June27 children were identified with Hirschsprung’s disease HDincluding twenty males and seven females – [male: The mean age at presentation was 30 months ranging from day 1 of life to 24 years. Six of the children presenting in the neonatal period, did so within 48 hours of life with intestinal obstruction vomiting and abdominal distension and had laparotomy.
The majority of children presented in the neonatal period 10the rest being referred during infancy 7childhood 8 and adulthood 1. All the children who presented in the neonatal period with one exception were local Barbadians, with children hirschspgung from outside Barbados tending to be referred at later ages Table 2.
The mean age at presentation for children in Barbados was four months range from day one of life to 32 months compared with a mean of 62 months ranging from day one of life to 24 years for referrals outside Barbados.
There were no associated chromosomal anomalies, however one child had congenital auricular atresia. Three of the children had a positive family history of HD.
Two children had cousins with HD and one child had an older sibling with the condition. The mean time to follow-up was 64 months ranging from two to months. The 11 children excluded from long-term follow-up included two with total colonic HD – one of whom had definitive surgery elsewhere, three who were lost to follow-up, one child whose parents declined to take part in the study, one child awaiting definitive surgery, one child who had a stoma at the time of study and three who did not have nois Martin’s modified Duhamel procedure as definitive surgery for HD.
[Hirschsprung’s disease: the immunohistochemistry as ancillary method for the diagnosis.]
These latter children had the following procedures: The child who required a stoma was one of three children in the series who suffered from chronic constipation following that pull-through procedure. This child had persistent constipation and abdominal distention unresponsive to medical management, and would most likely necessitate a repeat pull-through procedure in the future.
The other two children were able to maintain regular bowel habits with the use of regular laxatives 1 and enemas 1. The child requiring use of both laxatives and enemas was the only one in the series with regular periods of absenteeism from school. These two children were 26 months and 38 months at the time of assessment and were 38 months and 22 months post definitive surgery. The two children with Grade 1V continence were both developmentally delayed, one child having cerebral palsy with a perinatal history of maternal pregnancy-induced hypertension and fetal distress at birth and the other having congenital hydrocephalus.
None of the children experienced diarrhoea on long term follow-up.
The majority of patients with Hirschsprung’s disease HD have a satisfactory outcome after definitive surgery 3. However, despite advances in surgical procedures, some patients continue to have signs of persistent bowel dysfunction even in the presence of adequate resection of the aganglionic segment.
The aetiology of the persistent bowel dysfunction in these patients remains controversial, but is noted to improve with age, in particular fecal incontinence 4. In the study group of patients, the majority were fully continent Wingspread Groupl at a mean follow-up of 64 months. Only two children of hirschzprung 16 assessed soiled all the time [Wingspread Group 1V] Table 3.
These two children were well within the mean time to follow-up for this group of patients 64 months and in addition both were developmentally delayed and so the reason for their incontinence may be multi-fold. The surgical treatment of HD has evolved over the past 20 years from traditional multi-stage procedures to enfemedad pull-through techniquesand more recently to laparoscopic-assisted approaches 9, There is ample evidence that the primary pull-through procedure for HD is safe and effective in most situations, including the neonatal period 8.
The rate of complications for ne stage procedures is comparable to that seen in patients having a preliminary stoma but without the associated added complications of stomas.
Hence it is claimed that the single-stage repair is ideal for use in developing countries where access to medical enfermedxd may be hampered by poor transportation and ehfermedad systems 8. But how safe and feasible are these newer techniques for developing countries like the Caribbean? While transportation and hirdchsprung communication systems may indeed be a challenge for the Caribbean, especially in the presence of multiple small islands, a number of factors make routine use of the primary pull-through procedure inappropriate for the Caribbean, if not impossible.
These include the limited number of paediatric surgical specialists and support staff and lack of facilities and equipment for the handling of frozen section specimens hirdchsprung well as paediatric pathologists trained to read and interpret frozen section specimens. False-positive intra-operative frozen sections reported in centres where experienced pathologists are unavailable can have serious consequences Our institution had one experience of having had a patient as outlined above who had a total colectomy and Soave pull-through procedure for presumed total colonic HD based on frozen section analysis which was later not substantiated on paraffin section.
Enfermedad de Hirschsprung | Women on Wellness
For this reason, the senior author SUJ prefers not to perform primary pull-through dw. In addition, as evident in this study, a significant number of children enn diagnosed with HD outside the neonatal period, and by the time of referral have well-established megarectum or megacolon, which are contraindications to the primary pullthrough procedure 9.
None of the children in this series had a primary pull-through procedure. Njos 8 has claimed that the following factors have all contributed to earlier diagnosis of HD, which has resulted in increasing interest in performing a one-stage procedure over the last several decades: In reality, however, many of these factors continue to be lacking in the majority of the Caribbean isles resulting in late diagnosis and referral of children with HD. However, many factors outlined by Somme 8 which will affect earlier diagnosis and referral have improved and will continue to improve throughout the Caribbean.
For example, awareness of HD will improve with increased educational and training ejfermedad created both locally and abroad. With respect to Somme’s 8 finding of using a rectal suction biopsy as a factor enfermedaad to earlier diagnosis, we note the delay in diagnosis of some of the children who presented in the neonatal period Table 4.
The recently acquired rectal suction biopsy at the Queen Elizabeth Hospital ought to help in early diagnosis, but also eliminates the need for an open rectal biopsy, which has to be performed under a general anaesthesia unlike a rectal suction biopsy which can be performed without general anaesthesia.
Bruce et al 16 have reported on their preliminary experience with the use of the rectal suction biopsy some 10 years ago and have recommended its use in the Caribbean setting. During the review of patients’ records in this study, it was clear that a number of children who initially presented in the eb period and re-presented later in childhood were being treated for enfermdead enterocolitis NEC in the neonatal enfermrdad when most likely they were experiencing Hirschsprung’s enterocolitis.
It is a known fact that the clinical features of NEC – foul-smelling, explosive diarrhoea, often bloody in hirschpsrung, abdominal distention, hypovolaemic shock, vomiting, fever and lethargy may mimic that of Hirschsprung’s enterocolitis. Hence NEC in the term infant should raise one’s suspicion and a rectal suction biopsy performed to exclude HD In conclusion, Hirschsprung’s disease is a rare cause of constipation and can be successfully managed in Barbados with long-term results comparable to that seen both regionally 18 and internationally 4, 19, 20 despite the availability of limited resources and specialists personnel.
However, use of the more modern definitive surgical procedures, most of which are laparoscopically-assisted, singlestage procedures cannot be safely adapted in Barbados at present. Bowel function, mental health, and psychosocial function in adolescents with Hirschsprung’s disease.
Enfermedad de Hirschsprung | Neurology Associates of Kansas
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