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Hirschsprung disease is a relatively geometriaa condition managed by pediatric surgeons. Significant advances have been made in understanding its etiologies in the last decade, especially with the explosion of molecular genetic techniques and early diagnosis. The surgical management has progressed from a two- or three-stage procedure to a primary operation.

More recently, definitive surgery for Hirschsprung disease through minimally invasive techniques has gained popularity.

In neonates, the advancement of treatment strategies for Hirschsprung disease continues with reduced patient morbidity and improved outcomes. Motility disorders and Hirschsprung disease.

Nelson Textbook of Pediatrics. Hyperganglionosis mimicking Hirschsprung ‘s disease. Three patients with hyperganglionosis are reported in whom an initial diagnosis of Hirschsprung ‘s disease was suspected.

In one patient there was a classic presentation with constipation, in another Hirschsprung ‘s disease coexisted, and in the third the initial inadequate suction rectal biopsy specimen was suggestive of Hirschsprung ‘s disease on acetylcholinesterase staining.

Evidence of hypertrophy and hyperplasia of the intermuscular and submucosal plexuses on a full thickness bowel biopsy specimen was used to confirm the diagnosis of hyperganglionosis, suggested by the characteristic demonstration of moderate increase in the number of acetylcholinesterase stained nerve fibres in the lamina propria mucosae on rectal biopsy.

Surgical management was guided by clinical signs. Two patients had colonic resections; the third had temporary stomal diversion. Hyperganglionosis is rarer than Hirschsprung ‘s disease but is known to mimic it. We suggest full thickness bowel specimens are needed to confirm the diagnosis and that inadequate rectal suction biopsies must be interpreted with caution.

Redo pullthrough for Hirschsprung disease. Pullthrough procedures for Hirschsprung diseases typically have favorable results. However, some children experience long-term postoperative complications comprising stooling disorders, such as intermittent enterocolitis, severe stool retention, intestinal obstruction, as well as incontinence.

Reoperative Hirschsprung Disease surgery is complex. This begins with the workup after the initial presentation following primary pullthrough, continues with the definitive surgical correction with redo pullthrough, and ends with long-term follow-up of individuals. The decision tree can be varied with each patient. The operating pediatric surgeon must be able to utilize different operations and treatment options available.

While lesser procedures may provide relief in a select population, those with residual aganglionosis or transition zone pathology or mechanical problems will likely require a redo pullthrough.

Thus, the diagnostic workup, treatment plan, and definitive surgical care should be coordinated, and executed by an experienced, specialized team at a pediatric referral center. Hirschsprung ‘s disease is marked by constipation from the time of birth, with the development, if uncorrected, of a protuberant abdomen and flared costal margins.

The rectal ampulla is empty and the abdomen is filled with fecal masses. Pain is not prominent. Flatus is passed in large amounts. Encopresis does not occur. Barium enema shows the characteristic narrowed distal rectal segment and biopsy of the rectum shows absence of the ganglion cells of the myenteric plexus.

Treatment is operative resection of the distal narrow segment and a primary anastomosis. Hirschsprung ‘s disease may be mimicked in children with: Psychogenic constipation—pseudo- Hirschsprung ‘s disease.

Unlike Hirschsprung nakwmura disease, symptoms do not appear at birth, encopresis is common, and the barium enema shows no narrow distal segment. Mental retardation and cerebral defect. Corrected imperforate anus—on the basis of stenosis, imperfect innervation or poor habit training. Cretinism—with severe constipation and intestinal dilatation perhaps the presenting symptoms. Treatment of these four groups of children with severe constipation not due to Hirschsprung ‘s disease is: For Group 1, open discussion with parent and child.


Assumption by the physician of full control of the details of treatment, and relegation of parent to the role of the physician’s agent in following the prescribed regimen.

For Group 2, an enema regimen. Whereas fairly rapid restoration and then geojetria of normal bowel habit can be expected in Group 1, the basic defects in Group 2 may require indefinite continuation of treatment. For Group 3, regular dexcriptiva regimen, in the less severe cases—one identical with that makamura in Group 1, and dilatation of strictures or descripfiva.

In Group 4, thyroid hormone therapy relieves the constipation of hypothyroidism and causes reversion of radiographic changes in the colon and rectum. La Memoria De Nuestra Tierra: La Memoria de Nuestra Tierra combines a meticulously hand-painted landscape with historic photographs in a seamless blend imprinted on the holographic-like surface of a metallic coated substrate.

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However, this procedure is limited with only hematoxylin and eosin staining, especially in biopsies of premature babies or when non-expert pathologists make the evaluation. The immunohistochemistry from ganglia cell calretinin has been used to reduce the risk of misdiagnosis.

Our objective was to show the benefits of this antibody in diagnosis of Hirschsprung ‘s disease in biopsy specimens. We determined if there was enough paraffin block for immunohistochemistry with two markers: Three controls of autopsy of children under 3 geojetria of age with other diagnosis were included.

Geometriw immunohistochemistry confirmed the descriphiva in nine cases. In the other four cases there were initial misdiagnosis due to evidence of calretinin ganglion cells and, thus, Hirschsprung ‘s disease was discarded. Hirschsprung disease and hepatoblastoma: Hirschsprung disease is a desxriptiva disorder of the enteric nervous system that is characterized by absence of ganglion cells in the distal intestine, and it occurs in approximately 1 in everylive births.

Hepatoblastoma is a malignant liver neoplasm that usually occurs in children aged 6 months to 3 years, with a prevalence of 0. A boy diagnosed with intestinal atresia in the first week of life progressed to a diagnosis of comorbid Hirschsprung disease. Congenital cataracts and sensorineural deafness were diagnosed.

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It is known that Hirschsprung disease may be associated with syndromes predisposing towards cancer, and that hepatoblastoma may also be associated with certain congenital syndromes. However, co-occurrence of hepatoblastoma and Hirschsprung disease has not been previously described. We have reported a case of a male patient born with ileal atresia, Hirschsprung disease and bilateral congenital cataract who was later diagnosed with hepatoblastoma.

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Higher education degree attainment for…. The pathogenesis of Hirschsprung ‘s disease-associated enterocolitis. Hirschsprung ‘s disease-associated enterocolitis HAEC remains the most life-threatening complication in Hirschsprung disease HD patients. These include a possible causal relationship between the abnormal enteric nervous system development in HD and the development of enterocolitis.

Based on the complex genetic causes of HD that have been discovered and the resultant heterogeneous group of patients that exists, the causes of HAEC are likely multiple. New insights regarding the relationship of the role of the enteric nervous system and its interaction between intestinal barrier function, innate host immunity, and commensal microflora have been discovered, which may shed light on this perplexing problem. Published by Elsevier Inc. A bridge for science and surgery.

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Understanding the true nature of the disease provided the basis for appropriate surgery for Hirschsprung ‘s disease some 60 years ago. Nevertheless, surgical outcome remains unsatisfactory. Advances in diagnosis and treatment will depend on the elucidation of the pathogenesis and disease heterogeneity.

This lecture outlines the author’s attempt in the past 30 years to bridge some of the gaps of knowledge in Hirschsprung ‘s disease. Studies of human fetal gut and aganglionic gut gave insight into the complexity of the human enteric nervous system, but the more fruitful studies came from genetic studies in which disease-causing genes were discovered, and the importance of noncoding mutations conferring disease susceptibility was unraveled.

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Animal models and pluripotent stem cell studies allowed elucidation of the interacting gene-cell-microenvironment signaling pathways for desscriptiva crest proliferation, migration, and differentiation. Hirschsprung ‘s disease has been a bridge for science and surgery. An integrative approach could provide breakthroughs in the diagnosis and treatment strategies of this complex condition, leading to improved outcome.

This case report presents an incident of rectal carcinoma in a year-old man with Hirschsprung ‘s disease, for which he was operated in his early nzkamura, with a Soave pull-through procedure. No direct association between Hirschsprung ‘s disease and rectal cancer was found in our review of the literature. However, several case reports of rectal cancers following pull-through procedures exist.

A low threshold for further clinical investigations is recommended, if these patients are presenting with gastrointestinal symptoms. Surgical management of recto-sigmoid Hirschsprung ‘s disease. Over the years, the surgical management of recto-sigmoid Hirschsprung ‘s disease HD has evolved radically and at present a single stage transanal pull-through can be done in suitable cases, which obviates the need for multiple surgeries. The aim of this paper was to evaluate the role of transanal pull-through in the management of recto-sigmoid HD in our institution.

A retrospective analysis between January and December was carried out on all cases of Hirschsprung ‘s reporting to unity of pediatric surgery of Tunis Children’s Hospital that were managed by transanal pull-through as a definitive treatment. All selected patients including neonates had an aganglionic segment confined nakamur the rectosigmoid area, confirmed by preoperative barium enema and postoperative histology. Transanal pull-through was performed in 31 children. Mean operating time was minutes range 64 grometria minutes.

No patients required laparotomy because all patients including neonates had an aganglionic segment confined to the rectosigmoid area.

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Blood loss ranged between 20 to 56 ml without blood replacement. Since all children were given an epidural caudal block, the requirement of analgesia in these cases was minimal. Postoperative complications included perianal excoriation in 7 out of 31 patients lasting from 3 weeks to 6 months. Transanal endorectal pull-through procedure for the management of rectosigmoid HD is now a well-established and preferred approach.

Parental satisfaction is immense due to the lack of scars on the abdomen. As regards the continence, a long-term follow-up is necessary to appreciate better the functional results of this surgery.

Hirschsprung ‘s disease is the most common cause of pediatric intestinal obstruction.