A meta-analysis of clinical outcome of intestinal transplantation in patients with total intestinal aganglionosis

Nakamura, Hiroki; Henderson, Davina; Puri, Prem

doi:10.1007/s00383-017-4107-2

Cited by 24 publications

(5 citation statements)

References 45 publications

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“…Short-segment disease, defined as aganglionosis to the junction of the descending colon and sigmoid colon, accounts for 60–85% of patients; long-segment disease (10% of patients) is characterised by aganglionosis proximal to the sigmoid colon with ganglion cells present in the colon; and total colonic aganglionosis is defined as aganglionosis of the entire colon extending into the terminal ileum (3–12%) [ 8 , 9 , 10 , 11 ]. Total intestinal aganglionosis is exceedingly rare, occurring in less than 1% of patients with HSCR, and is the most severe form of the disease [ 12 ].…”

Section: Introductionmentioning

confidence: 99%

A Novel Method for Identifying the Transition Zone in Long-Segment Hirschsprung Disease: Investigating the Muscle Unit to Ganglion Ratio

et al. 2022

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Hirschsprung disease (HSCR) is characterised by the absence of enteric ganglia along variable lengths of the distal bowel. Current gold standard treatment involves the surgical resection of the defective, aganglionic bowel. Clear and reliable distinction of the normoganglionated bowel from the transition zone is key for successful resection of the entire defective bowel, and the avoidance of subsequent postoperative complications. However, the intraoperative nature of the tissue analysis and the variability of patient samples, sample preparation, and operator objectivity, make reproducible identification of the transition zone difficult. Here, we have described a novel method for using muscle units as a distinctive landmark for quantifying the density of enteric ganglia in resection specimens from HSCR patients. We show that the muscle unit to ganglion ratio is greater in the transition zone when compared with the proximal, normoganglionated region for long-segment HSCR patients. Patients with short-segment HSCR were also investigated, however, the muscle unit to ganglion ratio was not significantly different in these patients. Immunohistochemical examination of individual ganglia showed that there were no differences in the proportions of either enteric neurons or glial cells through the different regions of the resected colon. In addition, we identified that the size of enteric ganglia was smaller for patients that went on to develop HSCR associated enterocolitis; although the density of ganglia, as determined by the muscle unit to ganglia ratio, was not different when compared with patients that had no further complications. This suggests that subtle changes in the enteric nervous system, even in the “normoganglionated” colon, could be involved in changes in immune function and subsequent bacterial dysbiosis.

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Section: Introductionmentioning

confidence: 99%

A Novel Method for Identifying the Transition Zone in Long-Segment Hirschsprung Disease: Investigating the Muscle Unit to Ganglion Ratio

et al. 2022

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“…Intestinal transplantation represents the only alternative treatment to achieve intestinal autonomy in this patient population, and promising results have been reported after intestinal transplantation in conjunction with parenteral nutrition. Intestinal transplantation should thus be considered as a feasible treatment option for patients with TIA [12] .…”

Section: Discussionmentioning

confidence: 99%

Jejunal perforation as an unusual presentation of total colonic aganglionosis in a neonate: A case report

Han

Kim

Moon

2017

International Journal of Surgery Case Reports

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HighlightsUntreated distal bowel obstruction eventually leads to perforation near the obstruction.Total colonic aganglionosis can present as a proximal jejunal perforation.Distal bowel should also be sought in cases of proximal bowel perforation.Appendiceal biopsy can be a reliable tool for suspecting total colonic aganglionosis.Multiple colon and rectal biopsies are necessary to diagnose total colonic aganglionosis.

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“…Diese Kinder be-dürfen einer Langzeit-TPN und müssen in spezialisieren Zentren für chronisches Darmversagen behandelt werden. Therapieoptionen reichen hier bis zur Darmtransplantation (18).…”

Section: Gibt Es Risikogruppen?unclassified

Morbus Hirschsprung

Lacher¹

2018

Kinder- und Jugendmedizin

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ZusammenfassungDer Morbus Hirschsprung ist ein häufiges kinderchirurgisches Krankheitsbild.Auch wenn eine Vielzahl von genetischen Mutationen beschrieben wurde, besteht die Trias der Diagnosestellung weiterhin in der sorgfältigen Anamnese, Kolon-Kontrastmitteluntersuchung und Rektumbiopsie.Die Durchzugsoperation hat zum Ziel, den aganglionären Darm zu entfernen und die intestinale Passage durch Anastomosierung des innervierten Darms mit dem Analkanal unmittelbar oberhalb der Linea dentata zu rekonstruieren, sodass der Analkanal und die Sphinkterfunktion erhalten bleiben. Zu den häufigsten Techniken gehören die Operationen nach Swenson, Soave (de la Torre) und Duhamel. Diese können auch laparoskopisch assistiert durchgeführt werden.Obwohl der Morbus Hirschsprung durch Resektion des aganglionären Darmes primär gut zu behandeln ist, ist die Langzeitmorbidität hoch. Diese wird vor allem durch die fort-bestehende Obstipation bis hin zur Stuhlinkontinenz oder rezidivierende Episoden einer Enterokolitis bestimmt. Durch eine zielgerichtete Diagnostik und multidisziplinäre Behandlung können diese Symptome meist im Verlauf mehrerer Jahre erfolgreich behandelt werden.Der Pädiater spielt beim Erkennen und Management dieser Kinder eine wichtige Rolle.

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A meta-analysis of clinical outcome of intestinal transplantation in patients with total intestinal aganglionosis

Abstract: ITx appears promising in the management of TIA. ITx can be considered a feasible treatment option for patients with TIA who suffer from life-threatening complications of intestinal failure.

Cited by 24 publications

References 45 publications

A Novel Method for Identifying the Transition Zone in Long-Segment Hirschsprung Disease: Investigating the Muscle Unit to Ganglion Ratio

A Novel Method for Identifying the Transition Zone in Long-Segment Hirschsprung Disease: Investigating the Muscle Unit to Ganglion Ratio

Jejunal perforation as an unusual presentation of total colonic aganglionosis in a neonate: A case report

Morbus Hirschsprung

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