<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1665-1146</journal-id>
<journal-title><![CDATA[Boletín médico del Hospital Infantil de México]]></journal-title>
<abbrev-journal-title><![CDATA[Bol. Med. Hosp. Infant. Mex.]]></abbrev-journal-title>
<issn>1665-1146</issn>
<publisher>
<publisher-name><![CDATA[Instituto Nacional de Salud, Hospital Infantil de México Federico Gómez]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1665-11462026000200087</article-id>
<article-id pub-id-type="doi">10.24875/bmhim.24000139</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Hirschsprung's disease and Mowat-Wilson syndrome: should a pull-through be performed?]]></article-title>
<article-title xml:lang="es"><![CDATA[Enfermedad de Hirschsprung y síndrome de Mowat-Wilson: ¿debería realizarse un descenso transanal?]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Martain-Pérez]]></surname>
<given-names><![CDATA[Itzamara]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Dávila-Pérez]]></surname>
<given-names><![CDATA[Roberto]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Fernández-Portilla]]></surname>
<given-names><![CDATA[Emilio]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Lizárraga-Rodríguez]]></surname>
<given-names><![CDATA[Itzel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Moreno-Salgado]]></surname>
<given-names><![CDATA[Rodrigo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Nieto-Zermeño]]></surname>
<given-names><![CDATA[Jaime]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Costa-Roig]]></surname>
<given-names><![CDATA[Adria]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Domínguez-Muñoz]]></surname>
<given-names><![CDATA[Alfredo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Department of Pediatric Surgery Colorectal Center ]]></institution>
<addr-line><![CDATA[Mexico City ]]></addr-line>
<country>Mexico</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Infantil de México Federico Gómez Department of Genetics ]]></institution>
<addr-line><![CDATA[Mexico City ]]></addr-line>
<country>Mexico</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>04</month>
<year>2026</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>04</month>
<year>2026</year>
</pub-date>
<volume>83</volume>
<numero>2</numero>
<fpage>87</fpage>
<lpage>93</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S1665-11462026000200087&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S1665-11462026000200087&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S1665-11462026000200087&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract  Background: Hirschsprung's disease (HSCR) is characterized by the absence of ganglion cells. Five percent of cases are associated with syndromic conditions, one of which is Mowat-Wilson syndrome (MWS), with an incidence rate of 50%. HSCR may be the first feature of this syndrome to be diagnosed. MWS is an autosomal dominant genetic disorder caused by a variant in the ZEB2 gene (ZFHX1B). OMIM #235730. It involves severe clinical manifestations such as ocular hypertelorism, intellectual disability, congenital heart defects, epilepsy, and HSCR. The association between MWS and HSCR is regarded as a serious condition with unpredictable post-operative outcomes, and many reported complications related to motility disorders are noted.  Methods: We conducted a retrospective study and reviewed the medical records of patients with MWS treated at our center. We examined the relationship among HSCR, clinical features, molecular characteristics, surgical complications, and pre-operative and post-operative enterocolitis events.  Results: The study included four patients with MWS. Three (75%) were found to be associated with HSCR. Rectal biopsy confirmed HSCR in all patients. Two patients underwent a transanal pull-through Swenson procedure, and both experienced surgical complications. Both cases encountered multiple episodes of enterocolitis, and one of them required a permanent stoma. The third patient has not undergone surgical correction but has responded well to medical treatment (laxatives).  Conclusions: The association between MWS and HSCR presents a severe condition with high morbidity. The outcome after the pull-through procedure is unpredictable. Further studies are necessary to gain a deeper understanding of this condition. We recommend evaluating these patients in a multidisciplinary consensus based on the existing literature and our findings. Those without recurrent enterocolitis or chronic motility disorders are suitable candidates for conservative management.]]></p></abstract>
<abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen  Introducción: La enfermedad de Hirschsprung es una entidad caracterizada por ausencia de células ganglionares; en el 5% de los casos llega a ser sindrómica. Uno de los síndromes más asociados (hasta 50%) es el síndrome de Mowat-Wilson, que es una enfermedad genética con variante en el gen ZEB2 (ZFHX1B), OMIM #235730. Localizada en el cromosoma 2 (2:144,384,081), esta asociación sindrómica se considera una condición grave con resultados poco predecibles y complicaciones posquirúrgicas graves hasta en el 80% de los pacientes.  Métodos: Se llevó a cabo una revisión retrospectiva de registros hospitalarios de pacientes con diagnóstico molecular de Mowat-Wilson tratados en nuestra institución. Se analizaron los siguientes datos: asociación con enfermedad de Hirschsprung, características clínicas, estudio molecular, complicaciones quirúrgicas y eventos de enterocolitis preoperatorios y postoperatorios.  Resultados: Se incluyeron cuatro pacientes con variantes patogénicas en ZEB2 con diagnóstico de Mowat-Wilson (tres de ellos [75%] asociados a enfermedad de Hirschsprung). Dos de los tres pacientes se detectaron en la etapa neonatal, cursando con eventos de enterocolitis; ambos se sometieron a descenso transanal tipo Swenson y ambos requirieron un redescenso transanal secundario a enterocolitis de repetición. El 100% cursó con complicaciones postoperatorias. La cuarta paciente ha cursado asintomática, sin tratamiento quirúrgico.  Conclusiones: La asociación de enfermedad de Hirschsprung con Mowat-Wilson es una condición grave con alta morbilidad, con evolución posterior al descenso transanal poco predecible. A pesar de que se necesitan más estudios en el futuro recomendamos que el abordaje de los pacientes con esta asociación sea de manera multidisciplinaria y aquellos sin episodios frecuentes de enterocolitis son buenos candidatos para tratamiento conservador.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Enfermedad de Hirschsprung]]></kwd>
<kwd lng="es"><![CDATA[Síndrome de Mowat-Wilson]]></kwd>
<kwd lng="es"><![CDATA[ZEB2]]></kwd>
<kwd lng="en"><![CDATA[Hirschsprung's disease]]></kwd>
<kwd lng="en"><![CDATA[Mowat-Wilson syndrome]]></kwd>
<kwd lng="en"><![CDATA[ZEB2]]></kwd>
</kwd-group>
</article-meta>
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