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INTRODUCCIÓN: El piometra es una afección infrecuente, pero grave, que en general se diagnostica en mujeres posmenopáusicas. En adolescentes es sumamente raro, y si se acompaña de amenorrea primaria hay que tener en mente las anomalías congénitas. CASO CLÍNICO: Adolescente de 13 años, sin antecedentes personales de interés salvo amenorrea primaria, que acude con abdomen agudo y es intervenida por una peritonitis difusa causada por un piometra secundario a disgenesia (estenosis) cervical congénita. Se realizó dilatación cervical y se dejó una sonda vesical intrauterina para prevenir la reestenosis. CONCLUSIONES: Un diagnóstico precoz y un tratamiento conservador con dilatación cervical y colocación temporal de un catéter urinario son esenciales para un manejo seguro y efectivo de la estenosis cervical en adolescentes.

INTRODUCTION: Pyometra is an uncommon but serious condition that is generally diagnosed in postmenopausal women. In adolescents it is extremely rare; if accompanied by primary amenorrhea, consider congenital abnormalities. CASE REPORT: A 13-year-old adolescent, with no relevant personal history except primary amenorrhea, who presented with an acute abdomen and was operated on for diffuse peritonitis caused by pyometra secondary to congenital cervical dysgenesis (stenosis). Cervical dilation was performed and a urinary catheter was temporarily placed inside the uterus to prevent restenosis. CONLUSIONS: An early diagnosis and conservative treatment with cervical dilation and temporary placement of a urinary catheter are essential for the safe and effective management of cervical stenosis in adolescents.

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BACKGROUND: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of nasal obstruction in neonates with respiratory distress manifestations. Diagnosis is made with craniofacial computed tomography, prompt and precise treatment creates good outcomes in these patients. AIM: To present our experience in diagnosis and management considerations with this rare pathology and a case series of our surgically managed patients. METHODS: A retrospective, analytical study of CNPAS patients surgically managed over a period of seven years. Evaluation and follow up was reviewed. RESULTS: Thirteen patients were evaluated; pyriform aperture mean width was 5.5mm. Of these patients, 31% also had Congenital Midnasal Stenosis. Medical treatment failed for all the patients and they required surgical enlargement of the pyriform aperture. No complications were seen and all patients improved in symptoms and development. CONCLUSIONS: Congenital nasal obstruction can be fatal in new-borns, CNPAS is a rare differential diagnosis that must be completely evaluated, properly treated with conservative management and if it fails, with well-planned and executed surgery. Follow-up shows high rates of success.

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Existe un amplio espectro de deformidades causadas por bandas amnióticas, siendo las extremidades la ubicación más común. La banda de constricción en el abdomen es un lugar raro para el síndrome de brida amniótica. Presentamos un caso de banda de constricción circunferencial congénita del abdomen sin otras malformaciones. La condición no afectó la ventilación, la alimentación, las deposiciones o el crecimiento. Se planificaron múltiples Z-Plasties en el abdomen antes del primer mes de vida. Revisamos la literatura que hace hincapié en la presentación clínica y el tratamiento de esta rara entidad clínica.

There is a wide spectrum of deformities caused by amniotic bands, being the extremities the most common location. The constriction band in the abdomen is a rare location for constriction band syndrome. We report a case of congenital circumferential constriction band of abdomen without other malformations. The condition did not affect ventilation, feeding, bowel movements, or growth. Multiple Z-plasties were planned in the abdomen before the first month from birth. We reviewed the literature emphasizing on the clinical presentation and management of this rare clinical entity.

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El síndrome del incisivo central único de la línea media del maxilar es un trastorno raro que implica anomalías de la línea media, como holoprosencefalia, anomalías de las fosas nasales, fisura palatina, labio leporino, hipotelorismo, microcefalia y panhipopituitarismo. La estenosis congénita del orificio nasal anterior es una causa mortal de dificultad respiratoria neonatal debido al estrechamiento del orificio nasal anterior, y podría confundirse con la atresia de coanas. En este informe, presentamos el caso de un recién nacido con síndrome del incisivo central único de la línea media del maxilar acompañado de otras anomalías, tales como holoprosencefalia, estenosis del orificio nasal anterior, microcefalia y panhipopituitarismo. El cariotipado mostró una deleción heterocigota en el gen SIX3 en la región 2p21, que produjo una forma más grave de holoprosencefalia.

Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities such as holoprosencephaly, nasal cavity anomalies, cleft palate-lip, hypotelorism, microcephaly, and panhypopituitarism. Congenital nasal pyriform aperture stenosis is a lethal cause of neonatal respiratory distress due to narrowing of the pyriform aperture anteriorly and it can be confused with choanal atresia. In this report, we present a newborn infant with solitary median maxillary central incisor syndrome accompanied by other abnormalities including holoprosencephaly, nasal pyriform aperture stenosis, microcephaly and panhypopituitarism. Chromosomal analysis showed heterozygous SIX3 gene deletion at 2p21 region resulting in a more severe form of holoprosencephaly.

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Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities such as holoprosencephaly, nasal cavity anomalies, cleft palate-lip, hypotelorism, microcephaly, and panhypopituitarism. Congenital nasal pyriform aperture stenosis is a lethal cause of neonatal respiratory distress due to narrowing of the pyriform aperture anteriorly and it can be confused with choanal atresia. In this report, we present a newborn infant with solitary median maxillary central incisor syndrome accompanied by other abnormalities including holoprosencephaly, nasal pyriform aperture stenosis, microcephaly and panhypopituitarism. Chromosomal analysis showed heterozygous SIX3 gene deletion at 2p21 region resulting in a more severe form of holoprosencephaly.

El síndrome del incisivo central único de la línea media del maxilar es un trastorno raro que implica anomalías de la línea media, como holoprosencefalia, anomalías de las fosas nasales, fisura palatina, labio leporino, hipotelorismo, microcefalia y panhipopituitarismo. La estenosis congénita del orificio nasal anterior es una causa mortal de dificultad respiratoria neonatal debido al estrechamiento del orificio nasal anterior, y podría confundirse con la atresia de coanas. En este informe, presentamos el caso de un recién nacido con síndrome del incisivo central único de la línea media del maxilar acompañado de otras anomalías, tales como holoprosencefalia, estenosis del orificio nasal anterior, microcefalia y panhipopituitarismo. El cariotipado mostró una deleción heterocigota en el gen SIX3 en la región 2p21, que produjo una forma más grave de holoprosencefalia.

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En este artículo se reporta el caso de un lactante menor de 2 meses de edad que a los 15 días de nacido presentó estridor y dificultad respiratoria, por lo que consultó a una clínica de primer nivel en donde se le realizó radiografía de tórax, en la cual se documentó la presencia de una hiperinfiación del campo pulmonar derecho. Se realizó una fibrobroncoscopía óptica (FBO) que fue reportada como normal y una gammagrafía de ventilación/perfusión que reportó una hipoplasia pulmonar derecha. Fue manejado con oxígeno suplementario y terapias respiratorias sin mejoría clínica, motivo por el cual fue trasladado a nuestra institución para manejo especializado. Con el fin de obtener un mapa vascular pre quirúrgico, se realizó una angiografía pulmonar por tomografía (Figura 2) reconstrucciones con mínima intensidad de proyección (Figura 3) y broncoscopía virtual (BV) multicorte (Figuras 4 y 5) documentándose una estenosis del bronquio fuente derecho, producida por un repliegue de la pared bronquial y con efecto de válvula que producía una hiperinsufiación secundaria del pulmón derecho. Estos hallazgos fueron confirmados en la cirugía correctiva.

This article describes the case of an infant under 2 months old, who at 15 days of age presented stridor and respiratory distress, a chest X-ray was performed which reported the presence of a hyperinflation of the right lung field. A fiberoptic bronchoscopy (FBO) was performed which resulted normal and a ventilation/perfusion gammagraph showed a right lung hypoplasia. The infant was treated with additional oxygen and respiratory therapy without clinical. In order to obtain a pre-surgical vascular map, a multislice CT angiography and virtual bronchoscopy were performed, documenting a right bronchial stenosis produced by a fold of the bronchial wall and with valve effect which produced a secondary hyperinsufflation of the right lung. These findings were confirmed in corrective surgery.

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El cornaje es un ruido generado por la alteración del flujo de aire al pasar por las fosas nasales. La principal causa en recién nacidos y lactantes es la rinitis inflamatoria o infecciosa. Con menor frecuencia, puede ser de origen congénito, neoplásico, traumático o iatrogénico.La estenosis congénita de la apertura piriforme es una etiología infrecuente de obstrucción nasal en el neonato. El diagnóstico precoz y el tratamiento apropiado son esenciales debido a surespiración nasal obligada. La dificultad o la imposibilidad de hacer progresar una sonda de 2,8 mm (sonda K30) a través de la región anterior de las fosas nasales, permiten sospecharla.El diagnóstico debe ser confirmado por una tomografía computada del macizo craneofacial. La conducta terapéutica dependerá de la gravedad de los síntomas.Describimos nuestra experiencia con 9 pacientes que presentaban esta patología cuya corrección quirúrgica fue exitosa.

Stertor is a noise generated by the disturbance of the air flow passing through the nose. Its main cause –in newborns and infants– is inflammatory or infectious rhinitis. Congenital,neoplastic, traumatic or iatrogenic causes are less frequent. Congenital stenosis of the pyriform aperture is a rare etiology of nasal obstruction in the neonates. Early diagnosis and appropriate treatment are essential because of their exclusive nasal breathing. Suspicion might arise when a difficulty or even an impossibility to pass a probe of 2.8 mm (K30 tube) through anterior nares, exists. Diagnosis should be confirmed by a computed tomography of the craniofacial massif. The therapeutic behavior will depend on the severity of symptoms. We describe our experience with nine patients with this conditionwhose surgical correction was successful.

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BACKGROUND: Colonic stenosis is rarely seen in pediatric practice. Its incidence has been reported as 1/40,000 births. Ascending and transverse colon are the most common sites affected. Symptoms may be present at birth. CLINICAL CASE: We present the case of a 2 year, 11 month old male. Since birth, the patient had symptoms of constipation, abdominal pain and abdominal distension. X-ray of the abdomen showed a dilated colon and foreign bodies on the right side. Barium enema was normal. The stenotic segment was resected. Foreign bodies were removed and alimentary continuity was restored by end-to-end anastomosis. The pathologist found a 5-cm lumen at the stenosis. Histological ganglion cells were present throughout the specimen. The patient evolved favorably postoperatively and was discharged 1 week after surgery. The patient has been in good health for 2 years. CONCLUSIONS: Colonic stenosis is uncommon. Abu-Judeh reported on one case and a review of the literature revealed eight cases of congenital colonic stenosis since 1941. Symptoms are abdominal distension, vomiting and constipation with symptom remission, making diagnosis difficult. This is the first report involving foreign bodies. Barium enema and colonoscopy are necessary for diagnosis. Resection of the stenotic segment with end-to-end anastomosis or colostomy is the treatment of choice. Laparoscopy is an alternative procedure.

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Introducción: La estenosis del colon es rara, con una incidencia de uno entre 40 mil nacimientos. El colon ascendente y transverso son los más afectados. Las manifestaciones clínicas pueden aparecer desde el nacimiento. Caso clínico: Niño de dos años 11 meses de edad, con cuadros periódicos de estreñimiento, dolor tipo cólico y distensión abdominal. La radiografía simple de abdomen mostró dilatación de asas y cuerpos extraños. El colon por enema fue normal. Por laparotomía exploradora se identificó estenosis en el colon transverso. Se practicó resección, extracción de cuerpos extraños y anastomosis término-terminal. El examen histopatológico mostró estenosis con lumen de 0.5 cm; las células ganglionares estuvieron presentes entre las capas musculares. A dos años de la cirugía, el paciente se encontraba asintomático. Conclusiones: La estenosis congénita de colon es poco frecuente. Abdu-Judeh informa un caso y refiere ocho descritos desde 1941. Las manifestaciones clínicas son estreñimiento, vómito y distensión abdominal, sin embargo, los pacientes pueden permanecer asintomáticos por periodos variables, lo que dificulta y retrasa el diagnóstico. No existe informe previo acerca de la retención de cuerpos extraños. El colon por enema y la colonoscopia son necesarios para el diagnóstico oportuno. El tratamiento electivo es la resección quirúrgica con anastomosis primaria o colostomía; una opción es la cirugía laparoscópica.

BACKGROUND: Colonic stenosis is rarely seen in pediatric practice. Its incidence has been reported as 1/40,000 births. Ascending and transverse colon are the most common sites affected. Symptoms may be present at birth. CLINICAL CASE: We present the case of a 2 year, 11 month old male. Since birth, the patient had symptoms of constipation, abdominal pain and abdominal distension. X-ray of the abdomen showed a dilated colon and foreign bodies on the right side. Barium enema was normal. The stenotic segment was resected. Foreign bodies were removed and alimentary continuity was restored by end-to-end anastomosis. The pathologist found a 5-cm lumen at the stenosis. Histological ganglion cells were present throughout the specimen. The patient evolved favorably postoperatively and was discharged 1 week after surgery. The patient has been in good health for 2 years. CONCLUSIONS: Colonic stenosis is uncommon. Abu-Judeh reported on one case and a review of the literature revealed eight cases of congenital colonic stenosis since 1941. Symptoms are abdominal distension, vomiting and constipation with symptom remission, making diagnosis difficult. This is the first report involving foreign bodies. Barium enema and colonoscopy are necessary for diagnosis. Resection of the stenotic segment with end-to-end anastomosis or colostomy is the treatment of choice. Laparoscopy is an alternative procedure.

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The congenital stenosis of pyriform aperture is an unusual cause of neonatal nasal obstruction. It is due to bony overgrowth of the nasal lateral process of the maxilla. Initially this narrowest part of nasal airway was considered an isolated deformity; subsequently the congenital Stenosis of pyriform aperture was thought to represent a microform of holoprosencephaly. In this report a male neonate had respiratory distress, cyclic cyanosis and apnea after delivery. The patient underwent surgical correction of pyriform stenosis by sublabial access. In the follow up, the patient had good evolution. The report of this deformity shows an important cause of neonatal nasal obstruction and its differential diagnosis with bilateral choanal atresia. Congenital stenosis of nasal pyriform aperture can be surgically corrected when necessary.

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A estenose congênita de abertura piriforme é causa rara de obstruçäo nasal no recém-nato descrita pela primeira vez em 1989 por BROWN et al relatou 6 casos, seguido por ARLIS et al que relatou outros 6 casos em 1992, totalizando 12 casos descrito na literatura até o momento. Esta anomalia produz sintomas e sinais clínicos de obstruçäo nasal, em recém-nascidos e crianças, similares aos encontrados na atresia coanal bilateral. A tomografia computadorizada confirma o diagnóstico e delimita a anomalia. Descrevemos um caso diagnosticado no Hospital de Clínicas da Universidade Federal do Paraná no ano de 1993, no serviço de otorrinolaringologia, tratado cirurgicamente por acesso sublabial

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Five newborn infants with evidence of nasal obstruction were shown to have congenital nasal stenosis. Conservative treatment, including temporary nasopharyngeal intubation in three, eventually resulted in symptomatic relief, so that surgery could be avoided.

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