Neonatal splenic necrosis not related to wandering spleen.

Splenic necrosis is extremely rare in neonates, the cases recorded so far being secondary to torsion of wandering organs. A newborn with an abdominal mass who underwent exchange transfusions through an umbilical catheter is presented here. Comprehensive investigation led to the suspicion of enteric duplication, but a splenic necrosis with no features of wandering spleen was found at laparotomy. The pathogenesis and preoperative diagnostic work-up of splenic necrosis are emphasised.

Congenital prepubic sinus.

Prepubic sinuses are rare congenital anomalies of the urinary tract. There have been few case reports in the literature. The authors describe 2 clinical cases of this entity. A review of the 20 cases previously reported, to clarify the anatomic features and the embryology of this infrequent congenital anomaly, also is reported. Furthermore, the authors propose to classify these lesions not as dorsal urethral duplications but as fistulas of the primitive urogenital sinus, with 3 anatomic types: high, toward the urachal remnant; middle, to the bladder; and low, reaching the urethra.

[Intestinal duplications. A survey of 18 cases]. / Duplicaciones intestinales. Análisis de 18 casos.

OBJECTIVE: The present study analyses the experience of two centers in the management of intestinal duplications in children. MATERIAL AND METHODS: We retrospectively reviewed the medical records of a series of 18 children with intestinal duplication (January 1993 to December 1999). The median age was 11 months (range: 9 days to 12 years). The variables analysed were: clinical presentation, diagnosis, localization, anatomic type, treatment, and complications. The relationship between age and clinical presentation was also analysed. RESULTS: Most cases were located in the ileum (n 14; 78 %) and presented with rectal bleeding (36 %) or intussusception (36 %). Most symptomatic cases in the first year of life presented with intussusception (n 5/6). All duplications, except one located in the ascending colon, were cystic. 99mTc-pertechnate abdominal scan was positive in three out of five patients. One case was diagnosed by prenatal ultrasonography. Diagnosis was confirmed by laparoscopy in three patients and by laparotomy in the remaining patients. In one patient, cystic esophageal duplication presented in the neonatal period as respiratory distress. Diagnosis as well as excision was performed by thoracoscopy. Of the two gastric duplications, one was diagnosed by prenatal ultrasonography and the other by laparotomy. Treatment consisted of segmental enterectomy (n 14) or excision of the lesion without enterectomy (n 4). No complications were found during a mean follow-up of 3 years. CONCLUSIONS: The onset of intestinal duplication may be late. The lesions were most commonly located in the ileum and were cystic. Clinical presentation was related to age. Minimally invasive surgery plays an important role in the management of these lesions.

Duplicaciones intestinales. Análisis de 18 casos / Intestinal duplications. A survey of 18 cases

Objetivo: El presente trabajo analiza la experiencia de dos centros hospitalarios en el manejo de las duplicaciones intestinales en niños. Material y métodos: Análisis retrospectivo de una serie (desde enero de 1993 a diciembre de 1999) de 18 niños afectados de duplicación intestinal con edad mediana 11 meses (límites, 9 días-12 años). Se evaluaron: forma de presentación, diagnóstico, localización, tipo anatómico, tratamiento y complicaciones. Se analiza la relación entre la edad y forma de presentación. Resultados: La localización más común fue la ideal (n = 14,78%) y las presentaciones clínicas más frecuentes fueron la invaginación intestinal (36%) y la rectorragia (36%). En los casos sintomáticos en el primer año de vida, la forma de presentación fue casi siempre la invaginación intestinal (5 de 6). Todos los casos excepto uno de localización en el colon ascendente, eran del tipo quístico. La gammagrafía con pertecnetato-99mTc fue positiva en 3 casos (3 de 5). Un caso fue reconocido por ecografía prenatal. El diagnóstico se confirmó por laparoscopia en 3 niños y por laparotomía en los restantes. Una duplicación esofágica se manifestó en el período neonatal por disnea. Su diagnóstico se estableció por toracoscopia, así como su exéresis. De las dos duplicaciones gástricas, una se diagnosticó por ecografía prenatal y otra por laparotomía. El tratamiento consistió en enterectomía del segmento intestinal afectado en la mayoría de los casos (n = 14) y en la exéresis de la lesión sin enterectomía en los restantes (n = 4). En un seguimiento medio de 3 años no se han registrado complicaciones. Conclusiones: Las duplicaciones intestinales pueden manifestarse de forma tardía. La localización más común fue la ileal y del tipo quístico. La forma de presentación está relacionada con la edad. La cirugía endoscópica tiene un papel importante en el manejo de las duplicaciones intestinales (AU)

[Intestinal duplication. A diagnostic and therapeutic challenge]. / Duplicações intestinais. Um desafio diagnóstico e terapêutico.

Duplications of the alimentary tract are rare congenital anomalies. The symptoms often mimic other surgical diseases and the diagnostic workup presents low accuracy. The charts of twelve children with intestinal duplications were reviewed. Their ages ranged from 13 days to 12 years (median: 12 months). The majority of the cases were located at ileum (n = 9). These children presented: rectal bleeding (n = 4), intussusception (n = 3), intestinal obstruction (n = 1) and hemoperitoneum (n = 1). All that were symptomatic before 1 year of age presented with intussusception (n = 3/3). All, except one colonic case was cystic. 99mTc abdominal scan was positive in 3 cases presenting rectal bleeding. One child with gastric duplication was diagnosed prenatally. Treatment consisted of enterectomy in all but one case, with no complications. Experience in the diagnostic workup and management is important to achieve a low morbidity rate in this congenital anomaly, which may present delayed manifestations.

[Bilateral cleft lip and palate: experience from a center with a multidisciplinary approach]. / Fisura labiopalatina bilateral: experiencia de un centro con abordaje multidisciplinar.

The aim of this work is to analyse the global experience of a center where a pluridisciplinar approach of the child affected with a cleft lip and palate is regularly done. Since january 1980 until january 2000, a total of 36 children (8 F, 28 M) with bilateral cleft lip and palate were treated. Only 5 children were born at this hospital. The others (n = 31) were referred soon after birth (24/31) or later for treat sequels. A multidisciplinary team evaluated every case. The parameters analysed were: surgical protocol, aesthetic and speech outcome, hearing disturbance, complications and the number of secondary lip surgeries. Surgical approach consisted on a soft palate closure before 3 months follow by a bilateral cheiloplasty (6 months) and a hard palate closure before 4 years of age, in the majority of cases (24/36). The esthetical result was evaluated in 25 children and was acceptable in the great majority (22/25). 16 children were submitted to tympanic draining in order to treat their secretory otitis. Speech outcome was analysed in 27 children and was good in 23. With a follow-up of 8.4 years, 15 children (8 treated soon on this center and 7 that came for their sequels) were treated for complications. There was a media of 4.5 surgeries per children.

Laparoscopic colonic mapping of dysganglionosis.

Retention of a proximal aganglionic segment or the unrecognized coexistence of other dysganglionoses may jeopardize the definitive surgical treatment of Hirschsprung's disease (HD). To assess the extent of the disease and/or the presence of other dysganglionoses without an additional laparotomy, we developed a laparoscopic-assisted technique to perform colonic full-thickness biopsies. After creation of a pneumoperitoneum, a 5-mm laparoscope is inserted in the supraumbilical area and a working 10/12-mm port is placed in the left iliac fossa. The sigmoid/descending colon is grasped and pulled through the abdominal wall and a full-thickness biopsy done. The same procedure is applied to the transverse and ascending colon. Rectal and colonic biopsy specimens were studied using enzyme histochemical methods. Over the last year, five children aged 7 months to 12 years with dysganglionosis underwent laparoscopic-assisted mapping of the colon. Previous rectal suction biopsies were diagnostic of HD in three patients and suspicious of hypoganglionosis in two. Proximal full-thickness biopsies revealed: normal colon in two cases of HD; coexistent type B intestinal neuronal dysplasia up to the descending colon in the other case of HD; and hypoganglionosis up to the ascending colon in the two patients with suspected hypoganglionosis. The procedures were performed easily, the patients being discharged after 36 to 48 h with no complications. Four children have already undergone pull-through procedures with resection of the affected colon in adhesion-free abdominal cavities and did not develop constipation or enterocolitis. Laparoscopic-assisted mapping of the entire colon is a simple, safe, and effective procedure that may contribute to improving the outcome of intestinal dysganglionosis by better characterization of the disease.

Fisura labiopalatina bilateral: experiencia de un centro con abordaje multidisciplinar / Bilateral cleft lip and palate: experience of a center with multidisciplinary approach

El objetivo de este trabajo es analizar la experiencia de un centro donde se hace un abordaje multidisciplinar del niño con fisura labiopalatina. Desde enero de 1980 hasta enero del 2000 se han intervenido 36 niños (8F, 28M) con fisura labiopalatina bilateral. Sólo 5 niños han nacido en el hospital y los restantes (n=31) fueron remitidos tras el nacimiento (n=24/31) o para tratar secuelas (n=7/31). Todos los casos fueron desde su ingreso valorados por un equipo pluridisciplinar. Los parámetros analizados han sido: protocolo quirúrgico utilizado, resultado estético, resultado de la voz y del habla, audición, complicaciones y número de cirugía efectuadas. El tratamiento consistió en una estafilorrafia precoz hacia los 3 meses, seguida de una queiloplastia bilateral (6 meses) y después una uranorrafia hacia los 4 años en la mayoría de los casos (24/36). El resultado estético fue evaluado en 25 niños y fue satisfactorio en la mayoría (22/25). Dieciseis niños fueron sometidos a drenaje timpánico para tratamiento de su otitis serosa. El resultado logopédico fue analizado en 27 casos y fue bueno en 23. Con un tiempo de seguimiento medio de 8,4 años, 15 niños (8 propios y los 7 que vinieron para tratamiento de secuelas) han sido tratados de complicaciones. La media de intervenciones por paciente fue de 4,5 (AU)

Invaginación intestinal secundaria a hamartoma mioepitelial en niños / Intestinal intussusception secondary to myoepithelial hamartoma in children

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Histochemical and immunohistochemical study of the intrinsic innervation in colonic dysganglionosis.

Defective innervation of the neuromuscular junctions (NMJ) was recently described in intestinal neuronal dysplasia type B (IND B). The aim of the present study was to correlate the alterations in NMJs to other classically described parameters in dysganglionoses and to determine the relationship between NMJ abnormalities in IND B and clinical symptoms. The rectal biopsies and full-thickness colonic biopsy specimens of 17 patients were studied applying histochemical (acetylcholinesterase [AChE], lactic dehydrogenase [LDH], and succinic dehydrogenase [SDH] reactions) and immunohistochemical (neuronal-cell adhesion molecule [NCAM] and SY antibodies) methods. Thirteen patients had Hirschsprung's disease (HD). IND B was diagnosed in 11 (associated with HD in 8 cases, isolated in 2, and associated with hypoganglionosis in 1). In the aganglionic segment of HD there was very intense AChE activity; in contrast, NCAM- and SY-immunoreactive nerve fibers were markedly decreased. A spectrum of abnormalities was observed in IND B, usually more severe in the most distal segments: giant and immature ganglia in the submucous plexus were observed in all cases; heterotopic myenteric ganglia were frequent (72.7%); hyperganglionosis was observed in 6 (54.5%) and was not related to the patients' age; thick and tortuous NCAM- and SY-immunoreactive nerve fibers, irregularly distributed in the colonic wall, were observed in 81.8% of the cases. No relationship was observed between abnormalities of NCAM- and SY-immunoreactive nerve fibers and AChE activity, ganglion-cell maturity, heterotopy, or the clinical symptoms presented by the patients with IND B. In hypoganglionism, low AChE activity and a slight decrease in NCAM- and SY-immunoreactive nerves were observed. Thick and tortuous, irregularly-distributed intrinsic NCAM- and SY-immunoreactive nerves were observed in every colon layer in IND B. Our results do not support IND B as a NMJ disorder.

Collar de Cobb. Causa rara de hematuria / Cobb's collar. A rare cause of haematuria

El collar de Cobb es un estrechamiento congénito de la uretra bulbar con presentación clínica y grado de obstrucción variables. Presentamos un caso clínico de un niño de 13 años de edad con hematuria macroscópica terminal y uretrorragia postmiccional. La cistoscopia identificó una membrana en la uretra bulbar, inmediatamente distal al esfínter externo, y vasos prominentes en la uretra prostática. La incisión endoscópica transuretral realizada fue curativa. Los autores hacen también una breve revisión de la embriopatogenia, clínica y tratamiento de esta patología rara (AU)

Acute gastric volvulus secondary to a Morgagni hernia.

Gastric volvulus (GV) is a rare surgical emergency in infancy and childhood. The first case of a child with an acute GV secondary to a Morgagni hernia (MH) is reported. The authors briefly discuss the etiology, clinical features, and management of acute GV and review the scattered reports of strangulated MHs in children.

[Cobb's collar. Unusual cause of hematuria]. / Collar de Cobb. Causa rara de hematuria.

The Cobb's collar is a congenital narrowing of the bulbar urethra with variable clinical presentation and obstruction grade. Herein we report a clinical case of a 13-year-old boy presenting terminal macroscopic haematuria and post-mictional urethral bleeding. At cystoscopy a membrane on the bulbar urethra distal to the external sphincter and prominent vessels in the prostatic urethra were found. Transurethral endoscopic incision was carried out with no complications or recurrence at 9-month follow-up. The authors briefly review the embryopathogenesis, clinical features and treatment of this uncommon anomaly.