Minimally invasive repair of Morgagni hernia - A multicenter case series.

Children may benefit from minimally invasive surgery (MIS) in the correction of Morgagni hernia (MH). The present study aims to evaluate the outcome of MIS through a multicenter study. National institutions that use MIS in the treatment of MH were included. Demographic, clinical and operative data were analyzed. Thirteen patients with MH (6 males) were operated using similar MIS technique (percutaneous stitches) at a mean age of 22.2±18.3 months. Six patients had chromosomopathies (46%), five with Down syndrome (39%). Respiratory complaints were the most common presentation (54%). Surgery lasted 95±23min. In none of the patients was the hernia sac removed; prosthesis was never used. In the immediate post-operative period, 4 patients (36%) were admitted to intensive care unit (all with Down syndrome); all patients started enteral feeds within the first 24h. With a mean follow-up of 56±16.6 months, there were two recurrences (18%) at the same institution, one of which was repaired with an absorbable suture; both with Down syndrome. The application of MIS in the MH repair is effective even in the presence of comorbidities such as Down syndrome; the latter influences the immediate postoperative recovery and possibly the recurrence rate. Removal of hernia sac does not seem necessary. Non-absorbable sutures may be more appropriate.

Thoracoscopy in the management of pediatric empyemas.

INTRODUCTION: Thoracoscopy is increasingly being used in the treatment of empyema. This study assesses feasibility, efficacy and safety in children. MATERIAL AND METHODS: Clinical files of patients who underwent primary thoracoscopy for empyema between 2006 and 2014 were reviewed. Demographic, clinical and surgical data were analyzed and a comparison between the period before (period1) and after (period2) the learning curve was performed. RESULTS: Ninety-one patients (53 males, 58%) were submitted to thoracoscopy at a median age of 4 years. There were 19 conversions to thoracotomy with a steady decrease of conversion rate until 2009 (period1) and no conversions thereafter (period2). There was no difference in any of the analyzed parameters between patients submitted to thoracoscopy alone and those requiring conversion in period1. Six cases (6.6%) needed redo-operation (five in period2) and thoracotomy was the elected approach in four. Necrotizing pneumonia was present in 60% of the reoperated cases; in other words, in period2 3 out of 9 cases with necrotizing pneumonia required reintervention (p=0.07). Thoracotomy was avoided in sixty-eight (75%) patients (62% in period1 versus 92% in period2, p=0.001). DISCUSSION AND CONCLUSIONS: Thoracoscopic approach for empyema is feasible and safe avoiding a significant number of thoracotomies after a short learning curve. An increase of reintervention rate should be expected, but throracoscopy alone is effective in the great majority of the cases. Necrotizing pneumonia may be associated with a higher risk of reintervention, as it is a contra-indication to thoracoscopy and probably surgery.

Miotomía de Heller laparoscópica: resultados en niños / Outcome of laparoscopic heller myotomy in children

Objetivos. El presente estudio tuvo como objetivo evaluar los resultados a corto y medio plazo de la miotomía de Heller laparoscópica. Métodos. Fueron incluidos niños sometidos a cirugía desde 2002 hasta 2010. La cirugía fue propuesta tras el fracaso de las dilataciones endoscópicas. Todos los pacientes fueron sometidos a esofagocardiomiotomía (Heller) con funduplicatura anterior (Dor) por laparoscopia. La demografía, síntomas de presentación y detalles perioperatorios fueron analizados. Los resultados fueron evaluados por los registros médicos y la aplicación de un cuestionario de auto-evaluación (GIQLI modificado), cubriendo síntomas gastrointestinales superiores) con 14 preguntas cada una puntuada de 0 (peor) hasta 4 (mejor o normal).Resultados. Seis pacientes (5 varones) con una edad media de 12,2 años (rango: 0,8-14,2) fueron sometidos a cirugía. Los síntomas de presentación fueron: disfagia (83,3%), pérdida de peso (50%), vómitos (33,3%), tos (33,3%) y dolor torácico (16,7%). Todos los pacientes fueron operados por laparoscopia sin conversiones y no hubo complicaciones intra, ni postoperatorias. Con una mediana de seguimiento de 5 años (rango: 2-10), ninguno de los pacientes fue re-operado. Cinco pacientes tienen hábitos alimentarios normales; el caso restante presentó episodios recurrentes de disfagia, requiriendo dilataciones endoscópicas periódicas (cada 6 meses). El GIQLI total presentó una media de 49,3 puntos (rango, 45-52), lo que representa 88,1% del máximo posible. La puntuación para la frecuencia de episodios de disfagia fue 1,6 ± 1,4; la puntuación para el grado de disfagia fue 3 en todos los pacientes. Conclusiones. La miotomía de Heller laparoscópica es efectiva y segura en niños, ofreciendo una calidad de vida buena y duradera; a pesar de frecuentes, los episodios de disfagia son ligeros (AU)

Aim. The appropriate management of achalasia in children remains debatable. The present study aimed to evaluate the outcome of laparoscopic Heller myotomy by assessing short- and mid-term issues. Methods. Children submitted to surgery from 2002 to 2010 were included. Surgery was proposed after failure of endoscopic dilatations. All patients underwent esophagocardiomyotomy (Heller) plus anterior fundoplication (Dor) by laparoscopy. Demographics, presentation symptoms and perioperative details were analyzed. The outcomes were assessed both by medical records and the application of a 14 items (score 0-worst to 4-best/normal) self-evaluation questionnaire (modified GIQLI - covering only upper gastrointestinal symptoms).Results. Six patients (5 males) with a median age of 12.2 years (range: 0.8-14.2) were submitted to surgery. The presenting symptoms were: dysphagia (83.3%), weight loss (50%), vomiting (33.3%), chronic cough (33.3%), and chest pain (16.7%). All patients were operated on by laparoscopy with no conversions; there were no intra or postoperative complications. At a median follow-up of 5 years (range: 2-10) none of the patients were re-operated. Five patients have normal eating habits; the remaining case presented recurrent episodes of dysphagia requiring regular endoscopic dilatations (every 6 months). The total GIQLI presented a mean score of 49.3 (range, 45-52) representing 88.1% of the theoretical maximum. The score for frequency of dysphagia episodes was 1.6 ± 1.4; all patients scored 3 for the grade of dysphagia. Conclusions. Laparoscopic Heller myotomy is effective and safe for achalasia in children, offering a good and durable quality of life; although frequent, the dysphagia episodes were mild (AU)

[Outcome of laparoscopic Heller myotomy in children]. / Miotomía de Heller laparoscópica: resultados en niños.

AIM: The appropriate management of achalasia in children remains debatable. The present study aimed to evaluate the outcome of laparoscopic Heller myotomy by assessing short- and mid-term issues. METHODS: Children submitted to surgery from 2002 to 2010 were included. Surgery was proposed after failure of endoscopic dilatations. All patients underwent esophagocardiomyotomy (Heller) plus anterior fundoplication (Dor) by laparoscopy. Demographics, presentation symptoms and perioperative details were analyzed. The outcomes were assessed both by medical records and the application of a 14 items (score 0-worst to 4-best/normal) self-evaluation questionnaire (modified GIQLI - covering only upper gastrointestinal symptoms). RESULTS: Six patients (5 males) with a median age of 12.2 years (range: 0.8-14.2) were submitted to surgery. The presenting symptoms were: dysphagia (83.3%), weight loss (50%), vomiting (33.3%), chronic cough (33.3%), and chest pain (16.7%). All patients were operated on by laparoscopy with no conversions; there were no intra or postoperative complications. At a median follow-up of 5 years (range: 2-10) none of the patients were re-operated. Five patients have normal eating habits; the remaining case presented recurrent episodes of dysphagia requiring regular endoscopic dilatations (every 6 months). The total GIQLI presented a mean score of 49.3 (range, 45-52) representing 88.1% of the theoretical maximum. The score for frequency of dysphagia episodes was 1.6 +/- 1.4; all patients scored 3 for the grade of dysphagia. CONCLUSIONS: Laparoscopic Heller myotomy is effective and safe for achalasia in children, offering a good and durable quality of life; although frequent, the dysphagia episodes were mild.

Escisión laparoscópica de quiste mesotelial hepático / Laparoscopic excision of a hepatic mesothelial cyst

Los quistes mesoteliales son lesiones congénitas extremadamente raras que tienen origen en restos celómicos. Los autores describen el caso de un niño de tres años de edad. Presenta como hallazgo casual imagen quística hepática en ecografía abdominal, en el contexto de estudio de dolor abdominal. El examen objetivo y el estudio analítico no demostraron ninguna alteración. La RMN confirmó la presencia de lesión quística, sugiriendo como hipótesis diagnóstica tratarse de un cistadenoma quístico. El enfermo fue sometido a quistectomía por laparoscopia, con alta al 2º día. El estudio de anatomía patológica concluye que se trata de un quiste mesotelial hepático. Los quistes mesoteliales hepáticos son lesiones muy raras, de difícil diagnóstico preoperatorio. Su escisión laparoscópica es un procedimiento exequible y seguro (AU)

Over the past few years there has been an increase in application of minimally invasive techniques in pediatric surgery, especially in the approach of liver lesions. The mesothelial cysts are extremely rare congenital lesions that arise from celomic remnants. The authors present the clinical case of a three year old child, referred to our institution after an incidental finding of a liver cystic lesion in abdominal ultrasound. The physical examination and blood study were irrelevant. The MRI confirmed the presence of the cystic lesion and pointed, as a possible etiology, a cystic cyst adenoma of the liver. The child was submitted to laparoscopic cyst resection. There peri and postoperative periods were uneventful. He was discharged 48h after surgery. The anatomo-pathological and immunohistochemical studies concluded it was a mesotelial cyst of the liver. The mesothelial cysts of the liver are very rare lesions, with difficult preoperative diagnosis. The laparoscopic excision is a feasible and safe (AU)

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.

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.

Antireflux role of free muscle transplantation. Experimental study in a reflux esophagitis rat model.

PURPOSE: Experimental work on free muscle transplantation has resulted in some successful clinical applications. After a previous experimental study that established the viability of this procedure on the distal esophagus of the rat, we investigated the antireflux efficacy of free muscle transplantation on the distal esophagus in a reflux esophagitis rat model. METHODS: Two groups of Sprague-Dawley rats were submitted to a reflux-inducing operation. Thirteen animals underwent esophagojejunostomy only (EJ) and fourteen were submitted to esophagojejunostomy plus free muscle transplantation of plantaris on the distal esophagus (ETx). Eight weeks later, the esophagi of survivors were removed after sacrifice (EJ, n = 10; ETx, n = 10) and the macroscopic longitudinal extension (mm) and the histological grade (1-4) of esophagitis evaluated. RESULTS: All esophageal specimens of both groups had esophagitis. However, the extent of esophagitis (median: ETx: 23 vs EJ: 32) and the frequency of high grades of esophagitis (ETx: 30 % vs. EJ: 100 %) were significantly lower in the ETx group (p = 0.005 and p = 0.001, respectively). All except one ETx subject presented with an extent and/or a grade of esophagitis which was less than the EJ subjects' lowest values. CONCLUSIONS: Experimental free muscle transplantation on the distal esophagus seems to have antireflux function, thus its therapeutic implications deserve further investigation. The incomplete efficacy of the procedure may suggest that a putative anatomic sphincter per se is not enough to promote an effective antireflux mechanism.

Neonatal perforation of a Y-shaped sigmoid duplication.

Tubular colonic duplications are exceedingly rare; the "Y"-shaped forms are exceptional. In the absence of associated low vertebral or urogenital malformations (often fistulas), the tubular colonic malformations frequently stay hidden for several years until a complication develops. The authors report a case of a spontaneous perforation of a Y-shaped tubular colonic duplication during the neonatal period.

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.

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

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Delayed gastric emptying and gastroesophageal reflux: a pathophysiologic relationship.

BACKGROUND: Delayed gastric emptying (DGE) is frequent in patients with gastroesophageal reflux (GER), but its pathophysiologic role has not yet been established. To identify a relationship between DGE and GER, we assessed whether DGE increases esophageal acid exposure and the related importance of possible mechanisms. METHODS: Thirty pediatric patients with pathological GER were divided according to gastric emptying scintigraphy into a DGE group (n = 14) and normal-emptying group (n = 16). The esophageal pH-monitoring parameters of the two groups were compared with respect to the individual variation between postprandial and fasting periods. RESULTS: Patients with DGE had less total acid exposure than did those with normal emptying, but patients in both groups had a pathological fraction of time when pH was below 4 in both the postprandial (median: 18 vs. 27.6; P = 0.49) and fasting (8.5 vs. 23.9; P = 0.01) periods. Patients in the normal-emptying group had similar fraction of time when pH was below 4 in the postprandial and fasting periods. However, patients in the group with DGE had a fraction of time when pH was below 4 in the postprandial period that was almost double that presented in fasting period (postprandial to fasting ratio: 2.11:0.90; P = 0.002). The postprandial to fasting ratio for episodes per hour was similar in the two groups (1.81 vs. 1.79; P = 0.62). Patients with DGE had a significantly higher frequency of long episodes in the postprandial period than did those with normal emptying (62.5% vs. 38.2%; P = 0.04). The occurrence of the longest episode in the postprandial period was also significantly higher for patients with DGE (57.1% vs. 6.2%; P = 0.003). CONCLUSIONS: DGE seems to accentuate postprandial reflux by increasing the volume of refluxate per episode of reflux through an underlying incompetent lower esophageal sphincter.

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.

A new fetal rat model of gastroschisis: development and early characterization.

BACKGROUND/PURPOSE: The perinatal management and pathophysiology of gastroschisis remain controversial. Large animal experimental models of gastroschisis are inherently limited by expense and length of gestation, making multiple studies and statistical analysis difficult. To address these limitations the authors have developed a model of gastroschisis in the fetal rat. METHODS: Twenty-one time-dated pregnant rats underwent laparotomy at 18 (1/2) day's gestational age. The exposed uterus was bathed in ritodrine for tocolysis. The right posterior leg was exteriorized through a hysterotomy, and under a dissecting microscope (16x) the fetal small bowel was exteriorized through a small incision performed on the right lower abdominal quadrant. The amniotic fluid was restored with saline solution and the hysterotomy closed with a purse-string suture. Control fetuses underwent hysterotomy and leg manipulation only. The surgical time was uniformly less than 60 minutes. Fetuses were harvested by cesarean section at 21 (1/2) days' gestational age. Fetal intestine was assessed by microscopic examination, and fetal weight, intestinal length, and intestinal weight per unit length were evaluated. RESULTS: There was a significant surgical and anesthetic learning curve, which is not included in this report. After this, the authors achieved a maternal survival of 100% (n = 21). We created gastroschisis in 64 fetuses (58 survivors, 90.6%), and 33 fetuses were only manipulated (30 survivors, 90.9%). The number of induced gastroschisis per pregnant rat varied between 2 and 5 with median of 3. On gross examination, eviscerated intestine appeared dilated, edematous, and covered by peel when compared with control intestine. Fetuses with gastroschisis had significantly reduced body weight (4.1+/-0.5 v 5.6 g +/- 0.5 g) and intestinal length (102+/-19 v 210+/-17 mm) relative to controls, whereas the intestinal weight per unit length (1.75+/-0.29 v 0.71 +/- 0.1 mg/mm) was markedly increased (P<.001). CONCLUSIONS: The pathophysiology observed in this experimental model appears to resemble human gastroschisis. In comparison with large animal models, the rat model offers the advantages of low expense, short gestation, littermate controls, and high maternal and fetal survival rates. In addition, there are specific probes and reagents available for application of molecular methodology to clarify the mechanisms responsible for the intestinal damage. This model appears appropriate for future experimental studies on gastroschisis.

[Intestinal invagination in children. Reduction with pneumo-enema]. / Invaginação intestinal na criança. Redução com pneumo-enema.

UNLABELLED: Pneumatic reduction of intussusception in children is an effective and safe procedure, although controversy persists concerning contraindications. The shown by this procedure when compared to barium reduction have led to its implementation in our Service. AIM: To evaluate the usefulness of a 'handicraft method' of pneumatic reduction of intussusception with very restricted contraindications (peritonitis or shock). MATERIAL AND METHODS: The prospective study included 50 attempts at pneumatic reduction in 48 children (age range: 1.5-24 months). After confirmation of the diagnosis by ultrasonography. Then, air insufflation of the colon was performed under manometric and fluoroscopic control. After confirmation of diagnosis by ultrasonography, air insufflation of the colon was performed under manometric and fluoroscopic control in an operative room. Evolution longer than 24 hours was considered diagnostic delay, leukocytosis if WBC > 15 x 10(9)/l and distal localisation after splenic angle. Immediate laparotomy was undertaken in case of unsuccessful or doubtful reduction. RESULTS: Thirty-five reduction procedures (70%) were effective ab initio. In the 15 children submitted to laparotomy, seven were completely reduced (14%), five were manually reduced (10%), two presented intestinal necrosis (4%) and one (recurrence) had an ileal duplication (2%). The "real" efficacy (84%) was lower, even though significantly affected by diagnostic delay (81% vs 83%), rectal bleeding (81% vs 100%) or leukocytosis (71% vs 81%); efficacy was significantly lower only in distal localised cases (67% vs 97%, p = 0.02). There was one more recurrence (4%) and no other complications. CONCLUSIONS: The adopted method of pneumatic reduction was highly effective with low morbidity. Restricted contraindications were appropriate.

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)