Anal canal duplication and triplication: a rare entity with different presentations.

Anal canal duplication (ACD) is the rarest of gastrointestinal duplications. Few cases have been reported. Most cases present as an opening in the midline, posterior to the normal anus. The aim of our revision is to contribute with eight new cases, some of them with unusual presentations: five presented as the typical form, one with a perianal nodule, and two presented as two separate orifices (anal canal triplication). Complete excision was performed in all patients with no complications. ACD is the most distal and the least frequent digestive duplication. Its treatment should be surgical excision, to avoid complications such as abscess, fistulization, or malignization. Anal canal triplication has never been described before.

[Left cardiac sympathetic denervation in congenital ventricular arrythmias: initial experience]. / Denervación cardiaca simpática izquierda en las arritmias ventriculares congénitas: experiencia inicial.

BACKGROUND: Left cardiac sympathetic denervation (LCSD) has been proposed as a second-line therapy for potentially lethal ventricular arrythmia with no response to the gold standard treatment with beta-blockers. It has been used mainly in channelopathies such as long QT syndrome (LQTS) and polymorphic cathecolaminergic ventricular tachycardia (PCVT). AIM: Analizing our preliminary experience in the treatment of congenital ventricular arrythmia with thoracoscopic LCSD. MATERIAL AND METHODS: We have reviewed the first 5 LCSD performed in the last two years (2011-2013) to 5 female patients with a mean age of 8 years (1 month-15 years). The underlying disease was the LQTS in three and the PCVT in two. RESULTS: Selective contralateral bronchial intubation was used in two cases and double-lumen tube in three. We performed in all cases T1 sympathicolysis, denervation of T2 ganglion and sympathicolysis of T3 and T5 levels. No intraoperatory nor postoperatory complications were observed. In four cases ventricular tachycardia disappeared after the procedure and in one case, although they did not completely disappeared, they could be better controled on medical therapy. Ventricular extrasystoles reappeared 17 months after the procedure in one PCTV case, but disappeared completely after T1-T5 left sympathectomy completion. With a mean follow-up time of 20 months, all patients are sympthom-free and continue on betablocker oral therapy. CONCLUSIONS: Thoracoscopic DCSI is a safe and effective therapy for prevention of severe congenital ventricular arrythmias.

INTRODUCCION: La denervación cardiaca simpática izquierda (DCSI) se ha propuesto como tratamiento de segunda línea de las arritmias ventriculares con potencial riesgo de muerte que no responden al tratamiento farmacológico gold standard con beta bloqueantes. Fundamentalmente se ha utilizado en las canalopatías, que incluyen el síndrome de QT largo congénito (SQTL) y la taquicardia ventricular polimórfica catecolaminérgica (TVPC). OBJERIVO: Analizar nuestra experiencia inicial en el tratamiento de las arritmias ventriculares congénitas mediante DCSI toracoscópica. MATERIAL Y METODOS: Hemos revisado las primeras 5 DCSI realizadas en los últimos dos años (2011-2013) a 5 pacientes femeninas con una edad media de 8 años (1 mes-15 años). La patología de base fue el SQTL en tres casos y la TVPC en dos. Se utilizó una intubación selectiva contralateral en dos casos y una intubación con tubo de doble luz en tres. En todos los casos se realizó simpaticolisis T1, denervación del ganglio T2 y simpaticolisis de los niveles T3 y T5. RESULTADOS: No se registraron complicaciones intra ni postoperatorias. En 4 casos las taquicardias ventriculares desparecieron tras el procedimiento y en un caso, pese a no desaparecer completamente, se controlaron adecuadamente con tratamiento médico. Un caso de TVPC presentó nuevamente salvas de extrasístoles ventriculares (EV) 17 meses después de la simpaticolisis, realizando una simpatectomía T1-T5 con desaparición de las EV. Con un seguimiento medio de 20 meses, todas las pacientes permanecen asintomáticas y reciben tratamiento betabloqueante oral. CONCLUSIONES: La DCSI vía toracoscópica es un tratamiento efectivo y seguro para la prevención de las arritmias ventriculares congénitas graves.

[Management of rectal traumatism in the pediatric age]. / Manejo del traumatismo rectal en la edad pediátrica.

OBJECTIVE: In pediatrics anorectal injuries are not very common and their management classically includes an intestinal derivation. In selected patients and with favourable conditions, there has been an increased interest in repair the injuries without derivation. We present our experience in the management of this pathology, looking through the literature. The aim of the study is to develop some guidelines to follow in these cases. PATIENTS AND METHODS: Retrospective and descriptive study of patients with anorectal injury admitted in our institution between 2005-2011. Data abstraction included patient demographic data, mechanism of injury, associated injuries, time between injury and treatment, methods of diagnosis, treatment, length of stay and resultant complications. RESULTS: 7 patients were reviewed and classified according to the type of the injury in two groups: iatrogenic injuries (all of them with associated malformations) and non-iatrogenic accidental injuries. A CT was performed in 4 cases. None of them was explored with sigmoidoscopy. We performed colostomy without primary repair in four patients, primary injury repair with colostomy in two patients, and one patient underwent primary repair without colostomy. All patients had a long evolution (mean hospital length of stay was 31,4 days) with many dressings and some of them required relaparotomy. Fecal continence and long term results have been, in all of them, satisfactories. CONCLUSIONS: An early and complete diagnosis of anorectal injuries is basic for an appropriate approach to the treatment. Primary repair of injuries without colostomy could be a safe procedure as a first treatment in selected patients: stables, with no contamination and no associated injuries. It is very important to individualize each patient to minimize the morbidity, reduce the hospital length of stay and reach a full continence.

[Utility of the ECMO in patients with congenital diaphragmatic hernia]. / Utilidad de la ECMO en pacientes con hernia diafragmática congénita.

At the moment the extracorporeal membrane oxygenation (ECMO) constitutes the last link in the therapeutic one of the handling of the respiratory failure in patients with Congenital Diaphragmatic Hernia (HDC). We presented our experience. From January 2001 we arrange the ECMO in neonative UCI. 76 HDC, 13 (3 rights and 10 lefts) they have needed ECMO (one in two occasions; altogether 14 procedures). Criteria of inclusion: refractory hypoxaemia, oxigenaction index > 40 and weight > 2 kg. 5 girls and 8 boys with gestacional age between 35 and 41 weeks (average: 38) and weight when being born between 2,300 and 3,500 grams (average 2,817). In 6 cases (5 transferred from other centers) the diagnosis was posnatal. Of the 7 with prenatal diagnosis, in 4 cases fetal therapy by means of traqueal occlusion had been made. Veno-venous in 8 and veno-arterial procedure in 5. Rank of duration: 68-606 hours, average of 228.35. The surgery has been made before the ECMO in 9 cases, 2 during and 1 later. In an occasion there was no surgery. The complications have been of hemorrágico type in one patient and infectious in three cases with sudden sepsis in one. Precocious mortality has been of 6 patients and delayed the 2 (total 61%). Although this procedure has the high morbi-mortality, it is necessary to consider that is patients very badly prognosis without another alternative (with mortality of the 100%). Multicentric studies are needed to establish indicators prognoses pre and postbirthdays.

Impact of fetal intervention on postnatal management of congenital diaphragmatic hernia.

UNLABELLED: We report our experience in the postnatal management of congenital diaphragmatic hernia (CDH) after fetal endoluminal balloon tracheal occlusion (FETO). MATERIAL AND METHODS: Out of 19 CDH fetuses diagnosed since December 2005, 13 had a lung-to-head ratio (LHR) < 1.1 (0.86 +/- 0.11) and underwent FETO at a mean gestational age (GA) of 27.9 weeks. The balloon was removed in 3 cases through puncture in EXIT (ex utero intrapartum treatment); there were 3 trans-utero punctures, 3 fetoscopies and 3 balloons deflated spontaneously. One patient died from bleeding during the FETO procedure. Six patients were not subjected to FETO: 3 had a LHR > or = 1.4 and 3 did not come to consultation prenatally. RESULTS: Twelve babies were born after FETO at 34.9 +/- 1.7 weeks GA; 3 died before surgery from refractory hypoxia. Six needed high frequency oscillatory ventilation (HFOV), and inhaled nitric oxide (NO), one progressing to extracorporeal membrane oxygenation (ECMO), and 3 needed conventional ventilation. CDH was repaired in 9: 2 with direct sutures and 7 with prosthetic patches. Extubation was at median of 5 days (range: 2 - 40), discharge was at 30.4 +/- 16.4 days. Early complications were 1 reherniation and 2 intestinal occlusions. Two patients died after CDH repair: the ECMO case at 15 days, and an infant discharged on oxygen at home, at age 4 months. Of the non-FETO patients, four needed HFOV and NO. CDH repair was performed in 5: 3 had direct repair and 2 had prosthetic patches. Extubation was at 13.4 +/- 12.9 days and discharge was at 43.8 +/- 26.5 days. One patient died before surgery from severe cardiac malformation. Early complications were 1 reherniation and 1 hiatus hernia. Late complication was 1 reherniation at 5 months of life. There was no postoperative mortality. One patient needs oxygen at home. CONCLUSION: CDH patients with a poor prognosis undergoing FETO had postnatal outcomes similar to non-prenatally studied cases and good prognosis cases.

[Which surgical technique should we perform for benign renal disease in children?]. / Qué técnica quirúrgica hemos de utilizar en la nefrectomía por patología benigna?.

AIM: The aim of this paper is to analyze our experience in different surgical techniques to perform a nephrectomy for benign renal diseases in children. MATERIAL AND METHODS: From 1993 to 2005 we have performed 98 nephrectomies. We have three groups of patients depending on the surgical technique: open nephrectomy (ON), transperitoneal laparoscopic nephrectomy (TLN) and retroperitoneal laparoscopic nephrectomy (RLN). ON was performed in 36 patients. Mean age was 3.3 years. TLN was performed in 39 patients. Mean age was 4.7 years old. RLN was performed in 23 patients. Mean age was 3.6 years old. Criteria to nephrectomy was a renographic function under 19%. We have compared the three surgical techniques in relation with surgical time and mean hospital stay. RESULTS: Mean operative time was 126.2 minutes in ON, 132.3 minutes in TLN and 134.1 minutes in RLN. Mean stay was 5.02 days in ON, 2.35 days in TLN and 1.86 days in RLN. The median hospital stay of the ON group is significantly longer than that of NLT and NR groups (p < 0.05). However, there are no differences related to surgical time between all the groups. CONCLUSIONS: Nephrectomy may be performed for benign disease in children using less invasive surgical techniques. They are associated with minimal morbidity, minimal postoperative discomfort, improve cosmesis and a shorter hospital stay. However, we haven't found differences between TLN and RLN.

[Lessons we've learned in the treatment of long gap esophageal atresias]. / Lecciones aprendidas en el tratamiento de las atresias de esófago con gran separación entre sus cabos.

BACKGROUND: A gap greater than 3 cm between both esophageal pouches is observed in 1 of 20 cases of esophageal atresia. Our goal was to critically review our experience in the management of these patients. MATERIAL AND METHODS: During 1995-2004, 15 patients were treated for a long gap esophageal atresia (LEA). Gaps ranged from 3 to 8 cm. Ten patients presented a pure esophageal atresia. They were managed with a gastrostomy and delayed repair: 8 Schärli interventions and 2 esophageal flaps. The other 5 patients had an esophageal atresia with distal fistula (LEA-DF), and primary repair was attempted: 3 end-to-end esophageal anastomosis were achieved under a strong tension; 1 a Schärli procedure; 1 ligation of the fistula, feeding gastrostomy and delayed esophageal flap. The use of esophageal flaps is a late event in our series. since its introduction we've performed 5 esophageal atresia repairs using 3 flaps (2 pure atresias and 1 LEA-DF). RESULTS: From 9 Schärli we have to practice 2 reinterventions for anastomotic leak; there was 1 parahiatal hernia that needed surgery after 8 years. From 3 flaps 2 patients presented a persistent stricture that needed reintervention. All of the 3 E-E anastomosis had reintervention for persistent stricture and also anti-reflux procedures (Nissen). Eight patients showed a normal growth and development (4/9 Schärli, 3/3 flaps and 1/3 E-E). Seven patients are growth retarded (4/7 with associated malformations, 1/7 who developed an eosinophilic esophagitis and 2/7 preterm babies). CONCLUSIONS: The esophageal flap is our first choice, because the own esophagic tissue fills in the gap. In LEA-DF, we prefer fistula ligation, gastrostomy and delayed rise of a flap (as we did in our last patient) better than a very tense primary anastomosis. As a second option, a Schärli procedure offers satisfying mid-term results. Keeping the patient paralyzed and mechanically ventilated for 5-7 days after surgery helps to avoid disrupting forces on the anastomosis.

[Nutritional and surgical management of short bowel syndrome. Our last 6 patients' experience]. / Manejo nutricional y quirúirgico del intestino corto como causa de fracaso intestinal en niños. A propósito de seis pacientes.

BACKGROUND: Children cause of intestinal failure is short-bowel syndrome. It provokes an altered absorption of nutrients and makes patients to be dependent on parenteral nutrition (PN) while they wait or not for an intestinal transplantation, with its side effects. It is crucial to achieve the maximum efficiency of remaining intestine. Many surgical techniques have been led to reduce stasis of dilated small intestine and improve the mucosal surface area for absorption. METHODS: Six patients have presented intestinal failure because of a surgical resection during newborn period. 2 gastroschisis, 2 intestinal atresias (Apple-peel), 1 necrotizing enterocolitis (NEC) and 1 midgut volvulus. 4 preserve ileocecal valve (ICV): 2 Apple-peel, 1 NEC and the midgut volvulus. The shortest length of bowel after resection were 12cm without ICV (gastroschisis) and 18cm with ICV in a preterm newborn of 24 weeks of gestational age (midgut volvulus). Tapering and plication have been done in Apple-peel cases. No complementary surgical techniques have been necessary in NEC and volvulus. Gastroschisis cases had 12 and 40 cm of small bowel without ICV. In the first one, during newborn period an intestinal lengthening according to Bianchi was done, followed by sequential transversal enteroplasty (STEP), partial gastrectomy and plication of 1st and 2nd duodenal portion. In the second, an STEP was done. All patients have received cycled and optimized PN (COPN) in our centre, only one case (gastroschisis) proceeding from another centre had received standard PN and developed a great hepatic affectation. RESULTS: 2 Apple-peel have been adapted to normal enteral nutrition after 3 and 18 months of COPN, such as NEC and volvulus (3-6 months). One gastroschisis (12cm) has a normal hepatic function with free oral nutrition and home COPN at 23 months. The other one (40cm) has COPN and started enteral nutrition 1 month after surgery, although its hepatic function remains altered. CONCLUSIONS: Parenteral nutrition is essential for these patients to survive. We would like to enhance the importance of COPN in order to preserve hepatic function. Surgical procedures aim to avoid stasis and bacterial overgrowth and improve intestinal motility. Different techniques may be used alone or sequentially. The purpose of this management is to achieve nutritional autonomy or increase waiting time before intestinal transplantation.