Complete Currarino triad in all affected members of the same family.

Currarino triad is a rare hereditary disease. The complete form is characterized by the presence of an anorectal malformation, usually anorectal stenosis, a presacral mass and sacral bony defect. The main symptom is the presence of constipation since early life. In over 80 % of cases, the syndrome is diagnosed during the first decade of life. We report on three patients, members of the same family, with the complete form of the syndrome. The main symptom in our patients was intense constipation and the common clinical finding was an ectopic and stenotic anus. In two of them, father and son, the presacral masses were not diagnosed at the time of previous unsuccessful operations in another hospital for correction of the ectopic anus. In the third patient, Currarino syndrome was associated with Hirschsprung's disease. This has not been described previously. Two of the three patients who were admitted in our institution for simultaneous excision of the presacral mass and correction of the anorectal malformation through a posterior midsagittal coccygo-perineal approach, are free of symptoms. To the authors' knowledge, this is the second report of complete Currarino triad in all affected members of the same family.

Rectosigmoid ectasia associated with anorectal anomaly repair: experience with sigmoid anterior resection and endorectal pull-through.

PURPOSE: Surgical treatment of anorectal anomalies may be followed by severe constipation with or without overflow incontinence secondary to dilation and ectasia of the rectosigmoid. The aim of this study is to evaluate the efficacy of anterior resection and endorectal pull-through for the treatment of rectosigmoid ectasia secondary to posterior sagittal anorectoplasty (PSARP). MATERIAL AND METHOD: Seven patients with a history of PSARP presented with serious rectosigmoid ectasia and overflow incontinence of fecal impaction. Four were boys and 3 were girls aged from 16 months to 15 years. Preoperative diagnosis was based on physical examination, barium enema, MRI and rectal biopsy. Surgical treatment of the post-PSARP rectal ectasia was made by anterior resection of the ectatic segment of the sigmoid combined with pull-through of normal colon into the mucosectomized ectatic rectum. RESULTS: During a four-to ten-year follow-up period all of our patients had normal bowel movements. Postoperative barium enema radiography demonstrated a normal rectal caliber and manometry revealed normal anorectal motor function. CONCLUSIONS: The good postoperative results draw attention to the following. a) The aim of the surgical treatment of megarectum is to establish a neorectum with normal bowel. b) Resection of megasigmoid and pull-through of bowel with normal caliber into the mucosectomized ectatic rectum is an effective procedure in patients with post-PSARP rectosigmoid ectasia. c) The neorectum attains its reservoir function through a gradual process after resection and endorectal pull-through.

Retrospective evaluation of long-term efficacy and safety of splenectomy in chronic idiopathic thrombocytopenic purpura in children.

AIM: To review the long-term efficacy and safety of splenectomy in children with chronic idiopathic thrombocytopenic purpura (cITP). PATIENTS AND METHODS: Data from 33 splenectomized children were retrospectively analysed (median follow up period: 18.8 y from the removal of the spleen). The median age of children at splenectomy was 12 y and the median ITP duration 3.3 y. Indications for splenectomy were: persistent severe thrombocytopenia with extensive purpura, epistaxis and/or gum bleeds, menorrhagia (n = 5) and severe or recurrent haemorrhage from various sites (n = 11). RESULTS: Eighty-five per cent of the patients showed an excellent (n = 26) or partial response to splenectomy. Five children (15%), all females, failed to respond. Of the responders, 25% experienced a transient recurrence of thrombocytopenia within 6 mo to 4 y from splenectomy. The mortality rate due to severe sepsis was 3%. However, the majority of the splenectomized patients have not so far suffered any severe or mild bacterial infection, despite incomplete vaccination and/or antibiotic prophylaxis. CONCLUSION: Splenectomy remains the only effective therapeutic modality for children with cITP, although it is associated with transient recurrence and rarely with post-splenectomy sepsis, which could be fatal. Nonetheless, splenectomy should be the last treatment option for the cITP patient, after all available therapeutic modalities have been exhausted and the child still remains profoundly thrombocytopenic and symptomatic.

Medical treatment of pulmonary hydatidosis: complications and surgical management.

Pulmonary hydatidosis is more frequently encountered in children than in adults. Chemotherapy with oral administration of particular antihelminthic agents (mebendazole and albendazole) has proved to be effective. This treatment, however, may be associated with serious complications that require surgical management. The aim of this study was to define the limitations of medical treatment, the subsequent complications, and their management. During a 16-year period (1985-2001), 36 children with pulmonary hydatidosis (Echinococcus cysticus) were medically treated. Oral antihelminthic agents (mebendazole until 1992 and albendazole thereafter) were given to all these patients. During this treatment, 11 patients developed complications requiring surgical intervention. In seven children, pleural empyema, or the presence of inflammatory residual fluid, was noted. The remaining four developed pulmonary abscess combined with fluid collection within the pleural cavity. In all complicated cases, the mean size of the cysts exceeded 6 cm in diameter at the beginning of medical treatment. At operation, suturing of communicating bronchi was insecure due to inflammation. Postoperatively, three patients had air leakage from the bronchial tree, requiring continuous suction and prolonged hospitalization. One patient presented with pneumothorax 4 months postoperatively and was operated on again. Overall, long-term results were good. We concluded that a) large pulmonary hydatid cysts should not be treated medically, b) incomplete expectoration of the cyst contents after the parasite death may lead to infection through bronchial communication, and c) patients surgically treated for complications following medical treatment are hospitalized twice as long as patients surgically treated in the first place.

[Transanal endorectal pull-through with or without laparoscopic assistance? Development of an experimental model]. / Abbassamento endorettale transanale con o senza assistenza laparoscopica? Sviluppo di un modello sperimentale.

The transanal approach is a relatively new technique for surgery of Hirschsprung's disease. The purpose of this experimental study was to evaluate and compare the transanal endorectal pull-through when it takes place with laparoscopic assistance, according to Soave-Georgeson procedure and without laparoscopic assistance. We used two separated groups of animals. In the 1st group the transanal endorectal pull-through was performed by retrograde transanal mucosectomy with laparoscopic devascularization of the colon and in the 2nd group the transanal endorectal pull-through was performed without laparoscopic assistance. In the 1st group the transitional zone of the colon was determined laparoscopically while in the 2nd group during the perineal devascularization of the bowel. In both groups end to end coloanal anastomosis was performed in the same way. The postoperative course was uneventful, no severe long-term problems were observed. Four months after operation the experimental subjects of the 1st group had 3-4 discharges in 24 hours. While the experimental subjects of the 2nd group had 5-7 discharges in 24 hours. Manometric resting anal sphincters pressure in the 1st group averaged 77 +/- 16 mmHg compared with 74 +/- 15 mmHg in the 2nd group. The transanal colectomy technically can be performed with or without laparoscopic assistance. However the advantages of laparoscopic assistance are the following: 1) more exact determination of transitional zone; 2) easier separation of peritoneal reflection, 3) better control of final position of the colon; 4) minor retraction on the perineal muscles.

Annular pancreas combined with distal stenosis. A report of four cases and review of the literature.

BACKGROUND: Congenital duodenal obstruction (CDO) in combination with more distal duodenal obstructions is a rare anomaly occurring in 4% of neonates with duodenal atresia. The experience of two European Pediatric Centers in treatment of congenital double duodenal obstruction (CDDO) is reported and the pertinent literature is reviewed. MATERIALS AND METHODS: During the last 15 years a total of 86 neonates were operated upon for CDO at the department of pediatric surgery of "St. Sophia" Children's Hospital of Athens in Greece and the department of pediatric surgery of "Federico II" Children's University Hospital of Naples in Italy; four of them had a CDDO. These ones presented with nonbilious vomiting and the plain film of the abdomen showed the typical "double bubble". Our cases with CDDO presented annular pancreas causing complete obstruction of the second part of the duodenum and dilatation of the duodenum distal to this obstruction due to an additional congenital stenosis (two cases) or a membranous web (two cases). A diamond shaped duodeno-duodenal (DDD) anastomosis was carried out to relieve the proximal obstruction and a Heinecke-Mikulicz plasty was used to relieve the distal stenosis. RESULTS: All patients with double obstruction underwent successful surgery with no complications. The postoperative course was uneventfuL An upper gastrointestinal barium study at one month postoperatively showed no blind loop, megaduodenum, anastomotic stenosis or malfunction. CONCLUSIONS: a) The combination of duodenal atresia with annular pancreas and distal duodenal stenosis or web is extremely rare. b) Patency of the duodenum distal to the usual obstruction should always be checked in order to avoid misdiagnosis of this combination.

The posterior midsagittal approach for the surgical excision of intrapelvic malignant tumours.

AIM: The Posterior Midsagittal Approach (PMA), originally used for the treatment of anorectal malformations, provides a wide exposure of the pelvic floor. Aim of this study is to describe and discuss the PMA in the treatment of intrapelvic malignant tumours which could not be radically excised by laparotomy alone. METHODS: Since 1997, four children were operated upon for pelvic Ewing's sarcoma, presacral sarcoma, intrapelvic neuroblastoma and prostatic rhabdomyosarcoma, respectively. The age of the patients ranged from two to eleven years. Two of them were males and two were females. In all patients, a laparotomy was performed, but inability to remove the tumour radically using this approach led to PMA. A midline incision extending between the tip of the coccyx and the posterior margin of the anus was made. The rectum was mobilised and retracted to the right side, using traction slings. Exposure of the lower part of the tumours was adequate, thus allowing them to be dissected in the midline anteriorly to the sacrum. RESULTS: In the patient with neuroblastoma diffuse bleeding was controlled by packing, which was removed on the 3rd postoperative day. Otherwise the postoperative course in all patients was free of complications. The patient with presacral sarcoma died one year after the operation from generalised disease. The remaining three patients are free of disease and continent two to six years postoperatively. CONCLUSION: PMA is a safe procedure enabling radical excision of intrapelvic malignant tumours which are too high to be approached through the perineum and too low to be reached by laparotomy alone. The posterior midsagittal incision and lateral traction of the rectum permits easy access to the tumour and radical excision, without disturbance of the sphincteric function. Colostomy is not necessary.

Intraabdominal desmoplastic small round cell tumour: report of two cases in paediatric patients.

Intraabdominal Desmoplastic Small Round Cell Tumour (IDSRCT) is a very rare neoplasia with a unique immunoprofile. Children and young adults are most commonly affected. We report two cases with IDSRCT in children who initially presented with ascites, pain and abdominal mass. Complete surgical excision was possible only in one patient. Although both patients underwent multiagent chemotherapy, they had a relapse of the disease. One patient died two years after diagnosis. The tumour has a very poor prognosis. Survival is correlated to the radical resection of the tumour combined with intense chemotherapy and radiotherapy.

Diffuse splenic and visceral hemangiomas complicated by chronic consumption coagulopathy.

The case of a 7-year-old girl with a 2 year history of easy bruising associated with thrombocytopenia is reported. On admission she presented with ecchymoses, abdominal distention and splenomegaly. Hemostasis investigation revealed a consumption coagulopathy. Several radiological studies failed to confirm the diagnosis of diffuse splenic and visceral hemangiomatosis, which was eventually established by an explorative laparotomy. Platelet count and the other coagulation abnormalities progressively returned to normal after splenectomy, although the remaining hemangiomas were extensive.

Isolated Caroli's disease presenting as an exophytic mass in the liver.

A case of isolated Caroli's disease in a 2-year-old female presented as an exophytic cystic mass of the liver. A palpable mass below the right costal margin and displacement of the right colon were the main points of clinical presentation. Preoperatively, sonographic signs of intraluminal vascular tracts were identified. At laparotomy, the mass was found at the inferior border of the right lobe of the liver next to the gallbladder and was easily removed by wedge excision. Intraoperative liver biopsies and histologic findings of the excised lesion confirmed the diagnosis.

Repair of congenital H-type vestibuloanorectal fistula through the posterior midsagittal approach: case report.

The posterior midsagittal approach was successfully used for the repair of congenital H-type vestibuloanorectal fistula in a patient 1.5 months old. Technical details of the operation which consisted of two main parts are described. Identification and dissection of the fistulous tract started following midsagittal division of the sphincters and the posterior half of the rectum with the patient in a knee-chest position. The dissected part of the fistulous tract was inverted per vagina. Completion of dissection and excision of the fistulous tract were accomplished with the patient in the lithotomy position. The main advantage of the procedure was the ability to completely excise the fistula under direct vision.

Total tubular duplication of the colon and distal ileum combined with transmesenteric hernia: surgical management and long-term-results.

A two-year-old male with total tubular duplication of the colon and distal ileum combined with transmesenteric hernia is presented. Recurrent abdominal pain, distention of the abdomen and chronic constipation were the main complaints. A side-to-side anastomosis created through the lowermost part of the common wall of the duplication and colon proper, plus closure of the mesenteric defect and excision of the duplicated ileal loops relieved the patient of all symptoms. Postoperative stagnation of the stool in the closed hollow end of the duplication caused distention developing into a prolapsing pouch which required excision by transanal approach six years after the laparotomy.

Inflammatory pseudotumor of the liver in children. A case report and review of the literature.

A case of hepatic inflammatory pseudotumor with preoperative diagnosis of malignancy in a 14-year-old girl is reported. The lesion manifested clinically with prolonged fever of unknown etiology. Accurate diagnosis was established by laparotomy, resection of the tumor and histological examination. The patient remains asymptomatic during a 2-year follow-up. Inflammatory pseudotumor of the liver should be considered in the differential diagnosis of such cases and the treatment might be more conservative after accurate diagnosis establishment, which is nevertheless difficult to achieve.

Urethral advancement, glanduloplasty and preputioplasty in distal hypospadias.

One hundred and thirty boys with subcoronal (78) and subglandular (52) hypospadias were submitted to Urethral Advancement, Glanduloplasty and Preputioplasty (URAGP). The main steps of the operation were the following: blunt dissection and mobilization of the urethra as extensively as needed to reach the tip of the glans without tension; embedding of the urethra into a groove of the glans created by excision of tissue between two incisions converging from each side of the primary meatal location up to the tip of the glans; fixation of the meatus up to the tip of the glans and suturing of the glandular flaps over the mobilized urethra; shaping the foreskin to normal appearance. Reconstruction was uncomplicated in 117 patients (90%). Postoperative fistulae and urethral retraction to an abnormal position occurred in 7 and 6 cases respectively. All meatal retractions and 2 fistulae next to the meatus were dealt with successfully using the Mathieu procedure. On the basis of this experience we conclude that the technique is applicable to any type of distal hypospadias regardless of the severity of glandular ventriflexion, it is amenable to the Mathieu procedure in case of failure and gives good functional and cosmetic results.

Reimplantation of the ureter in a transtrigonal mucosal groove.

Reimplantation of the ureter into a groove of the bladder mucosa has been used to simplify the procedure of reimplantation and overcome problems in trabeculated bladders by obviating the creation of a submucosal tunnel. Further experience is presented regarding this procedure on 40 ureters of patients with unilateral or bilateral vesicoureteric reflux, ureteric orifice(s) stenosis, bladder diverticula and posterior urethral valves submitted to simultaneous resection. Intra-operative observations, post-operative cystography and long-term results led to the conclusion that the procedure is technically easy, facilitates reimplantation in trabeculated bladders, safeguards the valve mechanism by preventing vesicoureteric reflux and shortens hospitalisation time.