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.

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.

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.

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.

Bochdalek's hernia combined with agenesis of the pericardium and intrathoracic solitary cyst of the liver.

A unique case of a neonate with agenesis of the fibrous pericardium combined with Bochdalek's foramen presenting herniation of a solitary cyst of the liver into the left hemithorax is presented. It is emphasised that the lack of the pericardium has not interfered with normal function of the heart, one year after the operation. The possible pathogenesis and embryology of this combination of malformations is discussed.

Basal-cell carcinoma of the lower lid in a 27-month-old child.

Basal-cell carcinoma of the skin is rare in childhood. An unusual case of a 27-month-old girl is described, in whom basal-cell carcinoma afflicted the left lower lid without predisposing cutaneous diseases. The lesion appeared as a naevus, four months before consultation; it developed rapidly and was ulcerated. Treatment was achieved by surgical excision of the lesion. No report of such a young child could be found in the literature.

The collateral arterial network of the spleen following ligation of the splenic artery in traumatic rupture of the spleen; an arteriographic study.

Ligation of the splenic artery, combined with splenorrhaphy, was used successfully on eight children with splenic trauma involving major segmental vessels. Evaluation of the collateral arterial network and of its adequacy was done by means of arteriography on four out of the eight patients. Aortographies were carried out on the twentieth day, the first month, the third month, and the fourth month following the ligation. A collateral arterial network, deriving mainly from the short gastric arteries was detected, dyestream disruption and retrograde filling of the artery. No complications after aortography were noted. Findings demonstrate that collateral blood supply develops rapidly and its pattern is rather uniform.

Bilateral hydronephrosis and hydroureter due to hydatid cyst in the pouch of Douglas.

A case of echinococcus cyst in the pouch of Douglas, causing bilateral hydroureter and hydronephrosis in a 10-year-old child is reported. The urinary tract complications, were readily reversed following surgical removal of the echinococcus cyst.

Duplication of the pylorus: an elusive diagnosis.

A 20-day old female with symptoms of intermittent gastric outlet obstruction since the third day after birth was subjected to surgical exploration. Laparotomy revealed duplication of the pylorus communicating with a normal gastric cavity. The patient was successfully treated by excising the wall separating the duplication from the pylorus. Five cases of pyloric duplication reported so far in the English literature are reviewed.

Ligation of the splenic artery; effects on the injured spleen and its function.

Treatment of splenic trauma by ligating the splenic artery was studied on 28 dogs. Bleeding was arrested, and the animals under experimentation tolerated the ligation of the splenic artery without complications. The histology of the spleen showed no abnormalities 2 mo after the experiment. The traumatized area of the spleen had healed. The platelets and reticulocytes were temporarily increased. The hemotocrit values, WBC counts, and serum immunoglobulin M, remained within normal levels. Bone marrow smears, and scintigrams of the spleen were normal. Ligation of the splenic artery, combined with splenorrhaphy, was also successfully used in children with splenic trauma involving major segmental vessels.

Volvulus of the sigmoid colon.

Sigmoid volvulus in children is quite rare and may occur in acute fulminating form requiring early diagnosis and surgery. In this case, bowel continuity after resection of the necrotic sigmoid was reestablished in a two-stage operation.