Paola Marchetti

Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases

BackgroundUterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. The diagnosis is generally made only if the suspicion of this genitourinary syndrome is raised.ObjectiveTo highlight the imaging diagnostic clues in this rare condition.Materials and methodsWe report on 11 adolescents with this condition.ResultsSonography mostly allowed the correct diagnosis by showing uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney. MRI provided more detailed information regarding uterine morphology, the continuity with each vaginal channel (obstructed and nonobstructed), and the bloody nature of the contents.ConclusionEarly and accurate diagnosis of this syndrome is important so that adequate and prompt surgical therapy (excision of the vaginal septum) can provide relief of pain and prevent further complications. It is also advisable to look for an obstructed Müllerian system whenever a multicystic dysplastic kidney or the absence of a kidney is discovered in a fetus, or girl postnatally.

Stenting for caustic strictures: Esophageal replacement replaced

METHODS From 1983 to 1996, 31 children with caustic esophageal strictures were seen at Bambino Gesù Childrens Hospital; they were all treated conservatively except for two cases complicated by tracheoesophageal fistula. The remaining 29 patients were divided into three groups depending on the treatment, which was modified over the years. Group A (1983 to 1987) consisted of seven patients treated by periodic dilatations; group B (1988 to 1992) consisted of 10 children treated by 40 days of esophageal stenting plus dexamethasone, 0.5 mg/kg/d plus ranitidine plus no oral feeding for 7 to 10 days; group C (1993 to 1996) consisted of 12 cases treated by 40 days of esophageal stenting plus dexamethasone, 1 mg/kg/d plus omeprazole plus early oral feeding resumption. RESULTS No differences were observed between the three groups of patients with regard to the mean age and to the ingested substance, whereas a significant difference (P = .007) was observed in the mean length of the stricture between group A and C (3.4+/-1.3 and 5.6+/-1.6 cm, respectively). In all but one of the patients (96.5%) complete healing of the stenosis was achieved by conservative treatment, with definitive relief of dysphagia. One patient in group C did not improve after a repeated stenting procedure and was surgically treated. However, in group A, resolution of the stricture was obtained after an average of 19.9+/-14.8 dilatations in a mean period of 25.3+/-17.2 months. In group B, a mean of 12+/-11.3 dilatations were required in a mean period of treatment of 14.1+/-10.6 months. In patients in group C, a mean of 3.5+/-3.2 dilatations were necessary in a mean of 5.8+/-4.8 months. A statistically significant difference was observed both with regard to the number of dilatations and to the duration of treatment, between group A and group C (P = .002) and group B and C (P = .03). CONCLUSION Esophageal replacement should be considered only in cases complicated by tracheoesophageal fistula or in the rare patients who do not respond to repeated esophageal stenting.

Abdominoscrotal hydrocele: a reliable surgical technique

Abdominoscrotal hydrocele (ASH) consists of two large sacs, both abdominal and scrotal, connecting with the inguinal channel. The diagnosis is made only by ultrasound scan. Surgical treatment is mandatory since no spontaneous resolution has been reported. A new surgical procedure used successfully to treat 11 patients with ASH is described.

Surgery for ulcerative colitis in pediatric patients: functional results of 10-year follow-up with straight endorectal pull-through

Children and adolescents affected by ulcerative colitis (UC) frequently require colectomy because of refractory or chronic symptoms. The aim of this paper is to present our experience and 10-year follow-up results of 28 patients who underwent endorectal pull-through (ERPT) as surgical treatment for UC, with special regard to surgical complications, stooling patterns (frequency of defecation, stool consistency, urgency period), fecal incontinence, and quality of life. A retrospective chart review of these patients was performed to evaluate age at colectomy, indication for surgical treatment, operative procedures, technical details, and early or late complications. Frequency of defecation was less than twice per day in two patients, between three and five times per day in nine patients, and more than six times per day in 10 patients. Stool consistency was normal in 14 patients, loose in five, and liquid in only two cases. Urgency period was normal (minutes) in 14 patients, short (seconds) in four, and absent in three. Ten patients (47%) have perfect or good fecal continence, whereas 11 (52%) patients present moderate to total incontinence. The self-reported emotional health was good in most of the patients. A large number are progressing well at school and are coping with their operations. Studies of quality of life in UC patients who underwent surgical treatment in childhood or adolescence, comparing as well the results according to the surgical technique adopted, must be encouraged.

Surgery in disabled children: general gastroenterological aspects.

UNLABELLED Cerebral palsy (CP) is a non-progressive but not unchanging disorder of movement and/or posture, due to an insult to or anomaly of the developing brain. Gastrointestinal surgery can play a role in the treatment of pathologies frequently associated with a condition of neurological impairment such as gastro-oesophageal reflux disease (antireflux procedure), feeding difficulties (percutaneous endoscopic gastrostomy/jejunostomy) and swallowing difficulties (ligation of salivary gland ducts). Gastro-oesophageal reflux occurs in up to 70-75% of children with cerebral palsy. Children with gastro-oesophageal reflux disease (GERD) may present with feeding difficulties, recurrent vomiting and recurrent chest infection associated with poor growth and nutrition, reactive airway disease particularly nocturnal asthma, choking attacks, anaemia, and wheezing. Nutritional deprivation in children with cerebral palsy is the summation of several factors which result in reduced intake. Percutaneous endoscopic gastrostomy (PEG) has radically changed the handling of children with nutritional problems who, before the introduction of this procedure, were force fed parenterally or enterally, by nasogastric tube, conventional surgical gastrostomy or central venous access. In children with CP, PEG is the preferred technique for long-term enteral feeding. Swallowing dysfunction is the main cause of drooling in cerebral palsy, and medical treatment is often inefficient. Surgical treatment involves neurectomy, translocation of the salivary duct, salivary gland resection or salivary duct (parotid and submandibular) ligation. CONCLUSION This review focuses on the role of surgery in managing gastrointestinal aspects in children with CP and, in particular, surgical experience at our department with fundoplication, PEG placement and ligation of salivary ducts.

The Obalon swallowable intragastric balloon in pediatric and adolescent morbid obesity

Background and study aims Incidence of morbid obesity has grown dramatically in the last half century and this phenomenon affects with particular severity the pediatric population. Dietary restrictions and careful programs to improve lifestyle are often ineffective to manage this particular group of patients, due to poor compliance typical of the adolescence. The aim of this study was to evaluate the effectiveness of a new intragastric balloon for treatment of morbidly obese children. Patients and methods A new swallowable intragastric balloon (Obalon) has been used for the first time in 17 obese children in order to assess its safety and effectiveness in terms of reduction in excess weight. In 9 of 17 children a second balloon was placed 30 to 40 days after the first insertion. All devices were endoscopically removed after a mean time of 18 weeks. Results In the group of 16 patients who completed the study (1 patient still under treatment) mean weight decreased from 95.8 ± 18.4 Kg to 83.6 ± 27.1 (P < 0.05). Mean body mass index (BMI) decreased from 35.27± 5.89 (range 30.4 – 48) to 32.25 ± 7.1 (range 23.5 – 45.7) (P > 0.05); mean excess weight, calculated according to Cole’s curves for pediatric populations, decreased from 36.2 ± 15.9 to 29.4 ± 18.3 Kg (P = 0.14), with an %EWL of 20.1 ± 9.8 (range 2.3 – 35.1). Waist circumference decreased from 109 ± 12.3 cm to 99 ± 10.5 cm (P < 0.05). Conclusions Obalon can be administered easily without complications, inducing an appreciable weight loss with a statistically significant reduction in BMI and an improvement in associated comorbidities.

YouTube and Pediatric Surgery. What Is the Danger for Parents

Internet became one of the most important sources of public health informations especially for relatives and/or caregivers of sick children. Use of YouTube as a source of information in pediatric surgery has not been evaluated. In this study, we want to evaluate the use of YouTube as a source of information about one of the most frequent surgical urgency in pediatric patients, the acute appendicitis, to evaluate the risks for patients and parents.

Comet-tail artefacts and abdominal pain: radiological mistake or an underestimated event?

The comet-tail artefact appears as a dense tapering trail of echoes just distal to a strongly reflecting structure. This reverberation type of artefact occurs when there is a marked difference in acoustic impedances between an object and its surrounding.1 A 12-year-old boy presented with a short history of acute abdominal pain without vomiting and fever was studied with an abdominal ultrasound showing the presence of multiple small echogenic foci with comet-tail artefact (figure 1). On subsequent …

Giant appendicolith causing severe lameness in a child

An 8-year-old girl presented with a 1 year history of intermittent abdominal pain in the right lower quadrant of the abdomen associated with lameness that, in the last 3 months, had forced the patient into a wheelchair. Blood tests were normal. Ultrasonography (US) of the abdomen showed an appendicolith measuring 2.5 cm (Fig. 1a), confirmed on X-ray of the hip and pelvis (Fig. 1b) and on computed tomography (CT; Fig. 1c). Trans-umbilical laparoscopic appendectomy was successful and the patient was well at follow up. Previous abdominal US (Fig. 2a) and X-ray of the legs (Fig. 2b,c), performed 1 year before, were judged normal, but on re-analysis the stone was identified (Fig. 2). This was probably due to the fact that on the first plain radiograph a lead plate obscured the stone, while the US was focused mainly on the right hip in order to explain the lameness. The stone had not increased in size (Fig. 3). a b c

Giant varicocele inducing chronic pelvic pain in a girl.

Figure 2. Three-dimensional computed tomography reconstruction showing the complete atresia/occlusion of the inferior vena cava and the dilatation of ovarian veins. A 15-year-old girl presented with a 2-year history of recurrent abdominal pain, stipsis, and pelvic pain. She was operated at birth for a correction of type 1 persistent truncus arteriosus using a “Hancock’s conduct” replaced 3 years after with an aortic homograft. She underwent several cardiac catheterizations. At admission, her vital signs were normal, and laboratory studies and urinalysis were unremarkable. An ultrasound showed the presence of a pelvic varicocele. The angio-computed tomography showed a giant pelvic varicocele (Figure 1) with an inferior vena cava obstruction/atresia (Figure 2). Chronic pelvic pain is a common problem and is defined as continuous or intermittent pain for