Three-dimensional assessment of umbilical vein deviation angle for prediction of liver herniation in left-sided congenital diaphragmatic hernia.

OBJECTIVES: To introduce a new sonographic marker of intrathoracic liver herniation in fetuses with left-sided congenital diaphragmatic hernia (CDH). METHODS: In a consecutive series of fetuses with isolated CDH, an ultrasound volume of the fetal abdomen was acquired. On this volume, offline calculation of the angle formed by the midline of the abdomen (joining the center of the vertebral body to the abdominal insertion of the umbilical cord) and a second line joining the center of the vertebral body to the intra-abdominal convexity of the umbilical vein was carried out to give the umbilical vein deviation angle (UVDA). The UVDA was measured in a group of normal fetuses selected as controls. At follow-up, the presence of liver herniation was investigated in all cases of CDH. UVDA values were compared between the CDH group and controls, and between CDH 'liver-up' vs 'liver-down' cases. A receiver-operating characteristics (ROC) curve was constructed to identify a cut-off value of the UVDA with the highest accuracy in predicting liver herniation in the CDH group. RESULTS: Between 2009 and 2015, 22 cases of left-sided CDH were included in the study group, of which nine cases had liver herniation. Eighty-eight normal fetuses were recruited as controls. The UVDA was significantly higher in the cases vs controls (15.25 ± 7.91° vs 7.68 ± 1.55°; P < 0.0001). Moreover, the UVDA was significantly increased in CDH fetuses with liver-up vs liver-down (21.77 ± 8.79° vs 10.75 ± 2.10°; P < 0.0001). On ROC curve analysis the UVDA showed good prediction of liver herniation (area under the ROC curve, 0.94; P < 0.0001) with the best cut-off of 15.2°, yielding a sensitivity of 89% and a specificity of 100% (P < 0.0001). CONCLUSIONS: In fetuses with CDH, umbilical vein bowing may be quantified by measuring the UVDA using three-dimensional ultrasound. This sonographic marker seems to be an accurate predictor of liver herniation in left-sided CDH. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

[Surgery of congenital malformations in developing countries: experience in 13 humanitarian missions during 9 years]. / Chirurgia delle Malformazioni Congenite nei paesi in via di sviluppo: esperienze maturate nel corso di 13 missioni umanitarie in 9 anni.

The authors report their surgical experience concerning seriously impaired children due to congenital malformations or other non-congenital anomalies such as burns and traumas. All the patients were operated in one of 13 humanitarian missions undertaken in four developing countries. Throughout the "Third World" the demand for reconstructive surgery is extremely high due to the high birth rate and consequently large number of patients, as well as the shortage of both medical staff and supplies. In developing countries Primary Health Care has always been considered a priority and so hospitals, which are used mainly for emergency operations, are usually few in number and badly equipped; elective surgery is considered a luxury. Children with congenital diseases and/or other non-congenital anomalies who are fortunate enough to reach a hospital will often be treated by general surgeons lacking specific training; those children suffering from disabling conditions are often neglected and left to live with their anomalies for the rest of their lives. Our surgical missions have always been undertaken at the request of Catholic missionaries and/or secular organizations which contribute to the individual health schemes of each country. Highly experienced volunteer staff took part in the missions; medical teams are made up of 2 surgeons, one or two anaesthetists and two scrub nurses. The assistance and cooperation of local medical staff was essential in the preselection of cases to be operated while each single medical team provided all the necessary supplies for surgery, which took place in small but well-equipped missionary hospitals provided by the local authorities. A total of 1140 children were operated on during the 13 missions: 32% of these had routine procedures performed in day surgery and 54% underwent major plastic and reconstructive surgery for facial, uro-genital and anorectal malformations or for serious consequences of burns or traumas. The proportion of emergencies was only 3% as these were normally excluded because of the short duration of the missions. 26 patients had to be reoperated as a result of complications or surgical failure and long-term follow-up provided by either the local staff or as a part of later missions was given in over 70% of the major surgery performed. On the whole, the surgical results were highly satisfactory even if a final evaluation should be made taking the cultural factors and the socio-environmental conditions of each individual country into consideration. Such an evaluation should most importantly be made on the basis of the well-being of the patient, general satisfaction of the families involved and improved quality of life of these children.

Treatment of the Mayer-Rokitansky-Küster-Hauser syndrome in Bangladesh: results of 10 total vaginal replacements with sigmoid colon at a missionary hospital.

PURPOSE: We report our experience with treating the Mayer-Rokitansky-Kuster-Hauser syndrome in a developing country. The operations were performed by a pediatric surgical team at a missionary hospital in Khulna, Bangladesh. MATERIALS AND METHODS: From 1995 to 1998, 10 young women with the Mayer-Rokitansky-Kuster-Hauser syndrome underwent complete vaginal replacement. In 4 patients the abnormality was discovered after marriage at the initial sexual approach and, thus, the husband abandoned 3. In 4 of the 10 cases the diagnosis was suspected because of absent menstruation. A physician made the diagnosis in only 2 cases. Preoperatively abdominal ultrasound in 3 patients showed a hypoplastic uterus in all and a right solitary pelvic kidney in 1. In all 10 women a neovagina was created using a 14 cm. segment of sigmoid colon. Two weeks postoperatively patients were taught to dilate and irrigate the neovagina. RESULTS: A minimum of 1 year of followup is available in 7 of the 10 patients. The vagina had a good appearing introitus. Mucous production significantly decreased 3 to 4 months after the operation. Two patients already had an active sexual life. The remaining 3 patients underwent surgery during the last mission and they had no complications 6 months postoperatively. CONCLUSIONS: Our experience shows the feasibility of treating patients with a severe abnormality in a hospital with basic facilities in one of the poorest countries in the world.

Total vaginal replacement with sigmoid colon for partial testicular feminilizing syndrome in pediatric age.

Full text is available as a scanned copy of the original print version.

Pediatric surgical missions in Bangladesh.

Bangladesh is one of the poorest countries in the world, with a population of more than 120 million people living in a territory of only 55,000 square miles. Pediatric surgery is not recognized as an independent branch of surgery: the Dhaka Shishu Hospital is the only pediatric hospital in the country. Sponsored by the World Organization for Pediatric Surgery in Emerging Countries and Azienda Ospedaliera di Parma, the authors performed four surgical missions in Bangladesh in the city of Khulna from 1991 to 1995. During these missions 395 operations were performed on children with general surgical urological, or maxillofacial problems. Besides the humanitarian significance of these missions, it is very edifying for a Western surgeon to observe the natural evolution of untreated congenital malformations.

[Reliability of ultrasonic diagnosis in hypertrophic stenosis of the pylorus]. / L'affidabilità della diagnosi ecografica nella stenosi ipertrofica del piloro.

Ultrasound diagnosis of pyloric stenosis depends on the typical "target" and "cervix" pictures, respectively in transverse and longitudinal scan. Furthermore, pyloric dimensions can be considered pathologic when the muscular wall is greater than or equal to 4 mm. in width, having a maximum diameter greater than or equal to 5 mm. and the pyloric canal is greater than or equal to 18 mm in length. At the Department of Paediatric Surgery of Parma, from 1986 to 1988, 34 infants were studied by ultrasound, basing on clinical suspect of pyloric stenosis. 22 of them were subsequently operated on. Pyloric ultrasound was diagnostic in 20 cases, negative in 1 and doubtful in 1, confirming its reliability in 90-95% of cases. No false positive findings were recorded. Basing on these results, the Authors believe that ultrasound must be the first choice in case of clinical suspect of pyloric stenosis in infancy, because of its high diagnostic value and safety. X-ray studies should be indicated only in doubtful cases.

Diagnostic problems in a Wilms' tumour of the horseshoe kidney.

The authors report on a case of Wilms' tumour in a horseshoe kidney in a child with correct preoperative diagnosis. They emphasise the superiority of the ultrasound and CT scan studies. A brief discussion of the surgical approach in the different presentations is made. The authors suggest, because of the predisposition of the horseshoe kidney to develop Wilms' tumour to perform, an annual ultrasound study for its surveillance.

[Intraluminal diverticulum of the duodenum]. / Diverticule intraluminal du duodénum.

A case of intraluminal duodenal diverticulum associated with partial situs inversus in a 15 year-old girl is reported. Etiopathogenicity is discussed. The pathological characteristics explain that surgery associated with upper digestive endoscopy had to be performed and why endoscopic excision was not possible in this case.

[Gastro-esophageal reflux in childhood. When to operate?]. / Reflusso gastro-esofageo in età pediatrica. Quando operare?

Gastroesophageal reflux has been well described in children as the cause of a variety of symptoms from nutritional to respiratory problems. If the regurgitation and vomiting are very common symptoms in newborns, their persistence after the first months of life will result in pathological entity leading to complications as esophagitis, failure to thrive, respiratory problems. The purpose of this article is to point out the functional and anatomical implications maintaining gastroesophageal reflux in children and the correct indications for surgery.

[Varicocele in childhood]. / Il varicocele in età pediatrica.

The Authors analyze 20 cases of left varicocele, treated surgically, in boys from 10 to 16 years with the mean age of 12 years. During the operation a testicular biopsy was carried out alternatively in both testicles, showing, mostly, a growth arrest of the spermatogenesis. A blood sample from the left internal spermatic vein was taken for the measurement of the testosterone. The Authors discuss the results and emphasize an early correction of the varicocele.

[Meatal advancement and glanuloplasty incorporated (MAGPI): a new surgical technic in the treatment of distal hypospadias]. / MAGPI: una nuova tecnica chirurgica nel trattamento delle ipospadie distali.

The authors refer their experience in the treatment of distal hypospadias using the MAGPI technique. The authors discuss the indications and the details of the operation. Over a 12 month period 20 patients with subcoronal hypospadias underwent repair, with no complication and an excellent cosmetic result. The authors conclude that this technique, as recommended by several authors, is a simple, safe and effective procedure for distal hypospadias.

[Indications for diagnostic and surgical digestive endoscopy in childhood: personal clinical experience]. / Indicazioni all'endoscopia digestiva diagnostica ed operativa in età pediatrica: esperienza clinica personale.

From January 1983 to December 1984 in the Division of Pediatric Surgery of Parma, 112 endoscopies of upper gastro-intestinal tract (UGT) were done. Besides the authors refer 49 endoscopies of the colon. Endoscopic indications of the UGT included evaluation of the esophagus in gastro-esophageal reflux, achalasia, lye ingestion, gastro-duodenal ulcer, bleedings of UGT, removal of foreign bodies. The indication of the colonoscopy included bleedings and all symptoms of colitis. The endoscopic procedures were done under sedation or in general anesthesia.

[Conservative treatment of splenic injuries in childhood]. / Trattamento conservativo dei traumi splenici nell'infanzia.

From 1978 to 1983, 11 children with splenic traumas were observed at the Pediatric Surgery of Parma. The diagnosis was confirmed by splenic scintigraphy with 99m Technetium. Those patients whose condition was stable with intravenous fluids or blood transfusions were not operated on. Emergency laparotomy was necessary only in 3 cases of 11 patients, when the general conditions were unstable.

[Traumatic intramural hematoma of the duodenum. Description of a case]. / Ematoma intramurale traumatico del duodeno. Descrizione di un caso.

The traumatic intramural hematoma of the duodenum is the result of contusion of the retroperitoneal portion of the duodenum. This lesion occurs most frequently in children and can result in complete obstruction of the second and third portion of the duodenum.

[Proposal for difficult nursing in neonatal Hirschsprung's disease]. / Proposte per il nursing difficile nel m. Hirschsprung neonatale.

Often the Hirschsprung's disease presents severe neonatal manifestations: enterocolitis, intestinal occlusion. The authors experienced a conservative treatment in 3 patients with Hirschsprung's disease using elemental diets. This behaviour avoided the colostomy, otherwise necessary with a normal nursing.

[Gastric acidity and serum levels of pepsinogen I and gastrin in children with primary duodenal ulcer]. / Acidez gástrica y niveles séricos de pepsinógeno I y gastrina, en niños con úlcera duodenal primaria.

A population of 8 children aged 7 to 13 and radiologically and/or endoscopically diagnosed of duodenal ulcer is compared with another made up of 12 normal children of similar ages and weights. In both groups gastric secretion, basal and after pentagastrin stimulation, and serum levels of gastrin and pepsinogen I, basal and after pentagastrin stimulation, and serum levels of gastrin and pepsinogen pepsinogen basal and after pentagastrin stimulation, and serum levels of gastrin and pepsinogen I, basal and after proteic meal, were studied. BAO, MAO and PAO were significantly higher in ulcer patients. Gastrinemia, both basal and stimulated, were rather similar in both groups. Serum pepsinogen I was always higher in ulcer patients in the basal state than in their healthy counterparts (greater than ng/ml vs. 30-50 ng/ml), but was not modified by proteic meal in either group. The fact that all ulcer children had a familial history and a basal pepsinogen I elevated, aside with the secretory response to stimulation, suggests that ulcer can be result either of an increased mass or of a higher sensitivity of gastric parietal cells that could be related to genetic factors.

[Clinical approach to constipation: proposal of a diagnostic and therapeutic protocol]. / Contributo all'inquadramento clinico della stipsi: proposta di protocollo diagnostico e terapeutico.

A study concerning constipation in children has been carried out in the University Department of Pediatrics and in the Division of Pediatric Surgery of Parma with the aim of reaching an homogeneus approach: it has been applied to 20 children up to now observed for such a problem. According to clinical and anamnestic criteria, children were divided into 3 groups on the basis of different degree of constipation: 1) moderate, 2) mild, 3) severe. Children belonging to the 2nd and 3rd group underwent barium enema and rectal manometry; two cases of ultrashort Hirschprung's disease and 7 cases of functional megarectum were diagnosed. Different treatment have been employed for each group and good results were observed in 18 cases. The AA. briefly discuss the different aspects concerning constipation and its therapy in childhood including diet, drugs, psychotherapy and surgical treatment.

Effects of somatostatin in a case of severe hypoglycemia due to nesidioblastosis.

The Authors have studied the effects of somatostatin (SRIF) treatment in an infant affected by hypoglycemia due to nesidioblastosis. During iv infusion with SRIF we observed a marked increase of blood glucose levels; concomitantly insulin secretion was almost completely suppressed. In contrast, during treatment with protamine zinc-somatostatin (PZ-SRIF), a long acting SRIF preparation, the blood glucose levels did not rise and insulin concentrations were inappropriately elevated. Therefore in this case the long term treatment of hypoglycemia due to nesidioblastosis with PZ-somatostatin was unfeasible.

[A middle esophageal ring]. / L'anello esofageo medio.

The Authors, having observed a case of middle oesophageal web giving rise to severe dysphagia, discuss the aetiopathogenesis, the diagnosis and therapy of these rare oesophageal lesions. Because of its pathological feature, the reported case was succesfully treated by surgical resection of the ring, using a transthoracic approach.