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Biomedical subjects

P Lelli Chiesa

Publications and source records attributed to P Lelli Chiesa.

12 recordsLinked to original sources

Anal canal duplication: a retrospective analysis of 12 cases from two European pediatric surgical departments.

Anal canal duplication (ACD) represents an extremely rare intestinal congenital anomaly of unknown origin. Usually evidenced within 2 years of age, nearly 45% of reported cases present associated malformations such as presacral mass, anorectal malformation (ARM) and genitourinary anomalies. The confirmative diagnosis is histopathological, with evidence of an anal mucosal lining (squamous +/- transitional epithelium), surrounded from a smooth muscle coat and anal glands. We review a conjoined experience from two European pediatric surgical departments. From 1970 to 2005, 12 patients were observed, seven in Pescara, Italy (1997-2005), five in Barcelona, Spain (1970-2004) - mean age at diagnosis 17.8 months, range 0-60; M:F = 1:11. Clinical presentation, diagnostic-surgical approach, and complications were reviewed. According to clinical presentation, patients could be divided in three age groups: asymptomatic (mean age 4.8 months, six patients - one with an associated complex genitourinary malformation, one with a presacral mature teratoma, one with ACD evidenced hysthologically on a retroanal mass removed during the correction of an ARM), mildly symptomatic - constipation, mucous discharge (mean age 29.2 months, four patients - one with associated presacral ependymoma and intestinal neuronal dysplasia type B, one with presacral mass) and complicated - perineal abscess, recurrent fistula (mean age 34 months, two patients). In 11 cases a perianal orifice was evident (ten posteriorly located). The pelvic-MRI was the preferred diagnostic tool in Pescara (5/7, with presacral mass in two patients), fistulography in Barcelona (5/5), where one presacral mass was discovered intraoperatively. Eleven patients underwent surgical removal of the ACD (five perineal approach, five posterior sagittal approach, and one PSARP). Histopathological findings confirmed the diagnosis in operated cases (11). The parents of the male patient denied the consent to surgical treatment. The only major post-operative complication was a sphincteric insufficiency (one case), surgically treated. When facing a perianal orifice, attention should be paid to ACD, particularly in female patients with coexistent genitourinary or intestinal malformations. Pelvic US and MRI are the gold standard to evidence the not rarely associated presacral mass. Surgical early removal (mucosectomy or perineal/posterior sagittal approach, depending on length of ACD and associated presacral mass) is warranted, also in asymptomatic patients, because of the risk of inflammatory complications and cancer (the latter reported in literature in adults).

Anal Canal↗

Serum cystatin C as a reliable marker of changes in glomerular filtration rate in children with urinary tract malformations.

PURPOSE: Cystatin C has been suggested as a simple method of estimating GFR more accurately than creatinine in children. We compared the diagnostic accuracy of cystatin C with serum creatinine and the Schwartz formula for estimating GFR in patients with UTMs. MATERIALS AND METHODS: We prospectively compared 72 patients with UTMs (20 days to 36 months old, 58 males and 14 females) with a group of 72 healthy controls (10 days to 48 months old, 53 males and 19 females). All patients underwent nuclear medicine clearance investigations with (99m)Tc DTPA. RESULTS: Serum concentration of cystatin C revealed a higher correlation with (99m)Tc DTPA (r = 0.62, p <0.001) than serum concentration of creatinine (r = 0.30, p <0.01) or Schwartz formula (r = 0.51, p <0.001). These results were more evident in patients with uropathy (19) with mild renal impairment. Agreement between methods was assessed using Bland Altman analysis. Mean differences between GFR calculated with (99m)Tc DTPA and cystatin C based GFR estimation or Schwartz formula were -2.6% +/- 46.7% and -73.4% +/- 53.6%, respectively. Diagnostic accuracy in identifying decreased GFR measured as AUC was always highest for cystatin C but hardly sufficient for the 3 variables. Cystatin C performed better in the 0 to 6-month-olds (0.70 +/- 0.08 for cystatin C, 0.58 +/- 0.07 for Schwartz estimate) and patients older than 12 months (0.82 +/- 0.09 for cystatin C, 0.65 +/- 0.11 for Schwartz estimate). CONCLUSIONS: Cystatin C proved to be a superior marker rate over serum creatinine in estimating glomerular filtration in children younger than 3 years with UTMs and mild renal impairment, thus, offering a more specific and practical measure for monitoring GFR.

Biomarkers↗

Obstructive ureterocele-an ongoing challenge.

Ureterocele is a cystic dilatation of the intravesical ureter that is most commonly observed in females and children, and usually affects the upper moiety of a complete pyeloureteral duplication. According to their position, ureteroceles are divided into intravesical, when the ureterocele is completely contained inside the bladder, and extravesical when part of the cyst extends to the urethra or bladder neck. Most ureteroceles are diagnosed in utero or immediately after birth during an echographic screening of renal malformations. Severe, febrile urinary tract infection is the most common postnatal presentation of ureteroceles, but they may, rarely, prolapse and acutely obstruct the bladder outlet. Once an ureterocele is identified sonographically, a voiding cystourethrogram to detect vesicoureteral reflux (VUR) and a 99m-technetium dimercapto-succinic acid renal scan to evaluate the function of the different portions of the kidney are mandatory. VUR in the lower pole is observed in 50% of cases and in the contralateral kidney in 25%. Simple endoscopic puncture of the ureterocele has recently been advocated as an emergency therapy for infected or obstructing ureteroceles and as an elective therapy for intravesical ureteroceles. The rate of additional surgery after elective endoscopic puncture of an orthotopic ureterocele ranges from 7 to 23%. Treatment of ectopic ureteroceles is more challenging and both endoscopic puncture and upper pole partial nephrectomy frequently require additional surgery at the bladder level. The reoperation rate after endoscopic treatment varies from 48 to 100%. It is 15 to 20% after upper pole partial nephrectomy if VUR was absent before the operation, but is as high as 50-100% when VUR was present. Thus, endoscopic incision is appropriate as an emergency treatment or when dealing with a completely intravesical ureterocele. Upper pole partial nephrectomy is the elective treatment for an ectopic ureterocele without preoperative VUR. In an ectopic ureterocele with VUR, no matter which type of primary therapy has been chosen, a secondary procedure at the bladder level, involving ureterocele removal and reimplantation of the ureter(s), should be anticipated.

Child↗

Delayed presentation of congenital diaphragmatic Bochdalek hernia. Case report.

The authors describe the case of a 1-month-old patient admitted for crisis of paleness, accompanied by hyporeactivity, tachycardia and polypnea; the symptoms, attenuated after detension of the abdomen, grew worse, with compromise of general conditions and respiratory failure. Chest X-ray shows the herniation of intestinal loops in the thoracic cavity. After the first emergency care, it became necessary to submit the baby to HFO and cardiovascular drugs for some hours before getting oxygenation index (OI) and circulation stabilization. Late presenting congenital diaphragmatic hernia (LCDH) is a condition that can occur during first or second infancy with respiratory or abdominal symptoms, quickly worsening or intermittent. The prognosis is good if diagnosis is timely. Patient stabilization before surgical intervention must be valued not only by O.I. but also by circulation conditions.

Age Factors↗

Blue rubber bleb nevus syndrome and gastrointestinal haemorrhage: which treatment?

PURPOSE: To describe a paediatric case of "Blue Rubber Bleb Nevus Syndrome" (BRBNS) or Bean's syndrome, a rare systemic disorder characterised by cutaneous and gastrointestinal vascular malformations that often lead to overt life-threatening gastrointestinal bleeding or occult blood loss with severe anaemia and iron deficiency. CASE REPORT: A 6-year-old girl with multiple characteristic cutaneous vascular lesions was admitted for a massive rectal bleeding. A few months previously she was endoscopically treated for gastric angiomas which developed into melaena. Preoperative investigations revealed the recurrence of gastric lesions. At laparotomy, more than 25 angiomas of the GI tract were found. Multiple intestinal resections were carried out. RESULTS: No intraoperative or postoperative problems occurred and the girl is completely healthy without further bleeding after a follow-up period of three years. CONCLUSIONS: BRBNS belongs to the group of vascular venous malformations. Most of the time it occurs sporadically, but it can be inherited as an autosomal dominant trait. Recent analysis identified a locus on chromosome 9 responsible for venous malformations. BRBNS patients present typical skin lesions, with some lesions having a rubber-like nipple appearance; the number of skin and GI lesions and the severity of anaemia are correlated. Treatment is dependent on the extent of gut involvement and the severity of the clinical picture. In the absence of massive bleeding, a conservative treatment will be sufficient; otherwise resections are mandatory, but additional lesions may subsequently develop. Management with electrocautery or laser photocoagulation are usually not effective even if some reports recommend them. Pharmacological treatment is useless. Prognosis of BRBNS is unknown.

Child↗

Transumbilical laparoscopic-assisted appendectomy (TULAA): a safe and useful alternative for uncomplicated appendicitis.

AIM: To compare transumbilical laparoscopic-assisted appendectomy (TULAA) and open appendectomy (OA) in cases of acute uncomplicated appendicitis. METHODS: 58 TULAA and 65 OA in patients with a preoperative diagnosis of acute uncomplicated appendicitis are compared. Pneumoperitoneum was obtained with a transumbilical 10 mm trocar (telescope access) and a 5 mm operative channel introduced in the left iliac fossa. Appendectomy was performed outside the abdomen, after the exteriorisation of the appendix through the transumbilical incision. RESULTS: In the TULAA group, operative time and hospital stay were reduced. Conversion was necessary in one case (1.7 %), and in one case (1.7 %), an additional 5 mm operative channel was introduced. Neither intra- or postoperative complications were found in the TULAA group, with excellent cosmetic results. In the OA group we had a wound infection (1.5 %) and in 8 cases (12.3 %) an enlargement of the incision was necessary. CONCLUSIONS: TULAA is the best approach in uncomplicated appendicitis. It is less invasive and traumatic, permits a complete evaluation of the peritoneal cavity with superior cosmetic results, especially in obese patients and in cases of ectopic appendicitis.

Acute Disease↗

The renal-resistive index from the last 3 months of pregnancy to 6 months old.

OBJECTIVE: To measure the renal resistive index (RI, an estimate of renal vascular resistance, used to assess upper tract obstructive uropathy) from the last trimester of pregnancy to the sixth month of life in a large series of healthy subjects, and thus to identify normal values that can be used routinely. During the first semester of life significant haemodynamic changes during the physiological development of the kidney cause considerable variability in RI, which is thus considered less reliable in this period. SUBJECTS AND METHODS: From September 1998 to October 1999, 93 subjects (186 renal units, RU) were enrolled; 32 were fetuses in the last trimester of pregnancy (group 1, 64 RU) and 61 were children (122 RU), 30 aged 0-1 month (group 2, 60 RU), 20 aged 1-3 months (group 3, 40 RU) and 11 aged 3-6 months (group 4, 22 RU). All subjects underwent colour Doppler ultrasonography and the RI of the renal artery was measured for each kidney. RESULTS: The RI was very high in group 1 but decreased noticeably during the first 6 months of life, reaching values similar to those in adults after the third month. The variability in RI continuously declined with age, becoming less important. The normal ranges for groups 1-4 were 0.67-0.88, 0.57-0.90, 0.60-0.84 and 0.65-0.75, respectively. There were no statistically significant differences between the left and right kidneys. CONCLUSION: In the first semester of life there is more than one landmark value of RI depending on the month of age of the infant. This should be considered when assessing upper tract obstructive uropathies after birth and the RI should be compared with the normal ranges reported herein.

Female↗

Conservative treatment of neonatal primary megaureter.

The growing agreement toward the conservative treatment of primary megaureter (PM) is supported by the increasingly frequent reports in the literature of spontaneous resolution of this pathology after few years of attendance. If the PM is asymptomatic without the presence of parenchymatous damage, and the diuretic scintiscan does not show a definite obstruction of the uretero-vesical junction, the conservative treatment should be the choice for the neonatal forms of PM. We report our experience of 14 neonatal PM, conservatively managed out of a total of 22 PM observed between 1990 and 1996. All the patients have been controlled with serial ultrasonography and Tc99 DTPA scintigraphy. Three of them underwent a surgical operation because of persistence or impairment of the clinical and scintigraphic pictures after a 12-18 months' follow-up. Some of the remainders are completely recovered while others are going toward resolution. The conservative treatment of neonatal PM is therefore confirmed to be sure and effective, and in spite of the different attitudes expressed by reliable authors in the up-to-date literature we believe it should be undertaken for the asymptomatic forms in which there is no documented uretero-vesical obstruction.

Dilatation, Pathologic↗

A simple and safe method to visualize the inferior pouch in esophageal atresia without fistula.

The authors studied the true "dynamic" distance between the esophageal stumps in type I atresia in order to perform the delayed anastomosis at the most favorable time. The position of the inferior pouch was fluoroscopically evaluated in four patients, inserting a Hegar dilator through the gastrostomy. The superior esophageal pouch was delineated by a Replogle tube. No anesthesia was required. In all cases the procedure was simple, safe, fast, and accurate. No complications occurred, and patients could be operated upon at the optimal time.

Dilatation↗

Multicystic kidney joined to the homolateral testis by an ectopic vas deferens: embryological hypothesis for a new description.

Ectopic location of the vas deferens is a rare congenital anomaly with multiple variations. The frequent association with ano-rectal abnormalities and hypospadias is well known. We report two cases of a newborn and a 3-year-old child with ectopic vas deferens coupled with a left multicystic kidney without any ureteral structure. Furthermore, the latter had a contralateral vesicoureteral reflux and anal agenesis with recto-bulbar fistula. Our attempt with this report is to explain the development of such anomalous deferential outlet into the multicystic kidney, according to the most qualified theory of the Wolffian duct embryology.

Child, Preschool↗

[Surgical treatment of thoracic wall malformations].

Anterior chest wall deformities in children are the expression of some congenital malformation such as Poland's syndrome and pectus excavatum. The surgical treatment of these malformations affecting children is aimed at correcting large wall defects and stabilizing the chest wall, assuring at the same time chest harmonious growth and postoperative functional assessment. We present the results of the treatment of 2 cases of Poland's syndrome, 20 cases of pectus excavatum and 2 cases of pectus carinatum, with a follow-up of 6 months--9 years.

Cartilage↗