Clostridium perfringens-induced massive hemolysis treatment with blood purification to target toxins: a case report.

Clostridium perfringens can rarely cause severe systemic infections, usually from an abdominal source, associated with massive hemolysis, which is usually fatal. Hemolytic anemia and acute renal injury resulting from toxin action are critical for the development of multiple organ dysfunction syndrome (MODs), making this condition a real emergency, requiring multispecialty skills and aggressive multimodal therapies. We herein describe a case of septic shock from acute cholecystitis with massive hemolysis caused by C. perfringens in a 55 year-old man that was successfully treated with early blood purification and continuous renal replacement therapy (CRRT) along with antibiotic therapy and surgery. The effect of the enormous amount of toxins produced by Clostridium which elicit a strong cytokine response and the damage caused by the hemolysis products are the main pathogenetic mechanisms of this rare but lethal clinical entity. The main goal of treatment is to remove toxins from plasma, block toxin action, and further production by achieving bacterial killing with antimicrobial agents and controlling the infectious focus, remove waste products and prevent or limit multiorgan damage. Blood purification techniques play an important role due to a strong pathophysiological rationale, as they can remove toxins and cytokines as well as cell-free products from plasma and also replace renal function. Although this condition is rare and robust data are lacking, blood purification techniques for C. perfringens-induced massive hemolysis are promising and should be further explored.

Analysis of the variation of costs for sewage sludge transport, recovery and disposal in Northern Italy: a recent survey (2015-2021).

The cost for the recovery/disposal of residues produced by wastewater treatment plants (WWTPs) represents a relevant aspect in the economics of WWTP management. We elaborated and applied a desk-based methodology to analyze tender processes and to investigate quantitatively and qualitatively the variability of unit costs for sewage sludge (EWC 190805) management in Northern Italy from 2015 to 2021. We conducted a qualitative survey for operators of the sector. Unit costs for sludge management increased over time. The unit cost was mostly affected by the type of sludge recovery/disposal being the cost for recovery in agriculture (direct or by composting) lower than incineration and landfilling. Other variables influencing the cost were the distance and discount offered by the contractor. Regulatory and judiciary events determined a sudden increase of sludge disposal costs in Italy between 2017 and 2019. Recovery in agriculture, when practicable, remains the preferable option. Results can orient and support the optimization of sludge management costs and be of interest for future studies at national or European level.

Evolution of the surgical management of bowel atresia in newborn: laparoscopically assisted treatment.

PURPOSE: The jejunal-ileal atresias are the most common cause of intestinal occlusion in neonatal period and the duodenal atresia is the most common cause of high bowel obstruction in newborn. The treatment classically used is performed by a transverse sovraumbilical laparotomy. The first "minimal-invasive" approach was performed at the end of the years '70. Nowadays three clusters of thought are delineated about of mini-invasive treatment of small bowel atresias: the intestinal continuity can be established with a circumbilical incision, a totally laparoscopic technique or a laparoscopically assisted approach. It's presented our initial experience of bowel atresia in newborn treated with laparoscopically assisted approach. MATERIALS AND METHODS: Three newborns with prenatal diagnosis of suspected bowel obstruction and one with suspected duodenal atresia, confirmed the diagnosis after birth, underwent to laparoscopically-assisted surgery to correct these congenital anomalies. We have treated with this procedure a duodenal atresia, two jejunal atresias and an ileal form. Surgical management was consisted of an explorative laparoscopy that allowed to recognize and isolate the pathological portion. The second step was traditional open surgery: after the exteriorization of the pathological tract through the umbilical wound, we performed the intestinal anastomosis. RESULTS: In all cases the operation was completed successfully, no conversion was necessary. Intraoperatively no complications occurred. The post-operative course was uneventful. CONCLUSION: The video-assisted technique for the correction of small bowel atresia adds the advantages of the classic laparotomic procedure to the laparoscopic ones.

One trocar assisted pyeloplasty (OTAP): initial experience and codification of a technique.

BACKGROUND: The Anderson-Hynes pyeloplasty is still the gold standard for the surgical treatment of the ureteropelvic junction obstruction (UPJO) in children. To reduce the surgical invasivity, laparoscopic (RPSc) and retroperitoneoscopic (RPSc) pyeloplasty have been proposed but concerns have been expressed. In the RPSc approach, the small operative space and the technical difficulties have limited the application and diffusion of this techinique while in case of laparoscopy, a transperitoneal technique is used to treat a retroperitoneal disease. We report our initial experience with the "one trocar assisted pyeloplasty" (OTAP) in the treatment of UPJO in children. METHODS: From January 2005 to December 2006 sixteen children (13 males--3 females) from 2 to 18 months of age (mean age 5.6 months), with impaired monolateral renal function underwent surgical treatment for UPJO. In 11 cases the obstruction was on the left side and in one case a crossing vessel was found. The renal pelvis was anteriorly reached using a 10 mm operative telescope via a flank 12 mm incision. The UPJ was exteriorized and a dismembered pyeloplasty performed also for the crossing vessel. In two cases a 6 Fr uretero-pyelo-nephrostomy catheter was used. RESULTS: The patients were discharged after four days without complications. The cosmetic results are excellent. In one case, a small perianastomotic leakage was seen. The ultrasonographic follow-up demonstrated the spontaneous resolution of the leakage. The post operative US follow-up showed the reduction of the dilatation in all cases. DISCUSSION: This is an initial experience and a longer follow-up is needed. However, in our opinion the OTAP could be considered as a safe and feasible alternative to the laparoscopic or retroperitoneoscopic pyeloplasty, especially in very small children and also in presence of crossing vessels.

The role of emergency thoracoscopy in the management of congenital cystic adenomatoid malformation of the lung associated with oesophageal atresia.

Congenital cystic adenomatoid malformation of the lung associated with oesophageal atresia is exceptional. The authors describe a case of a mixed type I - II congenital cystic adenomatoid malformation of the left lung associated with oesophageal atresia and tracheooesophageal fistula in a male infant. The interesting aspect of this case is not only the extremely rare association - only two reports in the literature - but the surgical choices. In fact, two weeks after surgical repair of the oesophageal atresia, the growth of the cystic volume of the congenital adenomatoid malformation made respiratory weaning very difficult, and it was therefore decided to solve the respiratory distress by opening the tensional cysts using a thoracoscopic access. The advantage of this treatment was that it decompressed the underlying healthy lung tissue and permitted the expansion of the normal lobar parenchyma. This is a palliative approach that allows the mandatory definitive resection of the affected lung lobe to be postponed until a later time.

Laparoscopic antireflux surgery in neurologically impaired children.

From May 1996 to April 2002, 48 laparoscopic fundoplications were performed after failure of medical treatment in 47 neurologically impaired infants and children affected by gastroesophageal reflux. Indications for surgery included vomiting, recurrent upper airway infections, failure of medical therapy, feeding difficulties with failure to gain weight, and instrumental (barium swallow and pHmetry) diagnosis of gastroesophageal reflux. A standard approach was adopted, with minimal access modifications according to the patients' characteristics. In two patients, laparoscopic surgery had to be converted to open surgery because of severe kyphoscoliosis and accidental left emidiaphragm perforation. In another patient undergoing a laparoscopic Nissen fundoplication, a re-do laparoscopic operation was performed. Postoperative analgesia was administered during the first 12 h, and fluid intake and feeding were begun on days 1 and 2, respectively. All patients clinically improved except two; a paraesophageal hernia developed in one, and a stenosis developed in the other. We strongly believe that laparoscopic fundoplication can be successfully adopted in neurologically impaired children as well as in pediatric patients as a whole, with the same advantages and far fewer drawbacks than are expected in adults.

[Thoracoscopic management of suspected thoraco-pulmonary malignant diseases in pediatric age]. / Management toracoscopico delle patologie toraco-polmonari di sospetta natura tumorale in età pediatrica.

Recent improvements and miniaturization of instruments have encouraged a wider use of thoracoscopy and laparoscopy as a modality for diagnostic and operative procedures in pediatric age. The utility of thoracoscopy in pediatric patients with suspected thoracopulmonary oncological diseases is shown by diagnostic accuracy and, if necessary, the possibility to perform at the meantime a mininvasive surgery. We report the experience of our Institution in 16 patients with suspected thoraco-pulmonary oncological diseases and treated for this reason with thoracoscopy. Thoracoscopic is indicated in cases of suspected oncological diseases in children both for diagnosis and treatment.

Agenesis of the appendix vermiformis.

Agenesis of the appendix vermiformis represents a rare condition that accounts in approximately 1 in 100.000 laparotomies performed for suspected acute appendicitis. L.B., female, born at term after a normal pregnancy. Shortly after birth she presented respiratory distress; chest x-ray showed a left diaphragmatic hernia (CDH). At operation was noted the absence of the appendix and of the mesenteriolum as well together with the presence of a mesenterium commune. Other associated anomalies were detected: dextroposition of the heart, hypoplasia cnemis, hexadactylism of right foot, congenital hip displacement and bilateral congenital cataract. Etiopathogenesis of the agenesis of the appendix can be easily understood from an embryological point of view, following the cecal pole development. Diagnosis of this malformation is possible only after an accurate laparotomic or laparoscopic exploration around the ileocecal and retrocecal zone.

[Malignant primary tumors of the liver in childhood]. / I tumori maligni primitivi del fegato in età pediatrica. Esperienza di un singolo centro.

Twenty-one children (16 males, 5 females) with malignant primary hepatic tumors were admitted to the Pediatric Clinic of the University of Bologna between June 1973 and July 2001. The diagnosis was hepatoblastoma (HBL) in 16 cases; hepatocellular carcinoma (HCA) in 3 cases; undifferentiated sarcoma in 1, malignant rhabdoid tumour of the liver in 1. Median age at diagnosis was 1.8 year (1 mounth-13 years). As to intrahepatic tumor's extension, patients were classified in groups (from I to IV) according to International Society of Pediatric Oncology staging. 2 patients were ascribed to group I; 9 to group II; 9 to group III and I to group IV. At diagnosis 3 pts presented lung metastases. Seventeen patients (81%) were treated with surgery, in 11 cases as first approach to the tumor. In 10 patients, initially with unresesectable tumor, chemotherapy was started first. Drugs used were mostly Cisplatinum or Carboplatinum with Doxorubicin. Sussequently 6 patients were submitted to surgery. At a median follow up of 12.5 years, 52.3% of patients is alive without disease. This percentage rises to 58% taking into consideration only HBL and HCA cases (alive 11/19). We conclude that excluding metastases at diagnosis (3 deaths), the main prognostic factor is resectability and radical surgery: in our experience 4 patients with unresectable tumor died, as 2 patients with microscopical residual after surgery.

Retroperitoneoscopic adrenalectomy in a residual of neuroblastoma.

The authors report a case of retroperitoneoscopic adrenalectomy in a child with neuroblastoma previously treated by chemotherapy. Usually the presence of malignant tumours is a contraindication to retroperitoneoscopic surgery. A restaging of the tumor was done by retroperitoneoscopy. The residual adrenal gland was excised by mini-invasive surgery of the retroperitoneal space. The histopathologic examination confirmed the presence of a residual neuroblastoma in the adrenal gland. This case report shows a possible of a new indication for the retroperitoneoscopy.

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.

The nonpalpable testis: an experience of 132 consecutive videolaparoscopic explorations in 6 years.

From may 1995 to may 2001, 114 children with nonpalpable testis (NPT) were evaluated at our institution (18 babies had bilateral cryptorchidism). The age range was 1-11 years. When ultrasonography and nuclear magnetic resonance cannot show the position of the NPT along the normal pathway, video laparoscopy is essential for diagnostic accuracy. 20 cases were observed to have blind-ending was deferens and testicular vessels; therefore, no other procedure was done. 4 had residual nonfunctional intra-abdominal tissue; in 52 cases, an intra-abdominal testis was found, and 17 microvascular and 26 traditional orchidopexies were performed. The remaining 50 patients (six with bilateral cryptorchidism) had normal vas and spermatic vessels entering the inguinal canal. In 15 cases a normal testis was present, and it was positioned into the scrotum with the standard technique; in 41 cases an atrophic testis was found and was removed through an inguinal approach. Diagnostic laparoscopy permits not only localisation of the testis but also planning for a better therapeutic program with a minimally invasive procedure, thus avoiding the knife in 18% of cases (in our experience 15% of blind-ending and 3% of abdominal vanishing testis).

The role of endoscopic surgery in paediatric oncological diseases.

Recent improvements in video imaging and instrumentation have encouraged a wider use of endoscopic surgery as a modality for diagnostic and operative procedures. To asses the utility and diagnostic accuracy of endoscopic surgery in children with oncological diseases, we reviewed our experience about thirty-eight patients affected by oncological diseases, referred to our Department since 1995. We performed laparoscopy in 22 cases, thoracoscopy in 14, 1 combinated procedure (laparoscopy + thoracoscopy) and retroperitoneoscopy in 1 case. Endoscopic surgery is indicated in cases of paediatric oncological diseases both for diagnosis and treatment.

Congenital giant megaureter: first neonatal case.

The authors report the first neonatal presentation of a congenital giant megaureter (CGM), a very rare unilateral urinary anomaly, which is defined as "a ureter whose lumen is congenitally, focally and segmentally dilated to more than 10 times the normal diameter, in presence of normal bladder volume and function." The definition of CGM is purely descriptive; it can be secondary to several different diseases, and in the series of 21 CGM (12 in duplex system) reported by Chen-Ju Huang, the morphology of the distal ureter varied from a near normal caliber to a ureteral stenosis, a ureterocele or a complete ureteral atresia. J Pediatr Surg 36:944-945.

Laparoscopic surgery of diaphragmatic diseases in children: our experience with five cases.

AIMS: In paediatric surgery the laparoscopic approach can be used to repair diaphragmatic anomalies originating from the abdomen or containing abdominal viscera. Candidates for laparoscopic correction are children with mild symptoms and good respiratory and haemodynamic conditions. The authors present their experience with 5 patients treated successfully for different types of diaphragmatic lesions. PATIENTS AND METHODS: Five children were treated laparoscopically since 1998. Two true Morgagni-Larrey hernias, one recurrent left Bochdalek hernia, one diaphragmatic dysontogenetic cyst and one huge congenital sliding and rolling hiatal hernia. All the herniated viscera were repositioned in the abdomen and the defects--including the diaphragmatic hole at the level of the dysontogenetic cyst--were directly sutured without the use of a mesh. RESULTS: All patients are healthy without signs of recurrence observed at chest X-ray after a follow-up of 3 months to 1 year. DISCUSSION: Under specific conditions the laparoscopic approach can be an effective and more advantageous alternative to laparotomy for diaphragmatic congenital diseases in a paediatric population.

Antenatal diagnosis and early laparoscopic treatment of a rare variation of androgen-insensitivity syndrome.

Androgen-insensitivity syndrome (AIS) (Testicular feminisation or Morris syndrome) is characterised by external female genitalia and bilateral testes with a normal male karyotype. This kind of syndrome is transmitted recessively. Presence of hypoplastic and short "pseudo-vagina" is one of the characteristics of this syndrome. We report here a case of AIS in which there was a well-formed vagina (6 cm). The case was diagnosed antenatally and managed laparoscopically.

Laparoscopic removal of a persistent Müllerian duct in a male: case report.

The authors refer to the use of the laparoscopic approach in Persistent Mullerian Duct Syndrome, starting from a case of "male vagina" with an anomaly inserted left ductus deferens inside the mullerian persistent duct. This patient was operated on using the laparoscopic technique.

Laparoscopic repair of Morgagni-Larrey hernia in a child.

Primary laparoscopic repair of Morgagni-Larrey hernia has been described in adult patients but not in children. This is the first report of primary laparoscopic correction in the pediatric age group without using a prosthesis. A Morgagni-Larrey hernia was found incidentally in a 3-year-old-girl. Laparoscopic correction of the defect was performed. After 6 months the patient is doing well. The chest radiograph shows complete resolution of the hernia. The laparoscopic approach allowed repair the hernia with minimal invasiveness. Laparoscopic correction is not difficult except for those hernias in which dense adhesions are present.