Colonic atresia: a clinicopathological insight into its etiology.

BACKGROUND: The pathogenesis of colonic atresia (CA) has not been discussed in detail, although the theory of a prenatal vascular insult, hypothesized from patients with small bowel atresia, has been accepted in the past. This review questions this etiology by reviewing all patients with CA treated in two institutions. MATERIAL AND METHODS: A retrospective analysis was done of the medical notes of 30 patients with CA treated in two tertiary centers of neonatal surgery in Glasgow, UK, and in Wroclaw, Poland, over a 30-year period with special emphasis on the intraoperative findings and morphology of the atretic bowel. RESULTS: Thirteen patients had CA in association with an abdominal wall defect. Eleven patients had gastroschisis. A single fibrous cord atresia was noted in 2 patients and it was located in the ascending colon and transverse colon, respectively. Four patients had type IIIa atresia affecting the ascending colon in 3 and transverse colon in one. In 5 patients an extensive defect of the intestine with two atresias of the small and large bowel was noted. In 3 of them, a separate conglomerate of the prolapsed intestine was found to be necrotic or presenting as a cystic structure. Two of these patients had an unusually narrow abdominal wall defect of less than 1 cm. Isolated CA was noted in 17 patients. A type IIIa atresia affecting the right colon was found in 14 of them. In 2 patients the ascending colonic atresia was accompanied by an extensive defect of the transverse colon. A single sigmoid colon atresia and two fibrous cord atresias of the sigmoid and transverse colon were noted in single patients respectively. CONCLUSIONS: Most cases of CA in babies with gastroschisis seem to result from bowel compression within the narrowing abdominal defect. A "two-point constriction" may lead to a wide spectrum of bowel pathologies and the morphology may depend on the viability of the intestinal segment between the atretic jejunum or ileum and the colon. Isolated CA presents with a wider spectrum in terms of anatomical types and location of the bowel pathology, but it seems that type II and IIIa atresias of the right colon may share a similar pathogenesis of temporary constriction within a closing umbilical ring.

Multiple atresia of the small intestine: a 20-year review.

BACKGROUND: Multiple intestinal atresia (MIA) presents with a wide spectrum of bowel pathologies. Its treatment is a challenging task since restoration of anatomical continuity of the affected intestine must be balanced against preservation of the intestine's maximal length. MATERIAL AND METHODS: A retrospective analysis of the medical notes of 26 patients with MIA treated over a 20-year period between 1986 - 2006 was undertaken with a special emphasis on the clinical and surgical perspectives. RESULTS: All 26 cases of MIA were sporadic with no familial history. The mean gestational age and birth weight were 36.1 weeks and 2781 g, respectively. Twenty-three of the infants underwent operative repair within the first days of life. Three patients with gastroschisis had a delayed diagnosis of bowel atresia. The number of atresias per patient ranged from 2 to 10. In 24 newborns atresias were confined to the small bowel, with 2 other patients having additional obstruction of the ascending colon. Various combinations of anatomical types of atresias were found, with type I and type III occurring in 19 patients each. Type II was diagnosed in 7 newborns. Surgical management of MIA consisted of one-stage restoration of bowel continuity with multiple anastomoses and/or enteroplasties in 22 patients. Four patients had an enterostomy performed at initial operation. Early and late postoperative complications requiring operative treatment occurred in 8 patients. The duration of parenteral nutrition ranged from 6 days to 20 months, exceeding 100 days in 6 children. The follow-up ranges from 3 months to 16 years. All the patients are alive and are on a full oral diet. CONCLUSION: Clinical observations of sporadic cases of MIA confined to the small bowel lend support to the hypothesis of a vascular incident etiology. One-stage restoration of intestinal continuity with preservation of maximal intestinal length should be the basic principle of any operative management of MIA. Despite a relatively high morbidity related to the primary damage of the fetal intestine, excellent results with 100 % survival rates can be obtained. After taking the differences in pathogenesis, anatomical and histological features, and the prognosis for sporadic and hereditary forms of MIA into account, these two entities should be classified separately in a modified classification of intestinal atresia.

Unilateral agenesis of the diaphragm: a separate entity or an extremely large defect?

Since the mid-1980s, unilateral agenesis of the diaphragm (DA) has attracted the attention of paediatric surgeons as more babies affected by this extreme form of congenital diaphragmatic hernia (CDH) survive. Some authors believe that it represents a separate clinical entity. We undertook a retrospective analysis of all babies with CDH treated in the South-West Regional Paediatric Surgical Centre in Bristol between 1981 and 1995. Of 108 babies 16 (14.8%) were identified as having DA. All presented with severe respiratory distress from birth. In comparison to the group of patients with postero-lateral hernia, neonates with DA had lower Apgar scores and required longer preoperative stabilisation with inotropic support and vasodilators. Nine were subjected to operation and all required diaphragmatic replacement. Only 3 survived; thus, mortality in the DA group was 81.25%, and among those who underwent surgery 66.6% The same data for babies with postero-lateral hernia were 15.2% and 7.2%, respectively. Our results indicate that DA is associated with high morbidity and mortality, but we have not found any evidence that this anomaly is a distinct entity. In addition, we reviewed all post-mortem reports of fetuses with diaphragmatic defects available for the same period. Of 19 fetuses, 10 (52.6%) had DA. The morphological details of the diaphragmatic defect and the presence of associated anomalies were analysed. Our observations support the hypothesis that DA occurs in the very early stages of embryonic life and may be attributed to developmental arrest of the septum transversum.

[The role of chemotherapy in treatment of nephroblastoma in children. Report of the Polish Pediatric Group re. treatments of solid tumors]. / Udzial chemioterapii w leczeniu nerczaka zarodkowego u dzieci. Raport Zespolu Polskiej Grupy Pediatrycznej ds. Leczenia Guzów Litych.

Retrospective analysis of chemotherapy results of children with nephroblastoma was performed in 220 patients aged from 1 yr to 14 yrs of live in 12 centers. Stage I nephroblastoma was documented in 24.5% but stage II--in 55.3%. Histologically 74.6% cases were diagnosed as medium malignant and 12.7%--high malignant. Therapy results were similar to observed in other centers.

Epidermoid cyst in a wandering spleen.

A wandering spleen and splenic cysts are uncommon conditions in children. A combination of both entities has only rarely been reported in the literature. Another case of this complex pathology in 12-year-old girl is presented. She was initially referred for evaluation of a large abdominal mass. Diagnostic studies raised the suspicion of a hematoma in an abnormally located spleen, but the definitive diagnosis was established at laparotomy. Successful surgical management consisted of total cystectomy and splenopexy.

[Reconstruction of the diaphragm in extensive congenital diaphragmatic hernia]. / Rekonstrukcja przepony w rozleglych wrodzonych przepuklinach przeponowych.

Congenital diaphragmatic hernia presents a wide spectrum of anatomical variants ranging from small defects to a complete absence of the hemidiaphragm. The surgical correction of this congenital anomaly consists of restoring the continuity of the diaphragmatic barrier after the herniated viscera has been replaced into the abdominal cavity. In some babies primary repair of the diaphragm is not possible due to a significant extent of a defect. In these cases prosthetic diaphragm reconstruction must be carried out. In this paper the pathophysiological aspects of reconstruction of the diaphragm in babies affected by congenital diaphragmatic hernia are discussed. The various methods of diaphragmatic repair including use of prosthetic materials, muscle flaps and autogenous graft are reviewed. The results of experimental studies in animals comparing nonabsorbable and absorbable materials and muscle flaps as a diaphragmatic patch are presented. In view of the fact that the modern neonatal intensive medicine allows more babies with an extensive diaphragmatic defect to survive, the use of various reconstructive techniques will be without doubt play a more important role in their surgical management.

[Use of septopal in treatment of acute blood-borne inflammation of bones in children]. / Zastosowanie Septopalu w leczeniu ostrego krwiopochodnego zapalenia kosci u dzieci.

The authors are presenting 70 cases of acute blood-derived inflammation of bones in children treated in the Clinic of Children's Surgery in the Academy of Medicine in Wroclaw in the last 10 years. Gentamicin balls were used for 21 children including 18 newborns and babies. In 16 cases implantation of gentamicin balls was the only way of local treatment, and in 5 cases it was preceded with short-lasting (3-7 days) flowing drain of the inflammatory focus. Implantation of gentamicin balls was renewed after 14-18 days achieving in all cases sterilization of the inflammatory focus within the bone. The applied treatment allowed to reject quickly the general antibiotic therapy. After operational course for all children was normal.