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Immaturity of Ganglion Cells - A Study of Our Own Material

100%
Markiewicz-Kijewska M., Kowalski A., Bacewicz L., Drewniak T., Ismail H., Kluge P., Kaliciński P.
Polish Journal of Surgery
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2009
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vol. 81
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issue 2
95-102
EN
The most common causes of clinical symptoms of bowel obstruction in newborn are the various dysmotylity disorders.The aim of the study was a retrospective analysis of the newborn's case history with the diagnosis of ganglion cells immaturity, their clinical course, diagnostic imagine and treatment in our own material.Material and methods. In the years 1981-2007 we treated in the Department of Pediatric Surgery in Children's Memorial Health Institute 420 children with various dysmotylity disorders so-called dysganglionosis. Among them were 15 infants, who finally were diagnosed ganglion cells immaturity. In all clinical symptoms associated with impaired motoric function of large bowel occurred in the first week of life. We performed a retrospective analysis of the case history of these children's including: demographic data, perinatal anamnesis, the dominant clinical symptoms, how to conduct an emergency and a final treatment, and long term follow-up.Results. The children were born at 28-40 week of pregnancy (mean 35,9 weeks) with the body mass between 0.95 and 4.15 kg (mean 2,72 kg), 7 of them were premature infant in 28-36 week of pregnancy and body mass 950-2900 g (mean 1970 g). All infants after birth were evaluated by the Apgar's scale of the 1-10 point (mean 8.5). The first meconium in the first 24 hours of life passed only 1 infant, the remaining 12 meconium passed delayed from 2 to 4 day of life. In 5 children were present concomitant diseases and malformations making it difficult to establish early correct diagnosis: congenital gastroschisis (1 child), intestinal volvulus (1 child) and necrotizing enterocolitis (NEC) (3 children). Diagnosis of ganglion cells immaturity was found at age from 1 to 365 day on the basis of the clinical course, radiological imagine (11 children) and histopathological test of rectal biopsy specimens (9 children) or surgical biopsy specimens (6 children). Four children were treated medically; the others require the temporary emergence colostomy or ileostomy. Functional maturity of ganglion cells identified between 2 and 16.5 month on the basis of electromanometric study (12 children), and/or rectal biopsy specimens (7 children) and in 3 only on clinical course. GI tract reconstruction was performed in the other 11 children at aged from 4.2 to 20.3 month of life, mean 10.6 month. After GI tract reconstruction in 1 child it was mechanical adhesive ileus and malabsorption of unknown origin requiring partial parenteral nutrition. In another child remained ileus and chronic cholestasis, this patient died after next laparotomy because of bleeding and failure liver function. Other patients live in a good general condition with a normal motoric function of the digestive tract from long term follow-up 1.8 to 17.8 years (mean 6.7 years).Conclusions. Early differential diagnosis of dysganglionosis is difficult, but possible subject to the full panel of diagnostic (radiological imagine, electromanometric and histopathological study) already in newborn with impaired bowel motoric function. Only in such cases with correct diagnosis patient can be adequately treated and to guarantee good initial and definitive treatment.
2
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The First Intestinal-Multivisceral Transplant in Poland - A Case Report

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Kaliciński P., Kamiński A., Markiewicz-Kijewska M., Drewniak T., Manowska M., Kierkuś J., Kluge P.
Polish Journal of Surgery
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2007
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vol. 79
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issue 2
120-125
EN
Intestinal transplantation is becoming more and more successful due to improved surgical techniques, immunosuppression medication and postoperative intensive care. It can be performed as an isolated transplantation or as part of a multivisceral graft including the liver in cases of associated liver cirrhosis requiring total parenteral nutrition (TPN). The authors describe the first intestinal and multivisceral (liver, small bowel, duodenum and pancreas) transplantation in Poland performed in an 11-month old infant with short bowel syndrome and TPN related liver failure. A composite graft was harvested from a deceased 4-month old infant. All transplanted organs showed good function; however, the recipient died of infectious complications one month after transplantation.
3
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Modified Renal Autotransplantation in Children with Renovascular Hypertension

84%
Ostoja-Chyżyńska A., Kaliciński P., Ismail H., Broniszczak D., Markiewicz-Kijewska M., Szymczak M., Kwiatkowski W., Antoniewicz J.
Polish Journal of Surgery
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2009
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vol. 81
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issue 3
142-148
EN
Hypertension in children is often associated with impaired blood flow through the kidneys as a result of the strictures of main trunks of renal arteries. Kidney autotransplantation with kidney implantation into iliac vessels is well recognized treatment for hypertension and arterial nephropathy in children, especially in cases involving complex reconstruction of arteries, or the need of reimplantation of artery outside the pathologically changed aorta.The aim of the study was to compare a group of children operated using classic and modified kidney autotransplantation technique with rotation of the kidney by 180 degrees.Material and methods. For the first time we performed such operation in 2001 in order to obtain favorable arrangement of anastomosed vessels and to avoid the need of shortening and anastomosing of the urether. In patients requiring reconstruction of arteries in the "back table" we usually used the classic method, but in cases with difficulty in matching short vessels, the modified technique was used even after "ex-situ" reconstructions.The subject of the report are 11 children aged from 8 years to 17 years in which we performed a modified kidneys autotransplantation with its rotation. In retrospective analysis we observed: function of operated kidney, occurrence of postoperative complications, need for reoperations or other vascular or urological interventions, degree of normalization of blood pressure.Results. During post operative observation we found no difference in the amount of vascular complications between the two groups. We observed difference in the occurrence of urological complications for the benefit of the group after the rotation of transplanted kidneys. At the same time using this method of operation, we obtained normalization of blood pressure and the possibility of significant reduction or discontinuation of pharmacological treatment. In all patients, we observe normal function of transplanted kidneys, except for one that was removed.Conclusions. Modified method of kidney transplantation with rotation seems to be a good and effective method of treatment in cases of difficulties with vascular and urethral matching. It is safe, reducing urological complications, alternative for classical operations.
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Liver Transplantation in Infants with Body Mass Less than 6 KG

68%
Ismail H., Broniszczak D., Apanasiewicz A., Kasprzyk W., Teisserye J., Bacewicz L., Teisserye M., Markiewicz-Kijewska M., Szymczak M., Jezierska E., Kaliciński P.
Polish Journal of Surgery
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2009
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vol. 81
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issue 2
75-80
EN
Liver transplantation (LTx) is the only treatment of fulminant liver failure and end stage liver disease (ESLD).The aim of the study was to assess indications, status at transplantation, surgical techniques, early and late complications and outcome of liver transplantation in children less than 6 kilograms of body weight.Material and methods. Between 1990 and 2008 in the Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, there were performed 350 LTx. Within them there were 13 (3.7%) newborn and infants with body weight below 6 kilogram. Retrospective analysis was carried out regarding indications for LTx, status at LTx, surgical techniques, early and late follow-up and outcome.Results. Follow-up of 11 living patients ranges from 5 to 111 months (mean 31.5 months). Patients and graft survival is 84.5%. In all patients liver function is very good or good. Advancement in liver surgery and perioperative care caused that LTx in small recipients became possible and are carried out with good outcome.Conclusion. Encouraging results of liver transplantation in newborn and infants caused that lower age and weight limits for liver recipients are no more significant.
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