Clinical problem-solving: Short bowel syndrome in an infant (original) (raw)

The Effect of Ileocecal Valve Removal in a Model of Short Bowel Syndrome

ABCD. Arquivos Brasileiros de Cirurgia Digestiva (São Paulo), 2019

Background: Short bowel syndrome is a harmful condition that needs experimental research. Aim: To assess the impact of the ileocecal valve removal in a model of short bowel syndrome, in order to investigate the evolution of the colon under this circumstance. Method: Fifteen Wistar rats were equitable divided into: Control (Sham), Group I (70% enterectomy preserving ileocecal valve) and Group II (70% enterectomy excluding ileocecal valve). After enterectomy was performed jejunoileal or jejunocecal anastomosis and sacrificed the animals on 30 th postoperative day for histomorphometric study of the colon. During this period, was observed the clinical evolution of the animals weekly including body weight measurement. Results: Group I and II presented progressive loss of weight. In Group I was observed diarrhea, perineal hyperemia and purple color of the colon during autopsy. Histomorphometry assay showed hypertrophy and hyperplasia of colon mucosa in Group I. In Group II the colon wall was thicker due to hypertrophy and muscular hyperplasia, and in mucosa vascular proliferation and inflammatory infiltrate were intense. Conclusion: This short bowel syndrome model is relevant and achieve 100% of survival. Animal's weight loss was not altered by the presence or exclusion of the ileocecal valve. Animals with 70% of small bowel removal and presence of the ileocecal valve attained a better clinical evolution and histological colon adaptation than those without ileocecal valve. RESUMO-Racional: Síndrome do intestino curto é condição clínica crítica e que precisa de pesquisa experimental. Objetivo: Avaliar o impacto da remoção da válvula ileocecal em um modelo de síndrome do intestino curto para investigar o comportamento do cólon nesta circunstância. Método: Quinze ratos Wistar foram divididos em três grupos de cinco: Controle (Sham), grupo I (enterectomia de 70% com preservação da válvula ileocecal), e grupo II (70% enterectomia de 70% excluindo a válvula ileocecal). Após a enterectomia foi restabelecido o trânsito com anastomose jejunoileal no grupo I e jejunocecal no grupo II. Os animais foram sacrificados no 30º dia do pós-operatório para histomorfometria do cólon. Durante este período, observou-se a evolução clínica semanal, incluindo a medição do peso corporal. Resultados: Grupos I e II apresentaram perda progressiva de peso. No grupo I houve diarreia, períneo hiperemiado e cor violácea do cólon durante a autópsia. A histomorfometria mostrou hipertrofia e hiperplasia da mucosa do cólon no grupo I. No grupo II a parede do cólon estava mais espessa devido à hipertrofia e hiperplasia das camadas muscular e mucosa onde a proliferação vascular e infiltração inflamatória foi intensa. Conclusão: Este modelo é factível e atingiu 100% de sobrevida. A perda de peso não foi alterada pela presença ou exclusão da válvula ileocecal. Animais com remoção de 70% do intestino delgado e presença da válvula ileocecal apresentaram melhor evolução clínica e adaptação histológica do cólon que os sem válvula ileocecal.

First STEPs: Serial transverse enteroplasty as a primary procedure in neonates with congenital short bowel

Journal of Pediatric Surgery, 2014

Background: Since its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty (STEP) has been increasingly used as the surgical treatment of choice for patients with refractory short bowel syndrome (SBS). While primary STEP for the treatment of congenital conditions was proposed in the original description of the procedure, emphasis was placed on a delayed or staged approach to these patients. To date, a comprehensive review of the outcomes from this sub-population has not been reported by the International STEP Data Registry. Methods: A retrospective review of the International STEP Data Registry was performed to identify all patients who underwent STEP as a primary operative procedure for the treatment of congenital SBS. Changes in preand post-STEP values were assessed using paired t-tests with significance set at p b 0.05. Data are presented as mean ± standard deviation. Results: Fifteen patients underwent primary STEP for congenital SBS between September 1, 2004, and April 10, 2012. Thirteen patients had follow-up information available. Causes of congenital SBS included closing gastroschisis, small bowel atresia, and midgut volvulus. Twelve patients had pre-and post-STEP bowel measurements taken. Average pre-and post-STEP bowel lengths were 32 ± 16 cm and 47 ± 22 cm, respectively. Intestinal length was increased by a mean of 15 ± 12 cm for a relative small bowel length increase of 50.4 ± 27.3% (p b 0.001). Only one patient required an ostomy at the time of primary STEP. A second patient required a temporary ostomy at 3 months of age that was later closed. There was one death from intestinal failure associated liver disease (IFALD). Another patient experienced IFALD progression and required liver and intestinal transplantation. The most commonly reported complication following primary STEP was obstruction or bowel re-dilatation requiring additional operative interventions. Nine patients underwent second STEP procedures under these circumstances. Eight patients remain dependent on parenteral nutrition, while three patients achieved enteral autonomy. Conclusions: Primary STEP is a feasible and safe surgical option for the treatment of congenital conditions resulting in SBS. Primary STEP establishes early bowel continuity, creates intestinal length from congenitally dilated bowel, and appears to obviate the need for interval stomas and their associated loss of bowel length in neonates with congenital SBS. However, with recent changes in SBS management emphasizing intestinal rehabilitation, additional studies are needed to assess the long-term impact on intestinal adaptation of STEP performed in the neonatal period prior to adoption of this technique.

Congenital intestinal anomalies, neonatal short bowel syndrome, and prenatal/neonatal counseling

Journal of Pediatric Surgery, 2006

Background: Short bowel syndrome (SBS) is a severe malabsorption caused by bowel loss. Congenital intestinal anomalies (CIA) detectable by prenatal ultrasound as jejunoileal atresia, meconium peritonitis, complicated meconium ileus, and fetal volvulus can be responsible for SBS. Aims: This study aims to investigate either frequency of SBS or the morbidity in CIA population during the first admission. Material and methods: Records of CIA treated from 1997 to 2003 were reviewed. The prenatal ultrasound findings were correlated with SBS. Student's t and v 2 tests were performed to analyze epidemiological data, growth at discharge, sepsis, liver disease, catheter-related complications, motor developmental delay, and hospital stay in CIA with and without SBS. Results: Forty-four CIA: SBS developed in 43%, ranging from 83% in volvulus to 0% in complicated meconium ileus. Thirty-six prenatal diagnoses: a strong correlation with SBS was observed in isolated dilated bowel (58%). In SBS neonates, birth weight, gestational age, and growth at discharge were statistically lower, whereas sepsis, motor delay, and hospital stay were statistically higher. Conclusions: Many neonates with CIA detectable by prenatal ultrasound develop SBS. Short bowel syndrome presents a significant higher morbidity. The counseling should stress the frequent association between CIA and SBS as well as the significant morbidity in SBS. D

Improved survival in very short small bowel of infancy with useof long-term parenteral nutrition

Journal of Pediatric Surgery, 1986

Thirteen children with very short small bowel (less than or equal to 38 cm jejunoileum) beginning in the first month of life were enrolled in a home parenteral nutrition program between 1977 and 1984. Their survival is compared with the collective reported experience with short bowel syndrome before 1972: nine (69%) of 13 have survived, compared with seven (23%) of 30 previously. Five discontinued parenteral nutrition after periods of 4 to 32 months of therapy, and have normal growth and development. Two still receive partial (50% and 60%) parenteral nutrition after 9 and 55 months, respectively, and two still receive total parenteral nutrition after 66 and 68 months of therapy, respectively; all four infants have grown normally, and three are developmentally normal. In the combined categories of 15 to 38 cm jejunoileum without the ileocecal valve and less than 15 cm jejunoileum with and without the ileocecal valve, seven (70%) of 10 have survived, compared with none (0%) of 16 before 1972; three of these discontinued parenteral nutrition. Ultimate survival with normal growth without parenteral nutrition is now possible with as little as 11 cm jejunoileum with an intact ileocecal valve and as little as 25 cm jejunoileum without an ileocecal valve.

INCIDENCE AND SHORT-TERM RESULTS OF TREATMENT OF NEONATAL INTESTINAL OBSTRUCTION QR code

Neonatal intestinal obstruction (NIO) is a common and difficult emergency in paediatric surgery. To successfully cope, it is needed to make a quick diagnosis and standard treatment. Management of neonatal intestinal obstruction has improved in many developed countries, but still shows high morbidity and mortality in developing countries. Aim: This study was conducted to assess the incidence and short-term effects of neonatal intestinal obstruction. Place and Duration: In the Department of Paediatric Surgery, Ayub Teaching Hospital Abbottabad for two year duration from March 2017 to March 2019. Methods: This retrospective study involved 84 patients who surgically treated intestinal obstruction during the first month in the emergency department at the Children's Hospital Lahore. Results: Of these 84 patients, 50 are males and 34 females. The average age at the time of presentation was 3.5 (2-10) days for duodenal atresia, 2.5 (3-5) days for jejunoileal atresia, 2 (1-10) days meconium ileus with perforation, volvulus seen in 2 (1-5) days, 7 (5-20) days for colonic atresia, 20 (10-30) days for Hirschsprung disease, 25 (5-30) days for patients with congenital inguinal hernia and 2 (1-4) rectum developmental defects. After resuscitation, all patients underwent surgery. Death occurred in 10 patients (12%). Three patients with atresia of the jejunum had leakage of the anastomosis, underwent a new surgery, but died. Three patients with duodenal atresia died after surgery due to sepsis and DIC. Two patients with major anorectal defects died two days after surgery due to associated cardiac abnormalities, and two patients with sepsis and electrolyte imbalance died after surgery. Conclusion: Anorectal anomalies and congenital inguinal hernia are the most common causes of intestinal obstruction in neonates. Mortality and morbidity remain high compared to statistics from developed countries due to the late presentation and due to improper setting.