Challenging management of a baby with congenital multiple intestinal atresia, trisomy 18 and extremely low birth weight: a case report

Abstract Background Extremely low birth weight (< 1000 g) still influences postsurgical prognosis in the neonatal and infantile periods. Additionally, the life expectancy of neonates with trisomy 18 is extremely poor owing to various comorbidities. Therefore, it takes courage to perform laparotom...

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Main Authors: Mitsumasa Okamoto, Sachiyo Fukushima, Satoshi Okada, Yudai Tsuruno, Hiroaki Fukuzawa, Tomoaki Ioroi, Masaaki Kugo
Format: Article
Language:English
Published: Japan Surgical Society 2023-10-01
Series:Surgical Case Reports
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Online Access:https://doi.org/10.1186/s40792-023-01761-1
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author Mitsumasa Okamoto
Sachiyo Fukushima
Satoshi Okada
Yudai Tsuruno
Hiroaki Fukuzawa
Tomoaki Ioroi
Masaaki Kugo
author_facet Mitsumasa Okamoto
Sachiyo Fukushima
Satoshi Okada
Yudai Tsuruno
Hiroaki Fukuzawa
Tomoaki Ioroi
Masaaki Kugo
author_sort Mitsumasa Okamoto
collection DOAJ
description Abstract Background Extremely low birth weight (< 1000 g) still influences postsurgical prognosis in the neonatal and infantile periods. Additionally, the life expectancy of neonates with trisomy 18 is extremely poor owing to various comorbidities. Therefore, it takes courage to perform laparotomy for the purpose of treatment of congenital multiple intestinal atresia in a baby with an unpredictable life prognosis. Case presentation Fetal ultrasonography revealed cardiac malformation, intestinal dilation, and physical characteristics suggestive of a chromosomal abnormality in this case. The patient was diagnosed with trisomy 18 after birth, with an extremely low birth weight. An atrial septal defect, ventricular septal defect, dilated jejunum, and a very thin collapsed small intestine were found on ultrasonography. With a diagnosis of congenital small intestinal atresia, a challenging laparotomy was done at 3 days of age, with jejunal atresia and multiple distal small intestinal atresia were observed. The jejunal end and distal small intestinal stump were separated into stomas at the wound edge. Hypertrophic pyloric stenosis developed at the age of 3 months and resolved with medication. The patient gained weight (2 kg) by daily stool injection into anal side of the intestine and decompression against poor peritonitis of dilated jejunum using enteral feeding tube for the long period. Finally, we could perform intestinal reconstruction safely and successfully at the age of 9 months. Tracheotomy was performed due to difficulty in extubation associated with chronic lung disease. The patient was discharged at the age of 1 year and 3 months, and no major problems were noted at the age of 2 years. Conclusions We treat congenital intestinal atresia in extremely low birth weight infants with severe chromosomal abnormalities and severe cardiac malformations as follows: Stoma creation is performed quickly to avoid deterioration of the patient's hemodynamics. After that, while continuing enteric management, palliative cardiovascular surgery is performed as necessary, and the patient's body weight and intestinal tract status are determined to allow safe intestinal reconstruction.
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spelling doaj-art-69ac7aeb48fa4a1391c8de92736433132025-08-20T03:56:19ZengJapan Surgical SocietySurgical Case Reports2198-77932023-10-01911510.1186/s40792-023-01761-1Challenging management of a baby with congenital multiple intestinal atresia, trisomy 18 and extremely low birth weight: a case reportMitsumasa Okamoto0Sachiyo Fukushima1Satoshi Okada2Yudai Tsuruno3Hiroaki Fukuzawa4Tomoaki Ioroi5Masaaki Kugo6Department of Pediatric Surgery, Himeji Red Cross HospitalDepartment of Neonatology, Himeji Red Cross HospitalDepartment of Neonatology, Himeji Red Cross HospitalDepartment of Pediatric Surgery, Himeji Red Cross HospitalDepartment of Pediatric Surgery, Himeji Red Cross HospitalDepartment of Neonatology, Himeji Red Cross HospitalDepartment of Neonatology, Himeji Red Cross HospitalAbstract Background Extremely low birth weight (< 1000 g) still influences postsurgical prognosis in the neonatal and infantile periods. Additionally, the life expectancy of neonates with trisomy 18 is extremely poor owing to various comorbidities. Therefore, it takes courage to perform laparotomy for the purpose of treatment of congenital multiple intestinal atresia in a baby with an unpredictable life prognosis. Case presentation Fetal ultrasonography revealed cardiac malformation, intestinal dilation, and physical characteristics suggestive of a chromosomal abnormality in this case. The patient was diagnosed with trisomy 18 after birth, with an extremely low birth weight. An atrial septal defect, ventricular septal defect, dilated jejunum, and a very thin collapsed small intestine were found on ultrasonography. With a diagnosis of congenital small intestinal atresia, a challenging laparotomy was done at 3 days of age, with jejunal atresia and multiple distal small intestinal atresia were observed. The jejunal end and distal small intestinal stump were separated into stomas at the wound edge. Hypertrophic pyloric stenosis developed at the age of 3 months and resolved with medication. The patient gained weight (2 kg) by daily stool injection into anal side of the intestine and decompression against poor peritonitis of dilated jejunum using enteral feeding tube for the long period. Finally, we could perform intestinal reconstruction safely and successfully at the age of 9 months. Tracheotomy was performed due to difficulty in extubation associated with chronic lung disease. The patient was discharged at the age of 1 year and 3 months, and no major problems were noted at the age of 2 years. Conclusions We treat congenital intestinal atresia in extremely low birth weight infants with severe chromosomal abnormalities and severe cardiac malformations as follows: Stoma creation is performed quickly to avoid deterioration of the patient's hemodynamics. After that, while continuing enteric management, palliative cardiovascular surgery is performed as necessary, and the patient's body weight and intestinal tract status are determined to allow safe intestinal reconstruction.https://doi.org/10.1186/s40792-023-01761-1Congenital multiple intestinal atresiaExtremely low birth weightLife prognosisTrisomy 18
spellingShingle Mitsumasa Okamoto
Sachiyo Fukushima
Satoshi Okada
Yudai Tsuruno
Hiroaki Fukuzawa
Tomoaki Ioroi
Masaaki Kugo
Challenging management of a baby with congenital multiple intestinal atresia, trisomy 18 and extremely low birth weight: a case report
Surgical Case Reports
Congenital multiple intestinal atresia
Extremely low birth weight
Life prognosis
Trisomy 18
title Challenging management of a baby with congenital multiple intestinal atresia, trisomy 18 and extremely low birth weight: a case report
title_full Challenging management of a baby with congenital multiple intestinal atresia, trisomy 18 and extremely low birth weight: a case report
title_fullStr Challenging management of a baby with congenital multiple intestinal atresia, trisomy 18 and extremely low birth weight: a case report
title_full_unstemmed Challenging management of a baby with congenital multiple intestinal atresia, trisomy 18 and extremely low birth weight: a case report
title_short Challenging management of a baby with congenital multiple intestinal atresia, trisomy 18 and extremely low birth weight: a case report
title_sort challenging management of a baby with congenital multiple intestinal atresia trisomy 18 and extremely low birth weight a case report
topic Congenital multiple intestinal atresia
Extremely low birth weight
Life prognosis
Trisomy 18
url https://doi.org/10.1186/s40792-023-01761-1
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