Okamoto Mitsumasa, Fukushima Sachiyo, Okada Satoshi, Tsuruno Yudai, Fukuzawa Hiroaki, Ioroi Tomoaki, Kugo Masaaki
Department of Pediatric Surgery, Himeji Red Cross Hospital, 1-12-1, Shimoteno, Himeji, Hyogo, 670-8540, Japan.
Department of Neonatology, Himeji Red Cross Hospital, 1-12-1, Shimoteno, Himeji, Hyogo, 670-8540, Japan.
Surg Case Rep. 2023 Oct 13;9(1):177. doi: 10.1186/s40792-023-01761-1.
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.
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.
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.
极低出生体重（<1000克）仍会影响新生儿和婴儿期的术后预后。此外，由于各种合并症，18三体新生儿的预期寿命极差。因此，对于一名生命预后不可预测的婴儿，为治疗先天性多发性肠闭锁而进行剖腹手术需要勇气。
胎儿超声检查显示该病例存在心脏畸形、肠道扩张以及提示染色体异常的身体特征。患儿出生后被诊断为18三体，出生体重极低。超声检查发现房间隔缺损、室间隔缺损、空肠扩张以及非常细的塌陷小肠。诊断为先天性小肠闭锁后，在患儿3日龄时进行了具有挑战性的剖腹手术，术中观察到空肠闭锁和多处远端小肠闭锁。空肠末端和远端小肠残端在伤口边缘分别造口。3个月大时出现肥厚性幽门狭窄，经药物治疗后缓解。通过长期经肠道喂养管向肠管肛门侧每日注入粪便并对扩张空肠的不良腹膜炎进行减压，患儿体重增加（2千克）。最终，我们在患儿9个月大时安全且成功地进行了肠道重建。由于慢性肺部疾病导致拔管困难，进行了气管切开术。患儿在1岁3个月时出院，2岁时未发现重大问题。
我们对患有严重染色体异常和严重心脏畸形的极低出生体重儿先天性肠闭锁的治疗方法如下：迅速造口以避免患者血流动力学恶化。此后，在继续肠道管理的同时，根据需要进行姑息性心血管手术，并根据患者体重和肠道状况确定是否允许进行安全的肠道重建。
