Imamoglu Mustafa, Cay Ali, Sarihan Haluk, Sen Yaşar
Department of Pediatric Surgery, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey.
Pediatr Int. 2004 Apr;46(2):167-70. doi: 10.1046/j.1442-200x.2004.01859.x.
Many different and non-specific clinic presentation modes of malrotation anomalies (MA) have been reported after neonatal period. The authors describe four children with MA presented with malabsorption-like clinical features.
Three children aged from 8 months to 7 years, with a history of long-standing diarrhea and failure to thrive attributed to malabsorption, were referred to Department of Pediatric Surgery, for evaluation of suspected MA. Another patient, a 10-year-old boy who was treated for malabsorption for 6 years, presented with acute duodenal obstruction findings. The duration of symptoms averaged 35 months, ranging 8 months to 6 years. All patients had undergone extensive evaluation and empiric trials of different formulas with no improvement in their symptoms. One underwent jejunal biopsy.
Primary presentation complaints were chronic diarrhea and failure to thrive in three patients. Their histories revealed chronic (in one) and intermittent colicky (in two) abdominal pain, and intermittent nonbilious vomiting (in three). The remaining patient presented with acute illness, with chronic diarrhea, failure to thrive, and intermittent abdominal pain and vomiting on his history. They were below 30th percentile according to body weight and height. Laboratory studies revealed hypoproteinemia, hypoalbunemia, raised liver function tests, and anemia in all patients. The patient who presented acutely had double-bubble sign on the plain abdominal film obtained at admission. In the other three, plain films obtained during an attack of abdominal pain and/or vomiting revealed findings of partial intestinal obstruction. The diagnosis was confirmed by upper gastrointestinal series. At their laparotomy, a classical type of malrotation with circumstantial evidence of chronic volvulus was noted. All patients had normal laboratory values between postoperative 3 and 5 weeks, and they were up to 30th percentile at the end of the 6 months.
Malrotation anomalies should be included in the differential diagnosis in a child presented with malabsorption-like clinical features.
新生儿期后，已报道了多种不同且非特异性的旋转不良畸形（MA）临床呈现模式。作者描述了4例表现为吸收不良样临床特征的MA患儿。
3例年龄在8个月至7岁的儿童，有长期腹泻病史且因吸收不良而生长发育迟缓，被转诊至小儿外科，以评估疑似MA。另1例患者是一名10岁男孩，因吸收不良接受了6年治疗，出现急性十二指肠梗阻表现。症状持续时间平均为35个月，范围为8个月至6年。所有患者均接受了广泛评估及不同配方奶的经验性试验，但症状均无改善。其中1例接受了空肠活检。
3例患者的主要就诊主诉为慢性腹泻和生长发育迟缓。他们的病史显示有慢性（1例）和间歇性绞痛（2例）腹痛，以及间歇性非胆汁性呕吐（3例）。其余患者表现为急性疾病，有慢性腹泻、生长发育迟缓，病史中有间歇性腹痛和呕吐。根据体重和身高，他们均低于第30百分位。实验室检查显示所有患者均有低蛋白血症、低白蛋白血症、肝功能检查结果升高及贫血。急性发病的患者入院时腹部平片显示双泡征。另外3例患者在腹痛和/或呕吐发作时拍摄的平片显示有部分肠梗阻表现。上消化道造影确诊了诊断。在剖腹手术中，发现了典型的旋转不良类型，并伴有慢性肠扭转的间接证据。所有患者术后3至5周实验室检查值正常，6个月末达到第30百分位。
对于表现为吸收不良样临床特征的儿童，鉴别诊断中应考虑旋转不良畸形。
