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复杂型腹裂:一家转诊中心的临床谱及新生儿结局

Complex gastroschisis: Clinical spectrum and neonatal outcomes at a referral center.

作者信息

Laje Pablo, Fraga Maria V, Peranteau William H, Hedrick Holly L, Khalek Nahla, Gebb Juliana S, Moldenhauer Julie S, Johnson Mark P, Flake Alan W, Adzick N Scott

机构信息

Division of General, Thoracic and Fetal Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Division of General, Thoracic and Fetal Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

出版信息

J Pediatr Surg. 2018 Oct;53(10):1904-1907. doi: 10.1016/j.jpedsurg.2018.03.011. Epub 2018 Mar 14.

DOI:10.1016/j.jpedsurg.2018.03.011
PMID:29628208
Abstract

AIM OF THE STUDY

To evaluate the outcomes of neonates with complex gastroschisis (GC), and correlate outcomes with each type of complication.

METHODS

Retrospective review of patients with complex GC owing to prenatal and/or postnatal abdominal complications; 2008-2016. Primary outcomes: time to discontinue parenteral nutrition (off-PN), length of stay (LOS) and neonatal survival.

MAIN RESULTS

We treated 58 patients with complex gastroschisis owing to abdominal complications, which were: intestinal necrosis at birth (n=9), intestinal atresia (n=16), medical necrotizing enterocolitis (NEC) (n=15), surgical NEC (n=1), in utero volvulus (n=1), vanishing gastroschisis (n=2), severe intestinal dysmotility (n=1), delayed abdominal closure (n=3), abdominal compartment syndrome (n=2) and hiatal hernia/severe gastroesophageal reflux disease (GERD; n=11). The off-PN time and LOS of the whole group were 92 (35-255) and 119 (42-282) days, significantly longer than those of a demographically equivalent contemporaneous series of 125 patients with uncomplicated gastroschisis (off-PN 32 [12-105] days [p<0.001]; LOS 41 [18-150] days [p<0.001]). Patients with intestinal necrosis at birth or with intestinal atresia had the longest off-PN and LOS times (133 [38-255] / 157 [43-282] and 114 [36-222] / 143 [42-262] days, respectively), followed by patients with complications of the abdominal wall closure (n=5) (69 [43-93] / 89 [58-110] days), patients with hiatal hernias/severe GERD who required fundoplication (63 [35-84] / 89 [57-123] days) and patients who developed medical NEC (67 [35-103] / 76 [50-113] days). Short-bowel syndrome/PN-dependence occurred in 6/58 (10%) patients (2 vanishing gastroschisis, 1 in utero volvulus, 2 intestinal atresias and 1 bowel necrosis at birth). There were no neonatal mortalities.

CONCLUSION

Gastroschisis can be complicated by a wide variety of prenatal and postnatal events. The most severe outcomes occur in patients with bowel necrosis at birth, intestinal atresias, or vanishing gastroschisis. Complications, however, did not affect neonatal survival in our experience.

LEVEL OF EVIDENCE

III.

摘要

研究目的

评估患有复杂腹裂(GC)的新生儿的预后,并将预后与每种并发症类型相关联。

方法

回顾性分析2008年至2016年因产前和/或产后腹部并发症而患有复杂GC的患者。主要结局指标:停止肠外营养的时间(停用PN)、住院时间(LOS)和新生儿存活率。

主要结果

我们治疗了58例因腹部并发症而患有复杂腹裂的患者,这些并发症包括:出生时肠坏死(n = 9)、肠闭锁(n = 16)、医学性坏死性小肠结肠炎(NEC)(n = 15)、外科性NEC(n = 1)、宫内肠扭转(n = 1)、消失性腹裂(n = 2)、严重肠道动力障碍(n = 1)、延迟腹壁关闭(n = 3)、腹腔间隔室综合征(n = 2)和食管裂孔疝/严重胃食管反流病(GERD;n = 11)。整个组的停用PN时间和LOS分别为92(35 - 255)天和119(42 - 282)天,显著长于同期125例无并发症腹裂患者的相应指标(停用PN 32 [12 - 105]天 [p < 0.001];LOS 41 [18 - 150]天 [p < 0.001])。出生时肠坏死或肠闭锁的患者停用PN和LOS时间最长(分别为133 [38 - 255] / 157 [43 - 282]天和114 [36 - 222] / 143 [42 - 262]天),其次是腹壁关闭并发症患者(n = 5)(69 [43 - 93] / 89 [58 - 110]天)、需要行胃底折叠术的食管裂孔疝/严重GERD患者(63 [35 - 84] / 89 [57 - 123]天)以及发生医学性NEC的患者(67 [35 - 103] / 76 [50 - 113]天)。58例患者中有6例(10%)发生短肠综合征/依赖PN(2例消失性腹裂、1例宫内肠扭转、2例肠闭锁和1例出生时肠坏死)。无新生儿死亡。

结论

腹裂可因多种产前和产后事件而复杂化。最严重的结局发生在出生时肠坏死、肠闭锁或消失性腹裂的患者中。然而,根据我们的经验,并发症并未影响新生儿存活。

证据级别

III级

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