Secondary closure of a giant omphalocele by translation of the muscular layers: a new method

The current report describes a case of an infant girl with a giant omphalocele in whom a new surgical technique was used for closing the abdominal wall after epithelialization of the omphalocele for 16 months. The technique used was translation of the muscular layers of the abdominal wall. The functional and cosmetic results appear superior compared with other suggested treatments used for this abdominal wall defect.     

Silo reduction of giant omphalocele and gastroschisis utilizing continucous controlled pressure

A method is described utilizing continuous controlled pressure to achieve smooth, rapid, and safe silo reduction of an anterior abdominal-wall defect. A metal tube with larger wheels at each end is suspended by runners and counterweights to slowly roll the silo and squeeze the contents into the abdominal cavity. Accepted: 26 April 1999     

Intraoperative vascular assessment for estimation of risk in primary closure of omphalocele and gastroschisis

Surgical repair of anterior abdominal wall defects is often complicated by a discrepancy between the eviscerated organs and the intra-abdominal space available. Primary closure of the abdominal wall may result in increased intra-abdominal pressure and consecutive circulatory impairment. We report the results of a retrospective and consecutive prospective study evaluating the influence of different witameters on mortality and morbidity in children with gastroschitis and omphalocele. Both studies demonstrated that real-time sonographic investigations, intraoperative Doppler duplex sonography, and colour-coded Doppler sonography provide the oppurtunity to collect objective intraoperative data. Our data indicate that intraoperative vascular assessment facilitates the discrimination between infants who benefit from primary closure and those in whom a staged repair is the treatment of choice.     

程序化包扎法、延期手术治疗巨型脐膨出的疗效分析

目的探讨新生儿期采用程序化包扎法、延期手术治疗巨型脐膨出的安全性和有效性。方法回顾性分析2016年12月至2018年7月广州市妇女儿童医疗中心收治的采用程序化包扎法,延期手术治疗的巨型脐膨出患儿临床资料。结果7例患儿出生时囊膜均无破裂,包扎期待治疗期间无囊膜感染、破裂、肠梗阻、中断包扎等并发症发生,未再次入院;囊膜达到干痂期中位数时间为5(4~5)d,囊膜完成上皮化中位数时间为65(57~68)d,家长培训时间中位数6(3~12)d,住院时间中位数21(19~29)d;7例患儿均存活,其中6例行腹壁缺损修补手术,2例术后分别需要5 h和17 h呼吸机支持,住院时间中位数9.5(7.25~10.25)d,术后均未出现伤口预后不良、切口疝等手术并发症。1例正在等待延期修补术。结论程序化包扎法、延期手术治疗巨型脐膨出的方法是安全有效的,其对巨型脐膨出远期预后的影响需进一步随访研究。     

The pathogenesis of omphalocele and gastroschisis

The embryology of gastroschisis and omphalocele remains a matter of speculation. Most authors still assume that they represent separate entities with a different pathology and embryology. In contrast, others feel that gastrochisis is simply the end-result of a ruptured omphalocele. Reviewing the current literature on the normal and abnormal embryology of the anterior abdominal wall, it becomes obvious that appropriate embryological knowledge of these processes is still missing. Animal models are not at hand that would allow clear definitions of morphological changes unique to either malformation. Nevertheless, our own observations of the pathological anatomy of these anomalies lead us to believe that the abdominal wall defects are the result of disturbed development of the embryonic umbilicus. This includes gastroschisis, which is more likely a ruptured small omphalocele than a developmental entity of its own. In our view, the common ventral abdominal wall defects fall into two main categories: (1) large omphaloceles; and (2) small omphaloceles, with gastroschisis as a subentity of this lesion.     

Pre- and postnatal diagnosis of abdominal wall defects (omphalocele and gastroschisis). Case reports

Whereas the omphalocele develops from a malformation of the mesoderm during the embryonal period, the gastroschisis appears to be the result of an intrauterine vascular occlusion; defined as disruption. Definite signs of malformations are seen in omphalocele. In our patient population observed during a three year period, eight cases of abdominal wall defects were seen, four of which with Wiedemann-Beckwith syndrome and one with trisomy 18, two with an isolated omphalocele and one with a gastroschisis. The concomitance of genetic malformation syndromes implies the necessity of intrauterine diagnosis evaluation. In proven cases of chromosomopathies and signs of omphalocele in ultrasound an interruption of pregnancy may be indicated. Similarly with very unfavourable prognosis such as Edwards syndrome, a post partum operative intervention is not necessary. This decision however can only be reached through interdisciplinary consultation between the pediatrician, pediatric surgeon, midwife and parents.     

Prenatal diagnosis and management of gastroschisis and omphalocele

Prenatal diagnosis of gastroschisis (GS) and omphalocele (OC) has allowed improved antenatal and perinatal management. It was the aim of this study to compare prenatal findings and assess fetal outcome. Twenty-four fetus with GS and 33 with OC were diagnosed prenatally. Maternal serum alpha-protein (MSAFP), sonographic (US), and perinatal data were analyzed. The average maternal age for GS was younger than for OC (24 vs. 30 years). The median MSAFP values were 7.7 multiples of median for GS and 3.6 for OC. The initial US diagnosis was made at 22 weeks for GS and at 19 weeks for OC. A pathological karyotype was observed in 4% of the fetuses with GS and 33% with OC. The mortality of fetuses with OC was twice as high as that of those with GS. Accurate prenatal diagnosis of GS and OC as well as their differentiation are of crucial importance for both counselling of the parents and the resulting antenatal consequences, and provide the basis for optimal interdisciplinary co-operation at a perinatal tertiary-care center.     

Muscular flaps for closure of giant abdominal wall defects

In this article we describe the use of muscle flaps in closing giant abdominal wall defects. The technique used to take the flaps from the anterior rectum, internal and external oblique muscles are shown. We emphasize the advantages of using this technique, which avoids the use of synthetic materials, such as Teflon and Marlex mesh. Correspondence to: Antonio F. Gallardo Meza     

Preliminary report on elective preterm delivery at 34 weeks and primary abdominal closure for the management of gastroschisis.

INTRODUCTION: We aimed to critically evaluate elective preterm delivery and immediate abdominal wall closure and other techniques for the management of gastroschisis, hypothesizing that the advantages of an elective preterm delivery outweigh possible complications related to prematurity at birth. PATIENTS AND METHODS: 13 gastroschisis patients were enrolled in the elective preterm delivery program (Group 1) since 1999. Patients were delivered by cesarean section in the 34th gestational week, with immediate primary closure of the defect. Data regarding parameters at and after birth were compared with a historical control group of 10 patients conventionally managed for gastroschisis in a similar period (1994 - 1999) (Group 2). The primary endpoints of this study were the initiation of oral feeding and the length of hospital stay. RESULTS: There was a significantly faster initiation of oral feeding (p = 0.0012) and a shorter hospital stay (p = 0.0160) in Group 1. The postoperative outcome was excellent in all patients. Acute and late complications were fewer and less severe in Group 1 and none were related to prematurity. CONCLUSIONS: Elective preterm delivery appears to be an effective method for the management of gastroschisis, and a method whose advantages thus far have outweighed the possible complications due to prematurity.     

脐带悬吊延期修补治疗巨型脐膨出

目的 探讨脐带悬吊延期修补腹壁缺损治疗巨型脐膨出的临床效果.方法 设计脐带悬吊囊膜、逐渐收紧囊膜还纳膨出内脏器官的方法治疗10例巨型脐膨出,对其治疗过程和临床效果作回顾性总结.结果10例开始悬吊的平均日龄1 d(1～2 d),悬吊平均时间21.7 d(15～37 d).10例均一次手术修补缺损,2例同时行Ladd术.术后2例出现腹壁切口疝,1例出现呼吸困难,行呼吸机辅助通气3d后好转.术后开始进食时间3 d(2～6 d),正常喂养进食平均时间7 d(5～10 d).结论脐带悬吊延期修补治疗巨型脐膨出具有治疗简单、经济、一次手术完成,同时具有Silo袋法及保守疗法的优点,临床效果较好.     

An easy method for adjusting a silo for delayed closure of gastroschisis

A method is described permitting rapid and safe tucks and adjustments to be made to a silo in staged reduction of an anterior abdominal wall defect.     

A strategy for treatment of giant omphalocele

Background  

The management of giant omphalocele (GO) presents a major challenge to pediatric surgeons. Current treatment modalities may result in wound infection, fascial separation, and abdominal domain loss. We report a GO infant who required a delayed closure and was managed using sterile incision drape and polypropylene mesh.     

The influence of delay in closure of the abdominal wall on outcome in gastroschisis

 To evaluate the effect of a delay in closure of the abdominal wall (AWC) on outcome in the management of gastroschisis, a retrospective analysis of 91 babies admitted over a 7-year period (1992–1998) to a single neonatal surgical unit with a diagnosis of gastroschisis was carried out. Antenatal diagnosis was made in 89 (98%) cases. Surgical intervention occurred in 90 babies at a median of 4 h (standard error 0.345, range 0.5–17) post-delivery. In 72 (80%) cases primary closure of the abdominal defect was achieved, with a silo fashioned in the remaining 18 (20%). One infant died prior to AWC. The median time to full oral feeding was 22 days (2.96, 5–160), and to discharge 28 days (4.03, 11–183). There was no correlation between time to AWC and any measured outcome parameter. There was no significant difference in mortality in those patients having closure before 6 h. Thus, no correlation between time to AWC and outcome was demonstrated. This would suggest that the time taken to optimally resuscitate a newborn infant prior to surgical closure does not have an adverse influence upon outcome and is to be recommended. Accepted: 31 January 2000     

Longitudinal growth and late morbidity of survivors of gastroschisis and omphalocele

Of 22 survivors of gastroschisis and omphalocele, most had poor weight gain. Although one-third of gastroschisis babies were small-for-gestational age at birth, no other predisposing factors for poor growth could be demonstrated. No child had intrinsic gastrointestinal or metabolic sequelae at 3 years of age, as demonstrated by radiographic studies, fecal fat excretion, or serum chemistry screen. One-third of those tested had IQs less than 90; five had abnormal electroencephalograms; one had impaired hearing. Intellectual impairment was related to length of hospitalization due to a variety of nongastrointestinal factors. Neither growth nor intellectual development was related to the type of lesion present, even when IQ is corrected for prematurity. Impairment of growth and intellectual outcome may be related to prematurity, small-for-gestation birth weight, and nongastrointestinal neonatal complications.     

Clinical risk factors for gastroschisis and omphalocele in humans: a review of the literature

Gastroschisis and omphalocele are usually considered together since they are both congenital abdominal wall defects, and yet their anatomy, embryogenesis, and clinical presentation and problems are quite different. In addition, it appears that the risk factors for their occurrence differ. Etiologic factors contributing to the development of these defects are unknown. To investigate this we have reviewed reports of risk factors for each anomaly and report them here. We conducted a literature search using PubMed () for risk factors implicated in the development of gastroschisis and omphalocele. The data reviewed here from clinical studies in the literature, closely parallels the data in animal studies which we reported earlier. There is little evidence for a genetic cause in the development of gastroschisis and much evidence supporting the possibility that environmental teratogens are important contributors to the development of this defect. On the other hand, in the case of omphalocele, there was little evidence for environmental factors and substantial data indicating that genetic or familial factors may play an important role.     

An international survey of gastroschisis and omphalocele (490 cases)

An international survey of gastroschisis and omphalocele by questionnaire yeilded 490 cases from 16 pediatric surgery centers on four continents. Factors influencing the management outcome of 203 cases of gastroschisis and 287 cases of omphalocele in the period 1954–1977 are reviewed. In 81% of the gastroschisis cases, the defect was 3 cm or larger in diameter; in only 6% was it as dangerously small as 1 cm. In 58% of cases, only intestines were eviscerated; stomach was included in the eviscerated mass in another 36% and genitourinary organs seldom (6%) were included. In this comparatively early experience with gastroschisis, the operative mortality was 39% but varied widely by and within areas (from 10%–75%). The lowest mortality was associated with primary and skin flap closures. The use of a variety of protheses to cover the eviscerated mass was associated with a high incidence of sepsis and intestinal fistulation. There were 246 cases of non-syndrome omphalocele and 41 cases of syndrome omphalocele. In the non-syndrome omphaloceles, the defect was 2.5 cm or larger in 80% and over 5 cm in 46%. Liver was present in 91% of the large omphalocele sacs, 43% of the intermediate-size sacs and none of the small sacs. Management mortality in non-syndrome omphalocele was 37%. It was lowest in Australia, where only sac painting and primary closure were employed. In syndrome omphalocele mortality was highest (68%) in lower midline syndrome, intermediate (33%) in upper midline syndrome and lowest (0%) in Beckwith-Wiedemann syndrome. The presence of multiple and serious additional malformations was the major factor in omphalocele management mortality. The incidence of additional malformations in small (under 2.5 cm) and large (over 5 cm) omphaloceles was identical (45% vs 46%). Offprint requests to: T. C. Moore     

An international survey of gastroschisis and omphalocele (490 cases)

In an international survey of 16 pediatric surgery centers on four continents, data concerning 490 cases of gastroschisis and omphalocele has been obtained for study. In 203 cases of gastroschisis, additional malformations were found to be infrequent, comparatively mild, rarely multiple, largely limited to the eviscerated gut (atresias and stenoses), and most likely acquired as antenatal complications (volvulus) of the gastroschisis condition. These additional malformations are more properly categorized as an integral part of the gastroschisis disorder, just as is the almost universally associated intestinal malrotation. In 287 cases of omphalocele, 41 cases (14%) were classifiable as syndrome omphalocele (lower-midline syndrome, upper-midline syndrome or Beckwith-Wiedemann syndrome) and 12 cases were trisomy-associated. In omphalocele cases, additional malformations were frequent, serious and generally multiple (74%). The most frequent malformations in omphalocele cases involved the musculoskeletal/neurologic area (32%), followed by the cardiac area (16%) where the malformations tended to be both multiple and serious. In omphalocele cases, the geographic incidence both of numbers of additional malformations and multiplicity of defects was found to be rather similar, with the major geographic difference being found in the relative incidence of cases of syndrome omphalocele. Offprint requests to: T. C. Moore at the above address     

The influence of gestational age,mode of delivery and abdominal wall closure method on the surgical outcome of neonates with uncomplicated gastroschisis

Aim of the study

To evaluate if gestational age (GA), mode of delivery and abdominal wall closure method influence outcomes in uncomplicated gastroschisis (GTC).

Methods

Retrospective review of NICU admissions for gastroschisis, August 2008–July 2016. Primary outcomes were: time to start enteral feeds (on-EF), time to discontinue parenteral nutrition (off-PN), and length of stay (LOS).

Main results

A total of 200 patients with GTC were admitted to our NICU. Patients initially operated elsewhere (n?=?13) were excluded. Patients with medical/surgical complications (n?=?62) were analyzed separately. The study included 125 cases of uncomplicated GTC. There were no statistically significant differences in the outcomes of patients born late preterm (34 0/7–36 6/7; n?=?70) and term (n?=?40): on-EF 19 (5–54) versus 17 (7–34) days (p?=?0.29), off-PN 32 (12–101) versus 30 (16–52) days (p?=?0.46) and LOS 40 (18–137) versus 37 (21–67) days (p?=?0.29), respectively. Patients born before 34 weeks GA (n?=?15) had significantly longer on-EF, off-PN and LOS times compared to late preterm patients: 26 (12–50) days (p?=?0.01), 41 (20–105) days (p?=?0.04) and 62 (34–150) days (p?<?0.01), respectively. There were no significant differences in outcomes between patients delivered by C-section (n?=?62) and patients delivered vaginally (n?=?63): on-EF 20 (5–50) versus 19 (7–54) days (p?=?0.72), off-PN 32 (12–78) versus 33 (15–105) days (p?=?0.83), LOS 42 (18–150) versus 41 (18–139) days (p?=?0.68), respectively. There were significant differences in outcomes between patients who underwent primary reduction (n?=?37) and patients who had a silo (88): on-EF 15 (5–37) versus 22 (6–54) days (p?<?0.01), off-PN 28 (12–52) versus 34 (15–105) days (p?=?0.04), LOS 36 (18–72) versus 44 (21–150) days (p?=?0.04), respectively.

Conclusion

In our experience, late preterm delivery did not affect outcomes compared to term delivery in uncomplicated GTC. Outcomes were also not influenced by the mode of delivery. Patients who underwent primary reduction had better outcomes than patients who underwent silo placement.

     

An international survey of gastroschisis and omphalocele (490 cases)

Gastroschisis, exceedingly rare in 1953 [6], has increased markedly in incidence in recent years. The present international survey by questionnaire of 490 cases of gastroschisis and omphalocele from 16 pediatric surgery centers on 4 continents reveals a marked spurt in the incidence of gastroschisis in 1972 and after. The gastroschisis-to-omphalocele ratio is highest in Scandinavia, northern Europe and the United States. The mothers of gastroschisis patients were found to be significantly younger (21.6 vs 26.7 years) than omphalocele mothers (P<0.0001). Gastroschisis patients were more frequently premature by birth weight (58% vs 23%) and period of gestation (49% vs 16%). The rural-to-urban ratio of omphalocele was fairly uniform in all parts of the world reviewed, whereas that of gastroschisis varied widely from area to area with a range of 13% to 61%. Twinning and a family history of congenital disorders were more frequent in the omphalocele group. There was no evidence, from limited data of the involvement of legal drugs in the etiology of either gastroschisis or omphalocele. The data presented here suggest that omphalocele is more likely due to genetic etiology and gastroschisis more likely results from environmental (drug and pollutant) factors of fairly recent appearance. The need for a continuing and ongoing worldwide study of environmental and other factors in the etiology of both gastroschisis and omphalocele is stressed. Offprint requests to: T. C. Moore at the above address