Distinguishing outcomes of neonatal intestinal volvulus: Review of our experience over the last 20 years.

AIM: There are two types of intestinal volvulus: midgut (MGV) and segmental (SV). Patients with different types of intestinal volvulus are often included in the same case series, which may affect the perception of how severe "intestinal volvuli" are. We aimed to compare both types of intestinal volvulus. METHODS: This is a retrospective observational study including all patients with MGV and SV up to 28 days of life admitted to a tertiary hospital in Spain over a 20-year-period (1999-2019). A comparison between groups and a logistic regression model for mortality were done. RESULTS: We identified 32 patients: 23 MGV and 9 SV. Malrotation was exclusive of MGV. Prenatal diagnosis, cystic fibrosis, and intestinal resection were significantly more frequent in SV. Surgery was performed at a significantly lower age in SV. The mortality observed in acute MGV with intestinal compromise (41.7%) is four times higher than the mortality of SV (11.1%). The overall mortality of all MGV patients (21.7%) is almost twice that of SV. Mortality was best predicted by the presence of hemodynamic instability (OR 27.5 95% CI 2.50-302.17; p = 0.007). CONCLUSION: SV and MGV have a different clinical presentation. Hemodynamic instability is the major risk factor for death.

Porcine dermal patches as a risk factor for recurrence after congenital diaphragmatic hernia repair.

PURPOSE: Recurrence of congenital diaphragmatic hernia (CDH) is a treatment-related morbidity which can be preventable. There is no consensus about the ideal material for diaphragmatic substitution. The aim of our study is to identify if the use of porcine dermis patches increases the risk of CDH recurrence. METHODS: Retrospective review of medical records of CDH patients treated between 2013 and 2017 in our center was carried out. Demographic, clinical and surgical variables were collected. Regression analysis was performed to identify which factors increase the risk of recurrence. RESULTS: 50 patients entered the study. 94% of the patients had a left CDH, mean observed/expected lung-to-head ratio was 46%. 17 patients underwent a primary closure, the rest a patch closure: 25 Gore-Tex® and 8 porcine dermis patches were used. Seven patients presented recurrence (14%). Median follow-up time was 3.5 years (1.2-6.2). Univariate analysis revealed that the use of a porcine dermis patch (75%) increased the risk of recurrence compared with Gore-Tex® patch (4%) and primary closure (0%) p < 0.001 (HR 58.7; IC 95%: 6.9-501.2; p < 0.001). CONCLUSION: The main risk factor for CDH recurrence is the use of a porcine dermis patch. We do not recommend the use of these patches for CDH repair.

Airway and vascular maturation stimulated by tracheal occlusion do not correlate in the rabbit model of diaphragmatic hernia.

BACKGROUND: In animal models of congenital diaphragmatic hernia (CDH), tracheal occlusion (TO) has induced maturation of both airway spaces and vascular structures. Airway and vascular response to TO are assumed to occur in parallel. This study aims to describe and measure the relationship between airway and vascular maturation induced by TO. METHODS: A rabbit model of CDH on gestational day (GD) 23 and TO on GD 28 (term = GD 31) has been used. Two study groups have been defined: DH (diaphragmatic hernia) and TO (DH treated with TO). Animals were collected on GD 30 and blood flow data of the pulmonary artery (pulsatility index (PI) and fractional moving blood volume) were ultrasonographically measured. Lung morphometry consisted of measurements of radial alveolar count (RAC) and arterial muscular thickness. RESULTS: Animals in the DH group (n = 9) had the worst hemodynamic parameters; their lungs were hypoplastic and had the thickest arterial muscular layer. Animals in the TO group (n = 10) had all these effects reversed. There were no correlations among hemodynamic, airway, and vascular parameters, except for RAC and PI (r = -0.528, P = 0.043). CONCLUSION: Airway and vascular maturation after TO appear to be uncorrelated effects. TO could trigger several pathways that separately regulate airway and vascular responses.

Transpyloric Tube Placement Shortens Time to Full Feeding in Left Congenital Diaphragmatic Hernia.

BACKGROUND: Nutritional complications have an impact in both short- and long-term morbidity of patients with congenital diaphragmatic hernia (CDH). We aimed to compare time to full enteral tube feeding depending on route -gastric (GT) or transpyloric (TPT)- in newborns with left CDH (L-CDH). METHODS: Retrospective cohort study of L-CDH patients admitted to a referral tertiary care NICU between January 2007 and December 2014. Lethal chromosomal abnormalities and death before initiation of enteral nutrition were exclusion criteria. RESULTS: 37 patients were fed through GT, 46 by TPT. TPT children took 11.0 (6.8) days to reach full enteral tube feeding and spent 16.6 (8.1) days on parenteral nutrition vs 16.8 (14.7) days (p = 0.041) and 22.7 (13.5) days (p = 0.020) of GT patients. TPT children had 3.9 (2.4) days of fasting due to GI issues and 20% had episodes of decreased rates of enteral nutrition for extra-GI complications vs 11.4 (11.1) days (p = 0.028) and 49% (p = 0.006). According to the best fitting model (R2 0.383, p < 0.001), the TPT-group achieved full enteral feeding 8.4 days earlier than the GT-group (95% CI -14.76 to - 2.02 days), after adjustment by severity of illness during the first days, o/e LHR_liver and class of diaphragmatic defect. There were no differences in growth outcomes and length of stay between survivors of GT and TPT groups. CONCLUSION: TPT shortens time to full enteral nutrition, especially in the most severe L-CDH patients. We propose that placement of a TPT at the end of the surgical repair procedure should be considered, especially in higher-risk patients. LEVEL OF EVIDENCE: Treatment study, Level III. Retrospective comparative, case-control study.

[The primary care of a patient with a history of a gastrointestinal malformation and abdominal wall or diaphragmatic defects]. / La atención primaria del paciente con el antecedente de una malformación digestiva, defectos de pared abdominal o diafragmáticos.

Survival of patients with congenital malformation has improved over the last decades. Primary care paediatricians must be aware of the most common problems that this group of patients suffers. More importantly, paediatricians can offer a holistic view that is often lost in specialised consultation. This article is focused on common congenital malformation, such as oesophageal atresia, abdominal wall defects, anorectal malformation and Hirschsprung disease, and congenital diaphragmatic hernia. The main problems are shown, with special emphasis on long-term complications and all the dimensions of the individual.

[Intestinal cystic duplication. Case report]. / Duplicación quística intestinal. Caso clínico.

Intestinal cystic duplications are rare congenital anomalies, with an estimated incidence of approximately 1:4500 autopsies. The etiopathogenesis is uncertain. These duplications are cystic, tubular or diverticular structures lined with gastrointestinal mucosa. They share a common smooth muscle wall with the gastrointestinal tract but usually their lumens do not communicate with each other. Gastric duplication cysts represent 7-9% of the gastrointestinal tract duplication. They can be diagnosed prenatally by fetal ultrasound; magnetic resonance imaging characterizes the cyst and excludes other malformations. Postnatal ultrasound shows a characteristic double walled cyst. Newborns are usually asymptomatic, although nonspecific gastrointestinal symptoms, intestinal obstruction due to mass effect, volvulus or infection are described. In asymptomatic patients, clinical follow-up and periodic image controls are recommended. Elective surgical resection is the treatment of choice, using minimally invasive technique whenever possible. A case of prenatally suspected intestinal cystic duplication is presented.

Las duplicaciones intestinales quísticas son anomalías congénitas infrecuentes, con incidencia de 1:4500 autopsias y etiopatogenia incierta. Son estructuras quísticas, tubulares o diverticulares revestidas de mucosa gastrointestinal con pared de músculo liso, adheridas al tracto gastrointestinal, pero no suelen comunicar con la luz intestinal. Los quistes de duplicación gástrica representan el 7-9% de los quistes de duplicación intestinal. Pueden diagnosticarse intraútero mediante ecografía y la resonancia magnética fetal permite caracterizar el quiste y descartar patologías concomitantes. La ecografía posnatal revela una imagen quística de "doble pared". Los neonatos suelen permanecer asintomáticos, pero pueden presentar desde síntomas gastrointestinales inespecíficos hasta síntomas de obstrucción por efecto masa, torsión o infección. En pacientes asintomáticos, se aconseja el seguimiento clínico y estudios de imagen periódicos. El tratamiento es la resección quirúrgica electiva, de manera preferente, con técnicas mínimamente invasivas. Se presenta un caso con sospecha prenatal de quiste de duplicación enteral.

Long term outcome of preterm infants with isolated intestinal perforation: A comparison between primary anastomosis and ileostomy.

AIM OF THE STUDY: Management of isolated intestinal perforation (IIP) poses a challenge for the pediatric surgeon. Intestinal resection and primary anastomosis is considered to be as good as the classical approach, namely, intestinal diversion by ileostomy. However, few reports compare primary anastomosis and ileostomy as IIP treatment. In our institution we favored primary anastomosis as first line treatment whenever patient's condition permitted. Our purpose is to retrospectively compare the outcomes of preterm infants treated with primary anastomosis or ileostomy during a laparotomy in which an IIP was found. METHOD: We identified all newborns who had abdominal operations for IIP from 2000 through 2013. Patients with extensive necrotizing entorocolitis and comorbidities were excluded, as well as those who died in the first 24h. Demographics, type of treatment and complications were reviewed. Major complications included the need for an urgent reoperation, development of late NEC and death. RESULTS: Twenty-three patients with a median gestational age (GA) of 27weeks and median birth weight (BW) of 883g had receive two types of treatment: group I included 9 patients who had intestinal resection of the affected bowel and ileostomy; group PA consisted of 14 patients who had intestinal resection and primary anastomosis. The decision to perform PA or I was based on the surgeon's judgment, in the absence of a specific protocol. There were no significant differences in GA and BW between both groups. Overall mortality was 30.4%. However mortality was restricted to group PA (n=7 cases; 50%) (p=0.019). Most major complications occurred in group PA (71% vs. 11%, p=0.029). There were six cases of late NEC, all in group PA (p=0.048), and four of those patients died. Other than the type of treatment, no differences could be identified between both groups. CONCLUSION: Preterm newborns with IIP are at a higher risk for developing life-threatening complications if treated with primary anastomosis than with ileostomy.

Duplicación quística intestinal: Caso clínico / Intestinal cystic duplication: Case report

Las duplicaciones intestinales quísticas son anomalías congénitas infrecuentes, con incidencia de 1:4500 autopsias y etiopatogenia incierta. Son estructuras quísticas, tubulares o diverticulares revestidas de mucosa gastrointestinal con pared de músculo liso, adheridas al tracto gastrointestinal, pero no suelen comunicar con la luz intestinal. Los quistes de duplicación gástrica representan el 7-9% de los quistes de duplicación intestinal. Pueden diagnosticarse intraútero mediante ecografía y la resonancia magnética fetal permite caracterizar el quiste y descartar patologías concomitantes. La ecografía posnatal revela una imagen quística de "doble pared". Los neonatos suelen permanecer asintomáticos, pero pueden presentar desde síntomas gastrointestinales inespecíficos hasta síntomas de obstrucción por efecto masa, torsión o infección. En pacientes asintomáticos, se aconseja el seguimiento clínico y estudios de imagen periódicos. El tratamiento es la resección quirúrgica electiva, de manera preferente, con técnicas mínimamente invasivas. Se presenta un caso con sospecha prenatal de quiste de duplicación enteral.

Intestinal cystic duplications are rare congenital anomalies, with an estimated incidence of approximately 1:4500 autopsies. The etiopathogenesis is uncertain. These duplications are cystic, tubular or diverticular structures lined with gastrointestinal mucosa. They share a common smooth muscle wall with the gastrointestinal tract but usually their lumens do not communicate with each other. Gastric duplication cysts represent 7-9% of the gastrointestinal tract duplication. They can be diagnosed prenatally by fetal ultrasound; magnetic resonance imaging characterizes the cyst and excludes other malformations. Postnatal ultrasound shows a characteristic double walled cyst. Newborns are usually asymptomatic, although nonspecific gastrointestinal symptoms, intestinal obstruction due to mass effect, volvulus or infection are described. In asymptomatic patients, clinical follow-up and periodic image controls are recommended. Elective surgical resection is the treatment of choice, using minimally invasive technique whenever possible. A case of prenatally suspected intestinal cystic duplication is presented.