Early weight measures and long-term neuropsychological outcome of critically ill neonates and infants: a secondary analysis of the PEPaNIC trial.

Neonates and infants surviving critical illness show impaired growth during critical illness and are at risk for later neuropsychological impairments. Early identification of individuals most at risk is needed to provide tailored long-term follow-up and care. The research question is whether early growth during hospitalization is associated with growth and neuropsychological outcomes in neonates and infants after pediatric intensive care unit admission (PICU). This is a secondary analysis of the PEPaNIC trial. Weight measurements upon PICU admission, at PICU discharge, at hospital discharge, at 2- and 4-year follow-up, and of different subgroups were compared using (paired) t-tests. Multiple linear regression analyses were performed to investigate the association between early growth in weight measures and neuropsychological outcomes at 4-year follow-up. One hundred twenty-one infants were included, and median age upon admission was 21 days. Growth in weight per week was less than the age-appropriate norm, resulting in a decrease in weight-for-age Z-score during hospitalization. Weight is normalized at 2- and 4-year follow-up. Weight gain in kilograms per week and change in weight Z-score were not associated with neurodevelopmental outcome measures at 4-year follow-up. Lower weight-for-age Z-score at PICU admission and at hospital discharge was associated only with lower weight and height Z-scores at 4-year follow-up. CONCLUSION: Growth in weight during hospital stay of young survivors of critical illness is impaired. Worse early growth in weight is associated with lower weight and height but not with neuropsychological outcomes at 4-year follow-up. WHAT IS KNOWN: • Critically ill neonates and infants show impaired early growth during admission and are at risk for later neuropsychological impairments. • Unraveling the association between early growth and later neuropsychological impairments is crucial since the first year of life is critical for brain development. WHAT IS NEW: • Critically ill neonates and infants had age appropriate weight measures at 4-year follow-up. • Poor growth in weight during hospital stay was not associated with poorer cognitive, emotional, or behavioral functioning four years after critical illness.

Peptide nutrient-energy dense enteral feeding in critically ill infants: an observational study.

BACKGROUND: Enteral feeding is challenging in critically ill infants. Target intakes are often not achieved as a result of fluid restriction, procedural interruptions and perceived enteral feeding intolerance. In those infants perceived to have poor feeding tolerance, the use of a peptide nutrient-energy dense enteral feed (PEF) may improve nutritional intake and minimise feeding interruptions as a result of gastrointestinal symptoms. The aim of this observational study was to characterise the use of a PEF amongst critically ill infants in two paediatric intensive care units (PICUs). METHODS: Records from critically ill infants aged <12 months admitted to two PICUs were retrospectively reviewed with a PICU length of stay (LOS) ≥ 7 days. Achievement of nutritional targets for the duration of PEF was reviewed. Gastrointestinal symptoms, including gastric residual volume, constipation and vomiting, were evaluated as tolerance parameters. RESULTS: In total, 53 infants were included, with a median age on admission of 2.6 months. Median admission weight was 3.9 kg in PICU-1 and 4.7 kg in PICU-2. Median (interquatile range) energy intake in PICU-1 and PICU-2 was 68 (47-92) and 90 (63-124) kcal kg-1 , respectively, and median (interquatile range) protein intake 1.7 (1.1-2.4) g kg-1 and 2.5 (1.6-3.2) g kg-1 , respectively. Feeding was withheld because of feeding intolerance in one infant (4%) on two occasions in PICU-1 for 2.5 h and in two infants (7%) on two occasions in PICU-2 for 19.5 h. Gastric residual mean (SD) volumes were 3.5 (5.4) mL kg-1 in PICU-1 and 16.9 (15.6) mL kg-1 in PICU-2. CONCLUSIONS: Peptide nutrient-energy dense feeding in infants admitted to the PICU is feasible, well tolerated and nutritional targets are met. However, with this study design, it is not possible to draw any conclusions regarding the benefit of PEF over standard PE feed in critically ill children and future work is required to clarify this further.

Weight improvement with the use of protein and energy enriched nutritional formula in infants with a prolonged PICU stay.

BACKGROUND: Reaching an optimal nutritional intake is challenging in critically ill infants. One possible way to minimise nutritional deficits is the use of protein and energy-enriched (PE)-formulas. We aimed to describe weight achievement and gastrointestinal symptoms in infants admitted to the paediatric intensive care unit (PICU) while receiving PE-formula for a prolonged period. METHODS: Records from infants admitted to a multidisciplinary PICU and using PE-formula were analysed retrospectively. Infants were eligible if they received PE-formula daily for at least 2 weeks. Weight achievement was determined as the difference between weight-for-age (WFA) Z-scores at the start and end of PE-formula use. Gastrointestinal symptoms, including gastric residual volume, constipation and vomiting, were evaluated as tolerance parameters. RESULTS: Seventy infants with a median [interquartile range (IQR)] age of 76 (30-182) days were eligible. The PICU duration was 50 (35-83) days during which they received PE-formula for 30 (21-54) days. Predominant admission diagnoses were post-cardiac surgery, respiratory and cardiac diagnosis. A significant mean (SD) WFA Z-score increase of 0.48 (1.10) (P < 0.001) and a median (IQR) weight gain of 5.80 (3.28-9.04) g kg-1 day-1 was observed. Multivariate regression showed that a lower WFA Z-score at start was associated with a higher WFA Z-score increase during PE-formula use (ß -0.35 (95% confidence interval =  -0.50 to -0.19); P < 0.001). The maximum 24-h gastric residual volume was 8.1 mL (IQR = 2.2-14.3) for each 1 kg in bodyweight. Three (4%) infants were treated for diarrhoea and three infants were treated for vomiting. CONCLUSIONS: The majority of infants with a prolonged PICU stay showed weight improvement when using PE-formula. PE-formula was well tolerated because gastrointestinal symptoms only occurred in few infants.

Plasma and red blood cell concentrations of zinc, copper, selenium and magnesium in the first week of paediatric critical illness.

BACKGROUND & AIMS: Critically ill children are at risk of micronutrient deficiencies, which might lead to poor clinical outcomes. However, the interpretation of micronutrient concentrations in plasma is complicated due to age-dependent and critical illness-dependent changes. Certain red blood cell (RBC) concentrations might reflect the overall body status more reliably than plasma levels in the presence of systemic inflammatory response. This study longitudinally examined micronutrient concentrations in both plasma and RBC in critically ill children. METHODS: This secondary analysis of the PEPaNIC RCT investigated the impact of early versus late initiation of parenteral macronutrient supplementation in critically ill children. All children received micronutrients when EN was insufficient (<80 % energy requirements). Blood samples were obtained on days 1, 3, 5 and 7 of Paediatric Intensive Care Unit (PICU) admission. Inductively coupled plasma mass spectrometry was used to measure zinc, selenium, and copper in plasma and selenium, copper, and magnesium in RBCs. Plasma magnesium was measured with colorimetric detection. Micronutrient concentrations were compared with age-specific reference values in healthy children and expressed using Z-scores. Changes in micronutrient concentrations over time were examined using the Friedman and post hoc Wilcoxon signed-rank tests. RESULTS: For 67 critically ill children, median (Q1; Q3) age 9.5 (5.5; 13.2) years, PIM3 score -2.3 (-3.1; -0.8), samples were available at various time points during their PICU stay. For 22 patients, longitudinal samples were available. On day 1, the median plasma Z-score for zinc was -5.2 (-5.2; -2.9), copper -1.6 (-2.9; -0.2), selenium -2.6 (-3.8; -1.0), magnesium -0.2 (-1.6; 1.3), and median RBC Z-score for copper was 0.5 (-0.1; 1.3), selenium -0.3 (-1.1; 0.7), magnesium 0.2 (-0.4; 1.3). In the longitudinal analysis, plasma zinc was significantly higher on day 5 (Z-score -3.2 (-4.6; -1.4)) than on day 1 (Z-score -5.2 (-5.2; -3.0), p = 0.032), and plasma magnesium was significantly higher on day 3 (Z-score 1.1 (-0.7; 4.0)) than on day 1 (Z-score -0.3 (-1.6; 0.5), p = 0.018). Plasma copper and selenium remained stable, and the RBC concentrations of all micronutrients remained stable during the first five days. CONCLUSIONS: Most patients had low plasma zinc, copper and selenium concentrations in the first week of their PICU stay, whereas they had normal to high RBC concentrations. More research is needed to examine the relationships between micronutrients and clinical outcome.

Early hypophosphatemia in critically ill children and the effect of parenteral nutrition: A secondary analysis of the PEPaNIC RCT.

BACKGROUND & AIMS: Hypophosphatemia during critical illness has been associated with adverse outcome. The reintroduction of enteral or parenteral nutrition, leading to refeeding hypophosphatemia (RFH), has been presented as potential risk factor. We investigated the occurrence of early RFH, its association with clinical outcome, and the impact of early parenteral nutrition (PN) on the development of early RFH in pediatric critical illness. METHODS: This is a secondary analysis of the PEPaNIC randomized controlled trial (N = 1440), which showed that withholding supplemental parenteral nutrition (PN) for 1 week (late-PN) in the pediatric intensive care unit (PICU) accelerated recovery and reduced new infections compared to early-PN (<24 h). Patients with renal replacement therapy or unavailable phosphate concentrations were excluded from this analysis. Early RFH was defined as serum/plasma phosphate <0.65 mmol/L and a drop of >0.16 mmol/L within 3 days of admission to the PICU. The association between baseline characteristics and early RFH, and the association of early RFH with clinical outcome were investigated using logistic and linear regression models, both uncorrected and corrected for possible confounders. To examine the impact of nutritional intake on phosphate concentrations, structural nested mean models with propensity score and censoring models were used. RESULTS: A total of 1247 patients were eligible (618 early-PN, 629 late-PN). Early RFH occurred in 40 patients (3%) in total, significantly more in the early-PN group (n = 31, within-group occurrence 5%) than in the late-PN-group (n = 9, within-group occurrence 1%, p < 0.001). Patients who were older (OR 1.14 (95% CI 1.08; 1.21) per year added, p < 0.001) and who had a higher Pediatric Risk of Mortality (PIM3) score had a higher risk of developing early RFH (OR 1.36 (95% CI 1.15; 1.59) per unit added, p < 0.001), whereas patients in the late-PN group had a lower risk of early RFH (OR 0.24 (95% CI 0.10; 0.49), p < 0.001). Early RFH was significantly associated with a 56% longer PICU stay (p = 0.003) and 42% longer hospital stay (p = 0.007), but not with new infections (OR 2.01 (95% CI 0.90; 4.30), p = 0.08) or length of mechanical ventilatory support (OR 1.05 (95% CI -3.92; 6.03), p = 0.68), when adjusted for possible confounders. Increase of parenteral nutrition intake (in % kcal of predicted resting energy expenditure) decreased phosphate concentrations (c = -0.002 (95% CI -0.002; -0.001). CONCLUSIONS: Early RFH occurred in 3% of critically ill children. Patients randomized to late-PN had a lower chance of developing early RFH, which may be explained by the more gradual build-up of nutrition. As early RFH might impact recovery, it is important to closely monitor phosphate concentrations in patients, especially of those at risk for early RFH.

Considerations for nutrition support in critically ill children with COVID-19 and paediatric inflammatory multisystem syndrome temporally associated with COVID-19.

There are reports of children COVID-19 or COVID-19 like symptoms with hyperinflammatory multisystem syndrome, ARDS, gastrointestinal and atypical Kawasaki disease presenting to PICU worldwide temporally associated with COVID-19, for which there are important nutrition support considerations. As a result, the European Society of Pediatric and Neonatal Intensive Care - Metabolism, Endocrine and Nutrition group (ESPNIC-MEN) and paediatric nutritionists working in PICUs are being consulted regarding nutrition management of critically ill children with COVID-19 or COVID-19 like symptoms. Therefore, the aim of this short report is to provide a summary of nutrition support recommendations for critically ill children with COVID-19. They are based on the ESPNIC-MEN section recommendations published in January 2020 and surviving sepsis recommendations from February 2020.

Achieving enteral nutrition during the acute phase in critically ill children: Associations with patient characteristics and clinical outcome.

BACKGROUND & AIMS: In the absence of methodologically sound randomized controlled trials (RCTs), current recommendations for timing and amount of enteral nutrition (EN) in critically ill children are based on observational studies. These studies have associated achievement of a higher EN intake in critically ill children with improved outcome. Inherent to the observational design of these underlying studies, thorough insight in possible confounding factors to correct for is essential. We evaluated the associations between EN intake and 1) patient and daily clinical characteristics and 2) clinical outcomes adjusted for these patient and clinical characteristics during the first week of critical illness with a multivariable mixed model. METHODS: This secondary analysis of the multicentre PEPaNIC RCT investigated a subgroup of critically ill children with daily prospectively recorded gastrointestinal symptoms and EN intake during the first week with multivariable analyses using two-part mixed effect models, including multiple testing corrections using Holm's method. These models combined a mixed-effects logistic regression for the dichotomous outcome EN versus no EN, and a linear mixed-effects model for the patients who received any EN intake. EN intake per patient was expressed as mean daily EN as % of predicted resting energy expenditure (% of EN/REE). Model 1 included 40 fixed effect baseline patient characteristics, and daily parameters of illness severity, feeding, medication and gastrointestinal symptoms. Model 2 included these patient and daily variables as well as clinical outcomes. RESULTS: Complete data were available for 690 children. EN was provided in 503 (73%) patients with a start after a median of 2 (IQR 2-3) days and a median % of EN/REE of 38.8 (IQR 14.1-79.5) over the first week. Multivariable mixed model analyses including all patients showed that admission after gastrointestinal surgery (-49%EN/REE; p = 0.002), gastric feeding (-31% EN/REE; p < 0.001), treatment with inotropic agents (-22%EN/REE; p = 0.026) and large gastric residual volume (-64%EN/REE; p < 0.001) were independently associated with a low mean EN intake. In univariable analysis, low mean EN intake was associated with new acquired infections, hypoglycaemia, duration of PICU and hospital stay and duration of mechanical ventilation. However, after adjustment for confounders, these associations were no longer present, except for low EN and hypoglycaemia (-39%EN/REE; p = 0.018). CONCLUSIONS: Several patient and clinical characteristics during the first week of critical illness were associated with EN intake. No independent associations were found between EN intake and clinical outcomes such as mortality, new acquired infection and duration of stay. These data emphasize the necessity of adequate multivariable adjustment in nutritional support research and the need for future RCTs investigating optimal EN intake.

Micronutrient status during paediatric critical illness: A scoping review.

BACKGROUND: No evidence based recommendations for micronutrient requirements during paediatric critical illness are available, other than those arising from recommended nutrient intakes (RNI) for healthy children and expert opinion. OBJECTIVES: The objective of this review is to examine the available evidence from micronutrient status in critically ill children considering studies which describe 1) micronutrient levels, 2) associations between micronutrient levels and clinical outcome, and 3) impact on clinical outcome with micronutrient supplementation during PICU admission. DESIGN: Scoping review. ELIGIBILITY CRITERIA: Any study which used a qualitative and quantitative design considering causes and consequences of micronutrient levels or micronutrient supplementation during paediatric critical illness. SOURCES OF EVIDENCE: NICE Healthcare Databases Advanced Search website (https://hdas.nice.org.uk/) was used as a tool for multiple searches, with a content analysis and charting of data extracted. RESULTS: 711 records were identified, 35 were included in the review. Studies evaluated serum micronutrient status was determined on admission day in majority of patients. A content analysis identified (n = 49) initial codes, (n = 14) sub-categories and (n = 5) overarching themes during critical illness, which were identified as: i) low levels of micronutrients, ii) causes of aberrant micronutrient levels, iii) associations between micronutrients levels and outcome, iv) supplementation of micronutrients. CONCLUSION: During critical illness, micronutrients should be provided in sufficient amounts to meet reference nutrient intakes for age. Although, there is insufficient data to recommend routine supplementations of micronutrients at higher doses during critical illness, the 'absence of evidence should not imply evidence of absence', and well designed prospective studies are urgently needed to elucidate paediatric micronutrient requirements during critical illness. The absence of reliable biomarkers make it challenging to determine whether low serum levels are reflective of a true deficiency or as a result redistribution, particularly during the acute phase of critical illness. As more children continue to survive a PICU admission, particularly those with complex diseases micronutrient supplementation research should also be inclusive of the recovery phase following critical illness.

Definitions, predictors and outcomes of feeding intolerance in critically ill children: A systematic review.

BACKGROUND & AIMS: Clinicians and researchers often use feeding intolerance (FI) as main cause for insufficient enteral nutrition (EN). However, there is no uniform definition for FI. A uniform definition is essential for future studies focusing on predictors and outcomes of FI and enteral nutrition. A systematic review was performed to investigate the definitions, prevalence, predictors and outcomes of FI in critically ill children. METHODS: The databases Medline, Embase, Cochrane CENTRAL, Web of Science were searched. Inclusion criteria were interventional, observational or case-control studies (>10 patients) in which a definition of FI was reported in critically ill children (0-21 years). RESULTS: FI was defined in 31 unique studies performed in 2973 critically ill children. FI was most commonly defined as presence of gastrointestinal (GI) symptoms and/or large gastric residual volume (GRV) (n = 21), followed by discontinuation of EN due to GI symptoms (n = 7) and inadequate delivery of EN (n = 3). Median prevalence of FI was 20.0% [IQR 7.4%-33.0%]. Large GRV, abdominal distention, diarrhoea and vomiting/emesis, were the predominantly reported GI symptoms to define FI. FI was associated with severity of illness, mortality and nosocomial infections. CONCLUSIONS: Feeding intolerance is inconsistently defined in the current literature, but appears to be a prevalent concern in critically ill children. FI is most frequently defined by the presence of GI symptoms. A standardized definition is needed for both clinical and research purpose to determine the consequences of FI in relation to short-term and long-term outcomes. The new proposed definition for FI entails the inability to achieve enteral nutrition target intakes in combination with the presence of GI symptoms indicating GI dysfunction. PROTOCOL REGISTRATION: PROSPERO registration number: CRD42018092967. Registered on 07 June 2018.

Effect of late versus early initiation of parenteral nutrition on weight deterioration during PICU stay: Secondary analysis of the PEPaNIC randomised controlled trial.

BACKGROUND & AIMS: Critically ill children are at increased risk of weight deterioration in the paediatric intensive care unit (PICU). Whether early initiation of parenteral nutrition (PN) prevents weight deterioration is unknown. The aims of this study were to assess the effect of withholding supplemental PN during the first week on weight Z-score change in PICU and to evaluate the association between weight Z-score change in the PICU and clinical outcomes. METHODS: This is a secondary analysis of the Paediatric Early versus Late Parenteral Nutrition in Intensive Care Unit (PEPaNIC) randomised controlled trial (N = 1440), which focused on the subgroup of patients with longitudinal weight Z-scores available on admission and on the last day in PICU. Patients were randomly allocated to initiation of supplemental PN after one week (Late-PN) or within 24 h (Early-PN) when enteral nutrition was insufficient. The effect of Late-PN versus Early-PN on the change in weight Z-score was investigated, adjusted for risk factors. Moreover, the association between weight Z-score change and clinical outcomes was explored, adjusted for risk factors. RESULTS: Longitudinal weight Z-scores were available for 470 patients. Enteral nutrition intake was equal in the Early-PN and Late-PN group. Less weight Z-score deterioration during PICU stay was associated with a lower risk of new infections (adjusted OR per Z-score increase 0.72 [0.55-0.96], p = 0.02), and with a higher likelihood of an earlier discharge from PICU alive (adjusted HR per Z-score increase 1.22 [1.10-1.37], p < 0.001). During PICU-stay, the change in weight Z-score did not differ among both groups (Late-PN median 0.00 [-0.34-0.12] vs Early-PN median -0.03 [-0.48-0.01], adjusted ß = 0.10 [-0.05-0.25], p = 0.18). CONCLUSIONS: Weight deterioration during the PICU stay was associated with worse clinical outcomes. Withholding supplemental PN during the first week did not aggravate weight Z-score deterioration during PICU stay. TRIAL REGISTRATION: clinicaltrials.gov NCT01536275.

Effects of a high-protein intake on metabolic targets for weight loss in children with obesity: a randomized trial.

OBJECTIVE: The objective of this research is to study effects of a 4-week high-protein (HP) diet on energy intake, resting energy expenditure (REE), protein turnover and body composition in children with obesity. METHODS: In this randomized placebo-controlled single-blind crossover study, children with obesity (n = 14; mean age: 10.1 years ± 1.2 standard deviation; body mass index-standard deviation score [BMI-SDS]: 2.8 ± 0.5) received an ad libitum HP (+50 g protein per day) or normal-protein (NP) diet for 4 weeks with a washout period of ≥2 weeks. Energy intake, REE, protein turnover, weight, BMI-SDS and body composition were measured. RESULTS: No differences were found in energy intake or REE between HP and NP. There was an increased urea production and phenylalanine hydroxylation after HP compared with NP (p < 0.05). There was an increased rise in fat-free mass after HP compared with NP (∆HP: 0.8 ± 0.8 kg vs. ∆NP: 0.1 ± 0.6 kg, p < 0.05). BMI and BMI-SDS increased during the study (BMI-SDS start: 2.8 ± 0.5, end: 2.9 ± 0.5, p < 0.05) without a difference between groups. CONCLUSIONS: A 4-week HP diet with ad libitum food intake did not affect energy intake and energy expenditure in children with obesity. BMI increased, although that could be partly explained by an increase in fat-free mass.

[Fatal asthma in childhod preventable by recognizing risk factors and presenting features]. / Fataal astma op de kinderleeftijd te voorkomen door herkenning van risicofactoren en presentatiewijze.

A 6-year-old child known with asthma died from an asthma attack after having had severe dyspnoea which lasted for 1 day. She had been having an average of 40 salbutamol 'puffs' each day for 1 month. For the preceding 8 months she had been having just over half this number as well as fluticasone. A 13-year-old girl died of an asthma attack. Three weeks previously she had been dyspnoeic and had taken salbutamol and prednisone as well as amoxicillin at a later stage. Each year between 8 and 10 children die of an acute exacerbation of asthma in the Netherlands. There are 2 different types of acute fatal asthma: a slow type (I) and a rapidly progressing type (II). In type I there is progressive obstruction of the airways due to oedema, mucous and spasm. Type II predominantly consists of bronchoconstriction. The main risk factors are previous hospital admission with asthma and inadequate maintenance medication. Effective maintenance therapywith the correct dosage ofinhalational corticosteroids administered correctly can probably stop the potentially fatal asthma type II from developing.

[Respiratory insufficiency caused by acute disseminated encephalomyelitis in a child]. / Respiratoire insufficiëntie bij een kind door acute disseminerende encefalomyelitis.

A 1.5-year-old boy presented with progressive ataxia and meningeal irritation after a period of general malaise and fever. He was eventually admitted to a paediatric intensive care unit for respiratory insufficiency. A diagnosis of acute disseminated encephalomyelitis (ADEM) with pontine involvement was made. The patient was mechanically ventilated and treated with immunoglobulins and corticosteroids, after which he recovered almost completely. ADEM is characterised by rapidly progressive demyelination of the central nervous system. The exact incidence and aetiology are unknown. The disorder is considered to be an autoimmune reaction, and current treatment is aimed at the suppression of this reaction. Despite the dramatic clinical and radiological presentation of ADEM, the prognosis is favourable in most cases.

Achieving energy goals at day 4 after admission in critically ill children; predictive for outcome?

BACKGROUND & AIMS: Adequate nutritional intake is essential during pediatric intensive care admission. We investigated whether achievement of energy intake goals at day 4 after admission and route of nutrition were associated with improved outcome. METHODS: Observational study using prospectively acquired data. Patients receiving enteral and/or parenteral nutrition were included. The energy intake target range at day 4 after admission was 90-110% of resting energy expenditure +10%. Acute malnutrition was defined as weight-for-age <-2 SD. Clinical outcome measures were length of stay, days on ventilator, duration of antibiotics and number of new infections. Data as median (min-max). RESULTS: Of 325 subjects (age 0.14 (0.0-18.0) year), 19% were acutely malnourished upon admission. Median 86% of energy goals were administered via the enteral route. With enteral energy intake, 7% of patients were fed within the target range, 50% were fed below and 43% were fed above the target range. In a subgroup (n = 223) the acutely malnourished proportion at discharge (26%) was not significantly different from that upon admission (22%). Whether the energy intake was below, within or above the target range did not affect changes in clinical outcome, nor did the route of nutrition. CONCLUSIONS: Acute malnutrition was highly prevalent upon admission and at discharge. With our nutritional protocol we achieved high rates of (enteral) energy intake. A high percentage of our population received enteral energy above the target energy range. However, there was no association between the amount of energy intake or route of nutrition and clinical outcome.

Megacystis-microcolon-intestinal hypoperistalsis syndrome: a case report.

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital disorder characterized by a dilated, non-obstructive urinary bladder and hypoperistalsis of the gastrointestinal tract, which is considered lethal. About 90 patients have been reported, predominantly female. We present the case of a female newborn with MMIHS in whom antenatal ultrasound was suggestive for the diagnosis, which was confirmed after delivery. Diagnostic features by antenatal ultrasound are described.