Omphalocele at school age: What do parents report? A call for long-term follow-up of complex omphalocele patients.

OBJECTIVE: Many children with omphalocele experience morbidity in early life, which could affect long-term outcomes. We determined parent-reported outcomes in school-aged children treated for minor or giant omphalocele. STUDY DESIGN: We sent paper questionnaires to the parents of all children treated for omphalocele in 2000-2012. Giant omphalocele was defined as defect diameter ≥ 5 cm with liver protruding. Motor function (MABC-2 Checklist) was compared with Dutch reference data; cognition (PedsPCF), health status (PedsQL), quality of life (DUX-25) and behavior (Strengths and Difficulties Questionnaire; SDQ) were compared with those of controls (two per child) matched for age, gender and maternal education level. Possible predictors of cognition and behavior were evaluated using linear regression analyses. RESULTS: Of 54 eligible participants, 31 (57%) returned the questionnaires. MABC-2 Checklist scores were normal for 21/26 (81%) children. Cognition, health status, quality of life and behavior were similar to scores of matched controls. One quarter (26%) of children with omphalocele scored ≤ - 1 standard deviation on the PedsPCF, compared with 9% of matched controls (p = 0.07). Giant omphalocele and presence of multiple congenital anomalies (MCA) were most prominently associated with lower PedsPCF scores (giant omphalocele: ß -22.11 (95% CI: -43.65 to -0.57); MCA -23.58 (-40.02 to -7.13)), although not significantly after correction for multiple testing. CONCLUSIONS: Parent-reported outcomes of children with omphalocele at school age are reassuring. Children with an isolated, minor omphalocele do not need extensive long-term follow-up of daily functioning. Those with a giant omphalocele or MCA might be at risk for delayed cognitive functioning at school age; we recommend long-term follow-up to offer timely intervention.

[CLINICO-EXPERIMENTAL SUBSTANTIATION OF INTRAABDOMINAL PLASTY FOR UMBILICAL HERNIAS].

The impact of various methods of plasty, using net implants, on results of umbilical hernias treatment was studied in experimental and clinical investigation. The umbilical hernias plasty was performed in accordance to the IPOM (intraperitoneal on lay mesh) method, application of which have permitted to reduce a hospital stay of the patients as well as their period of social rehabilitation, and to guarantee the best cosmetic effect in comparison with such while making umbilical hernias plasty in accordance to a sub lay method.

[Application of C-QUR V-Patch system while performing preperitoneal alloplasty for small and middle umbilical hernias].

Own experience of application of a system C-QUR V-Patch, manufactured by "Atrium" (Maquet Getinge Group) firm while performing preperitoneal alloherniolasty for small and middle umbilical hernias, was summarized. In this implant polypropylene mesh, covered by layer of preparation of omega-3 class of polyunsaturated fatty acids, capable of bioabsorption, joined with a newest technologies of a space memory. Its application guarantees lesser traumaticity and duration of operation, absence of peritoneal damage, small sizes of operation wound, what permits to accelerate significantly the patients' working ability restoration, as well as to achieve a quality of their life improvement.

Abordagem global de uma intervenção fisioterapêutica na onfalocele gigante / Global approach to a physiotherapy intervention in giant omphalocele

Descrever os efeitos da utilização de recursos instrumentais e cinesioterapêuticos em parâmetros cardiorrespiratórios, espirométricos e na qualidade de vida de uma adolescente com diagnóstico de Onfalocele Gigante (OG), doença pouco abordada na literatura em relação à intervenção da fisioterapia respiratória e motora. Paciente de 16 anos, com diagnóstico de OG, realizou acompanhamento fisioterapêutico, cuja conduta envolveu o uso do Threshold®, Respiron®, fortalecimento e alongamento da musculatura global e de grupos musculares específicos, para a melhora do padrão postural. Os dados de função pulmonar referente à saturação de oxigênio, freqüências respiratória e cardíaca, pressão inspiratória e expiratória máximas, teste de caminhada de seis minutos e parâmetros espirométricos foram as variáveis quantitativas de efeito consideradas antes e após o período de cinco meses de tratamento. Houve melhora de todas as variáveis quantitativas de função pulmonar em relação aos valores basais, bem como melhora da qualidade de vida e da sensação de dispnéia referidas pela adolescente. O uso de recursos fisioterapêuticos instrumentais para fortalecer a musculatura inspiratória, melhorar a ventilação, diminuir a dispnéia e aumentar a tolerância ao exercício, além de uma abordagem postural para desenvolver equilíbrio da biomecânica músculo-esquelética, podem ser uma alternativa a ser utilizada como conduta no tratamento de pacientes com OG.

To describe the effects of using specific physiotherapeutic resources and kinesiotherapy on cardiorespiratory and spirometric parameters, and on the quality of life of an adolescent diagnosed with Giant Omphalocele (GO), a disease little-known in the literature as regards respiratory and motor intervention. In a 16-year-old patient diagnosed with GO, the physiotherapy was monitored involving the use of Threshold® and Respiron®, strengthening and stretching the musculature overall as well as in specific groups to improve the postural alignment. Data for pulmonary function regarding blood oxygenation, breath and heart rate, maximum inhalation and exhalation pressures, six-minute walk tests, and spirometric parameters were the effective quantitative variables considered before and after the five-month treatment period. There was a general improvement in all the quantitative variables for pulmonary function compared to baseline values, as well as an improvement in the quality of life and of the feelings of dyspnea mentioned by the adolescent. The use of specific physiotherapeutic resources to fortify the natural inhalational musculature, to improve ventilation, to reduce dyspnea, and to increase the tolerance to exercise, aside from a postural approach to developing bio-mechanical musculoskeletal equilibrium, can be an alternative to be used as a path of treatment for patients with GO.

Cardiopulmonary performances in young children and adolescents born with large abdominal wall defects.

BACKGROUND/PURPOSE: As long as the survival rate of patients with abdominal wall defects (AWD) increases, information about long-term follow-up is becoming necessary. Even though quality of life in these patients, in absence of associated anomalies, appears to be unaffected, respiratory impairment soon after birth has been documented; therefore, participation in sports rarely is addressed. METHODS: Eighteen patients, ranging in age from 7 to 18 years, operated on at birth for large abdominal wall defects (> 4 cm for gastroschisis; >6 cm for omphalocele) were asked to come for a stress test on a treadmill, with measurements of time of exercise (TE), maximal oxygen consumption (VO2 max) and continuous recording of vital parameters. Respiratory function also was assessed by Forced Vital Capacity (FVC). RESULTS: Ergometric data were compared with those of a normal pediatric population. All patients were able to perform the stress test with no cardiovascular abnormalities detected at rest or on exertion. Maximum heart rate was reached after a significantly shorter TE, and VO2 max was significantly reduced when comparing normal subjects with AWD subjects and AWD subjects in sports with those sedentary. FVC was only slightly reduced in AWD patients without reaching statistical significance. CONCLUSIONS: These findings indicate that patients operated on for AWD at birth exhibit a normal cardiorespiratory function; decreased TE and VO2 max are likely to reflect a lack of physical activity with a lower degree of fitness. Therefore, no limitations to motor performances should exist for these patients. Well-being may be greatly improved by regular exercise.