scholarly journals Associated anomalies among infants with oral clefts at birth and during a 1-year follow-up

2011 ◽  
Vol 155 (7) ◽  
pp. 1588-1596 ◽  
Author(s):  
Monica Rittler ◽  
Viviana Cosentino ◽  
Jorge S. López-Camelo ◽  
Jeffrey C. Murray ◽  
George Wehby ◽  
...  
PEDIATRICS ◽  
1955 ◽  
Vol 15 (6) ◽  
pp. 761-767
Author(s):  
Harriette Clark ◽  
Roland B. Scott ◽  
John B. Johnson

Two cases of agenesis of the lung, both with anomalies of the urinary tract, have been presented. In 1 patient the diagnosis was made at post-mortem examination at the age of 5 weeks. Associated anomalies in this patient included defect of the interatrial septum, anomalous drainage of the pulmonary vein into the right atrium, and congenital bilateral ureterovesicle obstruction with dilatation of the ureters and kidney pelves. In the second patient, the diagnosis was made at the age of 3 years. A follow-up study 12 years later substantiated the diagnosis and revealed pelvic location of fused kidneys with short ureters bilaterally. In the latter case, the lung anomaly has not resulted in any dysfunction of the heart or pulmonary circulation. There has been some decrease in ventilatory function but not sufficiently severe to result in respiratory symptoms. This patient is living and well at the time of this report.


2014 ◽  
Vol 36 (5-6) ◽  
Author(s):  
R. Angotti ◽  
A.L. Bulotta ◽  
F. Ferrara ◽  
F. Molinaro ◽  
E. Cerchia ◽  
...  

Objective. Over the past decade, multiple factors have changed the pattern of neonatal surgical emergencies. An increase in prenatal screenings and the development of neonatal tertiary care centres have changed the clinical approach to these kids. Materials and methods. Between 1995 to 2011 were retrospectively reviewed 34 patients with diagnosis of uncommon rare neonatal surgical emergencies at our institute. We analyzed: sex, gestational age, weight at birth, primary pathology, prenatal diagnosis, associated anomalies, age and weight at surgery, clinical presentation, start of oral feeding and hospitalization. The follow-up was performed at 6,12, 24 and 36 months. Results. There were 21 male and 13 female. The gestational age ranged between 28 and 36 weeks. The weight at birth ranged between 700 and 1400 grams. Oral feeding was started between 4th and 10th postoperative day. The average hospitalization was about 70.47 days. To date, all patients have finished the followup. They are healthy. Conclusion. The outcome of the patients with uncommon surgical emergencies is different based on the etiology. Overall survival is generally good but is influenced by the associated anomalies.


2006 ◽  
Vol 43 (1) ◽  
pp. 1-6 ◽  
Author(s):  
Diego F. Wyszynski ◽  
Andrea Sárközi ◽  
Andrew E. Czeizel

Objective To discuss methodological factors that account for the wide variation in the reported prevalence rates of anomalies associated with oral clefts. Conclusions The published prevalences of associated anomalies vary considerably because of the following: (1) differences in case definition and inclusion/exclusion criteria; (2) length of time after birth that cases are examined; (3) variability of clinical expression of associated anomalies; (4) knowledge and technology available to produce syndrome delineation; (5) selection of patients, sources of ascertainment, and sample size; and (6) true population differences and changes in frequency over time.


2021 ◽  
Author(s):  
Zhongshan Gou ◽  
Xinxin Yan ◽  
Baojuan Sun ◽  
Jie Zhang ◽  
Hongmei Liu ◽  
...  

Aim: To investigate the types, associated anomalies and postnatal outcomes of fetal hepatic venous system (HVS) variants by ultrasound in China.Material and methods: A large-scale and prospective investigation of HVS variants for low-risk singleton pregnant women was performed in three academic tertiary referral care centers in China. Ultrasound imaging wasused for the identification and follow-up of anatomical variants. Follow-up was conducted once every four weeks prenatally and every two months postnatally, mainly concerned on the adverse events that may appear.Results: There were 20848 cases with anatomical variants of fetal HVS identified from 46179 candidates during the study period. Following the anatomical position of variants occurring, four main divisions were present: main portal vein variants (17.9%), intrahepatic portal vein variants (21.30%), intrahepatic persistent right umbilical vein (0.27%) and hepatic vein variants (5.67%). In the fetal period, the pregnancy of all cases was normally continued, except that the pregnancy of two cases, which were associated with multiple anomalies and were terminated by their parents. After birth, approximately 99.47% of the cases with isolated variants orbeing associated no clinic significant anomalies were normally alive. Approximately 0.50% cases were associated with simple ventricular septum defect or tetralogy of Fallot and further treatment was needed.Conclusion: The anatomical variants of fetal HVS may appear as numerical, morphological or positional variants of MPV, intrahepatic PV branches, intrahepatic PRUV and HVs. The majority of cases are isolated or their associated anomalies are not clinically significant and have normal lifeafter birth.


2019 ◽  
Vol 11 (1) ◽  
pp. 19-24
Author(s):  
Aleksandra Matić ◽  
Milan Matić ◽  
Sonja Prćić

Abstract Introduction. Superficial infantile hemangiomas (IH) are rarely localized in the genital region. Case Report. We present 8 infants with IHs in the genital region (3 boys and 5 girls). Our patients had only superficial IHs, their internal organs were not affected and there were no associated anomalies. In the boys, IHs were localized on the scrotum and the foreskin in 2 cases and 1 case, respectively. In the girls, IHs were on the labia majora and near the vaginal introitus. None of the IHs showed any IH-related complications. No therapy for IHs was administered in any of the presented infants, either because IHs were small and non-aggressive, or because their parents rejected the proposed therapy. Conclusion. Genital IHs have increased tendency for ulceration, so thorough examination and follow-up are of outmost importance. If therapy is indicated, oral propranolol is the treatment of choice for genital IHs.


PEDIATRICS ◽  
1973 ◽  
Vol 52 (4) ◽  
pp. 609-612
Author(s):  
Barbara Jones ◽  
Havelock Thompson

Aase and Smith1 reported male siblings with congenital hypoplastic anemia and triphalangeal thumbs, and distinguished their disorder as an entity separate from Fanconi's pancytopenia and radial aplasia-thrombocytopenia. Recently, Murphy and Lubin2 described a third patient, also a male, with a similar thumb anomaly, aregenerative anemia, and several associated anomalies not present in the siblings described in the original report. This report describes a fourth male patient with this syndrome and adds the data of an eight-year follow-up with hematologic response to prednisone therapy. CASE REPORT R.P. was initially evaluated at West Virginia University Hospital in 1964 at 10 years of age; he had a history of chronic anemia.


Author(s):  
Sabrina A. Mhapankar ◽  
Annabelle S. Vadukkut ◽  
Utkarsha S. Patil ◽  
Sushil Kumar

Congenital encephalocele is a neural tube defect which is caused by an embryonic development abnormality. It is characterized by a sac-like protrusion of the brain, meninges and other intracranial structures through the skull. 75% of encephalocoeles are occipital. Pre-natal screening is very essential for timely recognition of the condition. At the same time, proper intake of folic acid in the first few weeks of pregnancy may reduce the occurrence of this form of NTD. The prognosis is variable depending on the presence of associated anomalies and presence of microcephaly (carries a much poorer prognosis). A favorable surgical outcome generally follows an accurate strategy taking into account individual features of the lesion. A Caesarian delivery may be considered to allow for less traumatic birth for the fetal head. Here we present a case of a foetus with posterior meningoencephalocoele diagnosed antenatally at 32 weeks of pregnancy. Patient was delivered by elective Cesearian section. The encephalocoele was resected and the defect was closed primarily. The baby was well on the regular follow-up at the neurosurgery outpatient department.


2018 ◽  
Vol 75 (3) ◽  
pp. 290-296
Author(s):  
Djordje Kravljanac ◽  
Radoje Simic ◽  
Ivan Milovic

Background/Aim. A great number of syndactyly release techniques have been described over last two centuries. The aim of our study is outcome assessment of congenital syndactyly surgery using temporary tissue expansion of the dorsal hand and local flaps, without skin grafts. Methods. This study included children with congenital hand syndactyly treated in period from 2009?2015 by operative technique with temporary tissue expansion of the dorsal hand skin and local flaps, without skin grafting. In all cases surgery was performed under general anesthesia. According to Weber?s descriptive method, the functional outcome at the end of the follow-up period was categorized as good, fair or bad. All patients were evaluated for associated anomalies. Results. A total of 26 children (20 males, 6 females), aged from 6 months to 6 years (average age of 23 months), were operated by previously described technique. There were 20 patients with complete syndactyly and 6 with incomplete, mostly involving the third web. Associated anomalies were diagnosed in 9 patients. The follow-up period ranged from 1 to 5 years with average duration of 2.6 years. The functional results were good in 20 patients, fair in 5 and bad in 1 patient. Conclusion. Surgical procedure with temporary tissue expansion of the dorsal hand skin and local flaps, without skin grafting is effective method of congenital syndactyly treatment in children with good functional and aesthetic results. The advantages of this technique are the reduction of surgery duration and avoiding certain complications, such as web hair growth, hyperpigmentation and hypertrophic scars.


2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Adeline Du Mortier ◽  
Pierre-Louis Docquier

We report a case of traumatic humeral neck fracture occurring in a patient with osteopoikilosis after a motorcycle accident. The radiograph revealed the fracture but also multiple bone lesions. A few years before, the patient had been operated for a maldiagnosed chondrosarcoma of the humeral head. Osteopoikilosis is a rare benign hereditary bone disease, whose mode of inheritance is autosomal dominant. It is usually asymptomatic and discovered incidentally on radiograph that shows the presence of multiple osteoblastic lesions. It can mimic other bone pathologies, in particular osteoblastic metastases. Osteopoikilosis is a diagnosis that should be kept in mind to avoid misdiagnosis, particularly with regard to cancer metastasis. This disorder does not require any treatment and complications are rare. However, there may be associated anomalies that require follow-up.


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