Malrotation in newborns following antenatal diagnosis of intra-abdominal cyst

Teele, Rita L.; Pease, P. W. B.; Rowley, R. Simon H.

doi:10.1007/s002470050450

Cited by 26 publications

(7 citation statements)

References 18 publications

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“…These benign tumors can show rapid growth resulting in displacement of the surrounding organs [8][9][10][11][12][13][14] . Although rare, spontaneous regression has been described [7,19] .…”

Section: Discussionmentioning

confidence: 99%

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Mesenchymal Hamartoma of the Liver: A Benign Tumor with Deceptive Prognosis in the Perinatal Period

Cornette¹,

Festen

Hoonaard

et al. 2009

Fetal Diagn Ther

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This article reports a case of perinatal mesenchymal hepatic hamartoma and reviews the literature on the subject. A fetus presented with polyhydramnios and a large multiloculated cystic abdominal mass at 33 weeks of gestation. The ultrasound appearance was most consistent with a mesenteric cyst. Prenatal drainage was considered, due to the size of the lesion. However, a conservative management was opted for. A female infant was born at 35 weeks by classical cesarean section. The immediate postnatal period was characterized by hemodynamic instability. Laparotomy revealed a pedunculated mesenchymal hamartoma of the liver, which could not completely be resected. The infant had an uneventful postoperative recovery and is doing well at 6 months of age. Hepatic mesenchymal hamartoma are rare benign tumors. Most cases are detected in early childhood. They usually present as a cystic rapidly growing abdominal mass. Prenatal diagnosis remains challenging. In children diagnosed in the perinatal period, the outcome seems worse and determined by the compressive effect of the mass.

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Section: Discussionmentioning

confidence: 99%

“…Seventeen cases, including ours, of HMH have been described in the English literature, in which an abdominal mass was observed prenatally by ultrasound [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] . Of these, 5 resulted in intrauterine demise or early neonatal mortality (29%) [8,10,12,13,15] .…”

Section: Discussionmentioning

confidence: 99%

Mesenchymal Hamartoma of the Liver: A Benign Tumor with Deceptive Prognosis in the Perinatal Period

Cornette¹,

Festen

Hoonaard

et al. 2009

Fetal Diagn Ther

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“…Gastrointestinal anomalies in the syndrome include short bowel, microcolon, persistently distended stomach, imperforate anus 4 and malrotation of the small bowel, colon and liver 6,7 . Our patient also had marked gastric dilatation.…”

Section: Discussionmentioning

confidence: 60%

Prune‐belly syndrome and pulmonary hypoplasia: A potential cause of death

Soylu

Kutlu

Sönmezgöz

et al. 2001

Pediatrics International

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Prune-belly syndrome (PBS) is a rare and complicated condition affecting mainly the abdominal wall and the genitourinary system. 1 In addition, respiratory, cardiovascular, gastrointestinal and orthopedic anomalies may accompany PBS. 2 Early recognition and treatment of PBS may prevent serious complications that may lead to fatalities. Herein, we report on a typical case of PBS in an infant, with a complex of genitourinary, respiratory, orthopedic and gastrointestinal anomalies, who died due to respiratory failure following a common respiratory infection. Case ReportA 4-month-old male infant presented to Turgut Ozal Medical Center, Clinic of Pediatrics, with fever, cough and respiratory difficulty. The complaints were noticed 8 days before admission and they gradually increased despite antibacterial treatment and oxygen support.He was delivered at full term and after an uncomplicated pregnancy. Due to the low socioeconomic status of the parents, the patient had no pre-or postnatal medical follow up. However, relative oligohydramnios was noted by an obstetrician in the delivery room.On physical examination, the child was febrile with a poor general condition. His pulse rate was 148 /min and his respiratory rate was 54 breaths/min with peripheral saturation of O 2 at 88% on 8 L/min of facemask oxygen. In addition to his dyspneic and grunting respiration, intercostal, supra-and substernal retractions were noted. Cracking rales were heard in both lungs. Kyphoscoliosis, abdominal distention and easily notable bowel loops secondary to a lack of abdominal wall muscles were determined. An enlarged liver, non-palpable testis and a right clubfoot deformity were determined. The body percentiles were within the normal ranges.Initial investigations were as follows: hemoglobin 9.9 g/dL, leukocyte count 27 200 /mm 3 (82% polymorphonuclear leukocytes, 12% band forms and 8% lymphocytes), erythrocyte sedimentation rate 60 mm/h, blood urea nitrogen (BUN) 10 mg/dL and serum creatinine 0.4 mg/dL. Liver function tests, serum total protein, albumin and electrolytes were within the normal ranges. Echocardiography and electrocardiography were also interpreted as normal. Although >10 5 /mm 3 Proteus vulgaris was grown in culture, microscopic examination of the urine was normal. Blood gas analysis upon admission revealed pH 7.32, PO 2 76 mmHg, PCO 2 45 mmHg and HCO 3 26 mmol/L.Plain chest X-ray showed thoracolumbal scoliosis, decreased right lung volume and ipsilateral displacement of the heart, an elevated right diaphragm, a compensatory increase of left lung volume, protruding liver secondary to an absence of abdominal wall muscles, dilated intestines and a consolidation from the middle lobe to the basal area on the right lung (Fig. 1). The computed chest tomography (CT) showed right lung hypoplasia, shifting of the trachea, mediastinal structures and the heart to the right hemithorax, a pneumonic parenchymal infiltration with air bronchograms on the hypoplastic lung and a compensatory expansion of the opposite lung ( Fig. 2a,b).Abdomina...

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“…Abnormal abdominal development, e.g. by early development of an intra-abdominal cyst in embryogenesis, is known to enhance the chance of developing IM [ 18 ]. Changes in intra-abdominal space caused by a CCVD might also increase the risk on IM development during embryogenesis [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

Congenital cardiovascular defects in children with intestinal malrotation

2007

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Intestinal malrotation (IM) and cardiovascular defects (CCVD) are both common congenital defects. We investigated the prevalence and types of CCVD in a 25-year IM population, and its association with post-IMoperative morbidity and mortality. Data on the type of CCVD, other congenital defects, syndromes, associations, post-IM-operative morbidity and mortality were retrospectively reviewed from the records of IM patients born between 1980 and 2005. Data were analyzed on (significant) differences between CCVD subgroups, and risk factors for both morbidity and mortality were calculated. Seventy-seven of 284 IM patients (27.1%) were diagnosed with a major or minor CCVD (37 and 40 patients, respectively). Syndromes and associations were more frequently diagnosed in patients with major than with a minor CCVD (67.6 vs. 40%, respectively). Post-IM-operative complications, although frequently observed (61%), did not differ between patients with major and minor CCVD. Physical CCVD signs before IM surgery increased post-IM-operative morbidity significantly (OR 4.0, 95% CI 1.4-11.0). Fifteen patients died (19.5%), seven due to cardiovascular cause. Mortality risk was increased by intestinal ischemia and post-IM-operative complications and by major CCVD after correction for age at weight at the time of IM operation. Congenital cardiovascular defects in children with intestinal malrotation are common, with high morbidity and mortality rates after IM operation. Elective IM surgery in young patients with CCVD should be performed in a centre with adequate paediatric cardiac care. Benefits of laparoscopic intervention need further study.

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Malrotation in newborns following antenatal diagnosis of intra-abdominal cyst

Cited by 26 publications

References 18 publications

Mesenchymal Hamartoma of the Liver: A Benign Tumor with Deceptive Prognosis in the Perinatal Period

Mesenchymal Hamartoma of the Liver: A Benign Tumor with Deceptive Prognosis in the Perinatal Period

Prune‐belly syndrome and pulmonary hypoplasia: A potential cause of death

Congenital cardiovascular defects in children with intestinal malrotation

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