Fractura craneal deprimida congénita... ¿actitud a seguir? / Congenital depressed skull fracture: the steps to follow?

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Multiple congenital skull fractures as a presentation of Ehlers-Danlos syndrome type VIIC.

We describe a newborn infant with multiple congenital skull fractures and intracranial hemorrhage. He also had multiple skin folds suggesting a connective tissue abnormality. Electron microscopy of the skin biopsy showed collagen abnormalities with a "hieroglyphic appearance." The analysis of the synthesis of collagen in the cultured dermal fibroblasts demonstrated an accumulation of procollagen I. Molecular analysis found a nonsense mutation Q225X in ADAMTS2 gene, which encodes procollagen I N-terminal proteinase. All these findings confirmed the diagnosis of Ehlers-Danlos syndrome type VIIC (MIM 225410). Family studies suggested a founder effect in Ashkenazi Jews originating from Belarus. Prenatal diagnosis in the subsequent pregnancy reassured the parents that the fetus was an unaffected carrier.

Vacuum extraction as a treatment modality of neonatal skull depression in a twin infant.

The management of depressed skull fractures in the newborn infant can be controversial. In this article, we report a case of twin pregnancy wherein one of the fetuses had depressed skull fractures that was not associated with any known trauma during the pregnancy or at delivery. This ping-pong skull depression was treated by elevation with an obstetrical vacuum extractor. No complications occurred. The possible etiologies and treatment modalities for neonatal depressed fractures, being conservative or operative, are discussed.

[Epidural haematoma in a newborn]. / Geburtstraumatisches epidurales Hämatom bei einem Neugeborenen.

Birth injuries occur occasionally but, in contrast, perinatally acquired epidural and intracerebral hematomas as well as neonatal skull fractures are extremely rare. The appropriate diagnostic and therapeutic modalities depend on the size and site of the injury as well as any accompanying diseases. We report the case of a neonatal epidural and intracerebral haematoma with skull fracture following secondary caesarean section. There was no evidence for trauma during pregnancy, birth injury or other possible causes of bleeding. The haematoma was decompressed surgically. One year after surgery the boy suffers from moderate neurological compromise and focal seizures.

[Spontaneous resolution of a congenital depressed skull fracture]. / Fractura hundimiento craneal congénita con resolución espontánea.

Congenital depressed skull fracture is rare and in most cases its etiology remains unclear. We present a female newborn infant with a congenital depressed skull fracture and no evidence of antepartum or intrapartum traumatism. The baby had normal neurological status and the depressed fracture healed spontaneously in a few weeks. Based on our experience of this case and a literature review, we conclude that congenital depressed skull fractures should be managed conservatively if the skull depression is less than 2cm, there is no previous trauma, no local edema or hematoma, and if the neonate shows normal neurological status.

Congenital skull fracture as a presentation of Menkes disease.

We report the rare presentation of Menkes disease with a congenital skull fracture, intracerebral bleeding, and seizures. The diagnosis was made at 3 months of age based on the characteristic features of the syndrome, by which time the child experienced uncontrollable seizures. Following progressive neurodegeneration, death occurred at 3 years of age. The prognosis in Menkes disease is dependent on early copper-histidine therapy. Effective treatment has led to children surviving into adulthood. Diagnosing the syndrome during the neonatal period is difficult. There are no published reports of congenital skull fracture as a presenting sign of Menkes disease. It is concluded that Menkes disease should be considered in any child who presents with congenital skull fracture as early diagnosis and treatment significantly improve the outcome.

Congenital depression of the fetal skull.

One hundred forty-seven cases of congenital skull depression are analyzed, including two presented by the authors, and a review of the literature follows. A management plan emphasizing a conservative approach is outlined.

Intrauterine depressed skull fracture. A report of two cases.

Two cases of intrauterine depressed fracture are presented. In each case, the mother had no history of abdominal trauma during pregnancy, the children were delivered normally without the use of forceps, but a round depression was present in the left frontal bone at time of delivery. CT scans demonstrated a depressed fracture of the left frontal bone and stagnation of cerebrospinal fluid around the fracture. Elevation of the depression was therefore made through a small incision along the rear edge of the frontal bone using a periosteal elevator. A review of intrauterine depressed skull fracture is made and the concept, mechanism, surgical indications and operative methods of this rare fracture are discussed.

Intrauterine skull depression and intracranial hemorrhage in a premature infant.

The authors describe a case of a premature infant born with a parietal skull depression who suffered an intraventricular hemorrhage and an ipsilateral intracerebral injury. At 21 months of life the infant's gross motor milestones were delayed and he had moderate spastic hemiplegia. Although skull depressions at birth are usually benign, they may be associated with long-term neurologic sequelae.

Congenital depression of the neonatal skull.

Congenital depression of the neonatal skull has had an incidence of 0.1% (1/10 000) in our newborn population during the past 8 years. These skull depressions have two pathogenetic types: deformation without fracture and fracture accompanied by depression. The cause of skull depression being the pressure exerted by the digits and fist of the newborn on his skull has not been previously reported. The treatment of choice for selected cases is nonsurgical elevation with an obstetric vacuum extractor. A CT scan should be performed prior to this treatment to rule out intracranial complications such as hemorrhage.

Spontaneous intrauterine depressed skull fractures.

A survey is made of neonatal skull depressions as a result of experience with ten neonates harboring noniatrogenic intrauterine skull fractures. Several mechanisms causing intrauterine skull depression are discussed. Diagnosis was made after delivery in all cases and was confirmed by skull radiography. Various modes of therapy are mentioned and a stepwise guideline is suggested for correction of the depression by applying CT as an adjuvant diagnostic tool.

Depressed skull fracture in the neonate. Report of three cases.

The authors describe three cases of neonatal depressed skull fracture that were elevated by means of an obstetrical vacuum extractor. In one case, a transparent breast pump shield replaced the metal vacuum extractor cup, permitting direct observation as the depression was elevated. Neonatal depressed skull fractures not associated with neurological signs may be safely elevated without surgery using the obstetrical vacuum extractor.