congenital cutaneous histiocytosis. case report

1
Conclusions Many of folk remedies are harmless and some of them are dangerous for mother safety. One conclusion drawn from our study is that nurses and midwives need to be aware of women's knowledge and experiences with abortions in order to support and empower women who seek a healthy abortion. doi:10.1016/j.earlhumdev.2008.09.383 Abstract UENPS.368 Congenital cutaneous histiocytosis. Case report Susana Soares, Catarina Magalhães, Maria José Vale, Alice Freitas, Clara Paz Dias Centro Hospitalar do Alto Ave, Guimarães, Neonatology Unit, Portugal Background and aim The clinical spectrum of Langerhans Cell Histiocytosis comprises a group of rare disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells and mature eosinophils. A congenital self- healing rarer form named Hashimoto-Pritzker has been described. Materials and methods Case report. Results At birth, after a full-term uncomplicated pregnancy, an otherwise healthy newborn boy presented multiple red-bluish papulonodules localized over the groin and limbs, associated with scattered purpuric/petechial lesions. There were no other abnormal physical findings. The 20-year-old mother had no known pathologies, and her serologic status revealed: negative hepatitis B surface antigen, negative human immunodeficiency virus, non-reactive venereal disease research laboratory, rubella immune and toxoplasmosis nonimmune. Fetal tachicardia led to a cesarian delivery, with Apgar scores 9 and 10 at 1 and 5 min. Initial laboratory studies were normal. Culture from lesion was negative, as well as blood culture. The immunohistochemical exam of skin biopsy revelead the diagnosis: Langerhans cell histiocytosis. Complementary studies excluded other systems involvement. Conclusions The differential diagnosis of skin lesions in neonates is broad. The biopsy of the skin is the gold standard for establishing the diagnosis. Althought this is a rare condition, its identification is essential allowing specific care and follow-up. doi:10.1016/j.earlhumdev.2008.09.384 Abstract UENPS.369 Superior vena cava thrombosis and hydrocephalus in a newborn Joana Rebelo, Maria Céu Ribeiro, Gustavo Rocha, Alexandra Adams, Fátima Clemente, Angelina Martins, Hercília Guimarães Hospital São João, Porto, Portugal Background and aim Symptomatic neonatal thromboembolic disease is a rare entity, especially when located at the superior vena cava (SVC). It is most often related to venous access lines and it is rarely associated with hydrocephalus. The authors present the case of a newborn with hydrocephalus and intraventricular haemorrhage following SVC thrombosis. Materials and methods Chart review of a newborn admitted in the NICU of Hospital São João. Results Female, 36 weeks gestational age. Pregnancy complicated with exposure to human immunodeficiency virus and with prenatal diagnosis of gastro- schisis at 12 weeks gestational age. Elective cesarean delivery at a tertiary hospital. APGAR 5/9. She was submitted to surgical correction of gastroschisis and central venous access line placement in the right internal jugular vein on the first day of life. Thirty-six hours later she developed important and progressive swelling of the face, head, upper limbs and superior aspect of the thorax, with cyanosis. SVC thrombosis was suspected, and central venous access line removed. Echocardiogram and angio-computed tomography confirmed the diagnosis, and unfractionated heparin was started. Progressive neurological deterioration was noticed, with hypertonic limbs; intracerebral and intra- ventricular haemorrhage was revealed by imaging studies, with further progression to active tetra-ventricular hydrocephalus. No seizures were observed. A ventriculo-peritoneal shunt was placed, and clinical status was complicated with long-lasting Staphylococcus epidermidis meningitis. On the 63rd day of life sequelae lesions of previous thrombotic event were revealed by cerebral angio-magnetic resonance imaging (angio-MRI); the thoracic angio-MRI showed an effective collateral venous system and partial SVC permeability. Progressive neurological improvement was noticed after clinical stabilization; nevertheless, she presents, at 5 months age, an important staturoponderal and neurodevelopment delay. Conclusions Neonatal SVC thrombosis has usually bad prognosis. The authors aim to point out and underline the fact that this rare situation, as a possible complication of central venous access placement, can lead to important neurologic sequelae, such as hydrocephalus. doi:10.1016/j.earlhumdev.2008.09.385 Abstract UENPS.370 Audit of premature infants and critically ill neonates requiring ex-utero transfer by the first Macedonian Neonatal Emergency Transport Service (NETS) A retrospective descriptive study Sofijanova Aspazija, Aleksandar Sajkovski, Fidanovski Dushko, Hristovski Antonio, Koic Ljiljana University's Pediatric Hospital, Department for Neonatal Intensive Care, Skopje, Macedonia Background and aim Studies have shown that mortality is lower for premature infants and critically ill neonates suffering different diseases, born in tertiary centres compared to those born in non-tertiary. The outcome for high-risk infants transferred in-utero is reported as better than the outcome of infants transferred after birth. The first Macedonian NETS is working since 2002 in the frame of the NICU in the Pediatric Hospital in Skopje. Materials and methods NETS provides transport with Ginevri baby shuttle and transport vehicle for the most premature and critically ill neonates in Macedonia, Kosovo and Albania. Every time NETS is contacted by a clinician regarding transfer the information is entered into the NETS database. We audited the NETS records of all premature infants (2735 G.A.) and critically ill neonates in a non- tertiary centers requiring the mini-hospital transfer at the NICU from January 2002 until July 2008. Results 270 infants were transferred during a 6 year period. The median response and retrieval times were 1 h 50 min and 3 h 50 min respectively, while transferring infant with cardiopathy for cardio surgery in Bulgaria. The overall survival was 60%. Newborn survival increased from 29% at 27 G.A. to 72% at 32 G.A. Survival rate at 3235 G.A. was 43%. Of the 79 babies known to Abstracts S148

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Page 1: Congenital cutaneous histiocytosis. Case report

Conclusions

Many of folk remedies are harmless and some of them are dangerous formother safety. One conclusion drawn from our study is that nurses andmidwives need to be aware of women's knowledge and experiences withabortions inorder to support andempowerwomenwhoseekahealthyabortion.

doi:10.1016/j.earlhumdev.2008.09.383

Abstract UENPS.368Congenital cutaneous histiocytosis. Case report

Susana Soares⁎, Catarina Magalhães, Maria José Vale,Alice Freitas, Clara Paz DiasCentro Hospitalar do Alto Ave, Guimarães, Neonatology Unit, Portugal

Background and aim

The clinical spectrum of Langerhans Cell Histiocytosis comprises a groupof rare disorders characterized by the proliferation of specialized bonemarrow-derived Langerhans cells and mature eosinophils. A congenital self-healing rarer form named Hashimoto-Pritzker has been described.

Materials and methods

Case report.

Results

At birth, after a full-term uncomplicated pregnancy, an otherwise healthynewborn boy presented multiple red-bluish papulonodules localized overthe groin and limbs, associated with scattered purpuric/petechial lesions.There were no other abnormal physical findings. The 20-year-old mother hadno known pathologies, and her serologic status revealed: negative hepatitis Bsurface antigen, negative human immunodeficiency virus, non-reactivevenereal disease research laboratory, rubella immune and toxoplasmosisnonimmune. Fetal tachicardia led to a cesarian delivery, with Apgar scores 9and 10 at 1 and 5 min. Initial laboratory studies were normal. Culture fromlesion was negative, as well as blood culture. The immunohistochemicalexam of skin biopsy revelead the diagnosis: Langerhans cell histiocytosis.Complementary studies excluded other systems involvement.

Conclusions

The differential diagnosis of skin lesions in neonates is broad. The biopsy ofthe skin is the gold standard for establishing the diagnosis. Althought this is arare condition, its identification is essential allowing specific care and follow-up.

doi:10.1016/j.earlhumdev.2008.09.384

Abstract UENPS.369Superior vena cava thrombosis and hydrocephalus in a newborn

Joana Rebelo, Maria Céu Ribeiro, Gustavo Rocha⁎, Alexandra Adams,Fátima Clemente, Angelina Martins, Hercília GuimarãesHospital São João, Porto, Portugal

Background and aim

Symptomatic neonatal thromboembolic disease is a rare entity, especiallywhen located at the superior vena cava (SVC). It is most often related tovenous access lines and it is rarely associated with hydrocephalus.

The authors present the case of a newborn with hydrocephalus andintraventricular haemorrhage following SVC thrombosis.

Materials and methods

Chart review of a newborn admitted in the NICU of Hospital São João.

Results

Female, 36 weeks gestational age. Pregnancy complicated with exposureto human immunodeficiency virus and with prenatal diagnosis of gastro-schisis at 12 weeks gestational age. Elective cesarean delivery at a tertiaryhospital. APGAR 5/9.

She was submitted to surgical correction of gastroschisis and centralvenous access line placement in the right internal jugular vein on the firstday of life. Thirty-six hours later she developed important and progressiveswelling of the face, head, upper limbs and superior aspect of the thorax,with cyanosis. SVC thrombosis was suspected, and central venous access lineremoved. Echocardiogram and angio-computed tomography confirmed thediagnosis, and unfractionated heparin was started. Progressive neurologicaldeterioration was noticed, with hypertonic limbs; intracerebral and intra-ventricular haemorrhage was revealed by imaging studies, with furtherprogression to active tetra-ventricular hydrocephalus. No seizures wereobserved. A ventriculo-peritoneal shunt was placed, and clinical status wascomplicated with long-lasting Staphylococcus epidermidis meningitis. Onthe 63rd day of life sequelae lesions of previous thrombotic event wererevealed by cerebral angio-magnetic resonance imaging (angio-MRI); thethoracic angio-MRI showed an effective collateral venous system and partialSVC permeability. Progressive neurological improvement was noticed afterclinical stabilization; nevertheless, she presents, at 5 months age, animportant staturoponderal and neurodevelopment delay.

Conclusions

Neonatal SVC thrombosis has usually bad prognosis. The authors aim topoint out and underline the fact that this rare situation, as a possiblecomplication of central venous access placement, can lead to importantneurologic sequelae, such as hydrocephalus.

doi:10.1016/j.earlhumdev.2008.09.385

Abstract UENPS.370Audit of premature infants and critically ill neonates requiring ex-uterotransfer by the first Macedonian Neonatal Emergency Transport Service(NETS) — A retrospective descriptive study

Sofijanova Aspazija⁎, Aleksandar Sajkovski, Fidanovski Dushko,Hristovski Antonio, Koic LjiljanaUniversity's Pediatric Hospital, Department for Neonatal Intensive Care, Skopje,Macedonia

Background and aim

Studies have shown that mortality is lower for premature infants andcritically ill neonates suffering different diseases, born in tertiary centrescompared to those born in non-tertiary. The outcome for high-risk infantstransferred in-utero is reported as better than the outcome of infantstransferred after birth. The first Macedonian NETS is working since 2002 inthe frame of the NICU in the Pediatric Hospital in Skopje.

Materials and methods

NETS provides transport with Ginevri baby shuttle and transport vehiclefor the most premature and critically ill neonates in Macedonia, Kosovo andAlbania. Every time NETS is contacted by a clinician regarding transfer theinformation is entered into the NETS database. We audited the NETS recordsof all premature infants (27–35 G.A.) and critically ill neonates in a non-tertiary centers requiring the mini-hospital transfer at the NICU from January2002 until July 2008.

Results

270 infants were transferred during a 6 year period. The median responseand retrieval times were 1 h 50 min and 3 h 50 min respectively, whiletransferring infant with cardiopathy for cardio surgery in Bulgaria. Theoverall survival was 60%. Newborn survival increased from 29% at 27 G.A. to72% at 32 G.A. Survival rate at 32–35 G.A. was 43%. Of the 79 babies known to

AbstractsS148