Paediatric Anaesthesia 1998 8: 245–247

Case reportDiagnosis and anaesthesia management ofhaemophilia during the neonatal period

CATHERINE BAUJARD, LOUISE GOUYET ANDISABELLE MURATDepartment of Anaesthesia, Hopital d’enfants Armand Trousseau, Paris, France

SummaryWe report here the case of a newborn infant admitted to ourhospital for moderate gastro-intestinal bleeding. Despite abnormalvalue for aPTT, diagnosis of moderate haemophilia A was onlymade preoperatively when surgery was deemed necessary to treatpyloric stenosis. Clinical circumstances of the diagnosis andanaesthesia management of haemophilia during the neonatal periodare discussed.

Keywords: pyloric stenosis; haemophilia; infant; coagulationdisorders

Introduction a haemophiliac. The pregnancy was normal and hewas delivered by caesarean section because of prior

Diagnosis of haemophilia is difficult during theuterine surgery. At birth the infant was normal andneonatal period owing to the paucity of usual clinicalno resuscitation was required. He received 5mg ofsigns as well as to the physiological abnormalities ofvitamin K intramuscularly but no local haematomahaemostasis in early life. Clinical circumstances ofwas observed. Bacteriological cultures were negative.diagnosis in neonates are a positive familial history,

On the fourth day of life, he presented withan unexplained spontaneous bleeding event or anmoderate gastro-intestinal bleeding after a feed.iatrogenic bleeding episode such as after surgery.Fibroscopy revealed a haemorrhagic gastritis withoutWe report here the circumstances of preoperativeoesophagitis. CMV and Campylobacter pylori serologydiagnosis of moderate haemophilia A in a neonatewere negative. Activated partial thrombo-scheduled for pylorotomy and anaesthesiaplastin time (aPTT) was 64 s (control, 33 s), pro-management.thrombin time (PT) was 85%, factor II level 120%,factor VII and X level 66%, factor V level 150% and

Case report fibrinogen 4 g·l−1. Platelet count and haemoglobinwere normal. The following days, postprandialThe patient was a male newborn, born at 37 weeksvomiting increased resulting in a weight lost of 210 g,gestation, with a birth weight of 3540g. The twobut without recurrence of gastrointestinal bleeding.sisters were healthy. The mother’s half brother wasAbdominal ultrasound showed an enlarged pyloruswithout muscular hypertrophy, attributed to mucosal

Correspondence to: Isabelle Murat, Department of Anaesthesia andoedema.Intensive Care, Hopital d’enfants Armand Trousseau, 26 avenue

du Dr Arnold Netter, 75571 Paris Cedex 12, France. Haemostasis investigation on the eighth day

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246 C. BAUJARD, L. GOUYET & I. MURAT

showed persistent abnormalities of aPTT (73/33) and tendency depends on the patient’s factor activity.Haemophilia is usually classified as severe (<2%),PT (60%). Factor II level was 57%, factor VII and X

level 43% and fibrinogen 1.4 g·l−1. Soluble complex moderate (2–5%) or mild (6–30%) according to factorVIII or IX clotting activity in plasma.and fibrin degradation products were negative, thus

eliminating disseminated intravascular coagulation. Basically, spontaneous bleeding events are rarelyobserved during the neonatal period and a diagnosisVitamin K deficiency was suspected and he received

10 mg of vitamin K intravenously. of haemophilia is generally made after the beginningof walking. In a retrospective study on 192As vomiting remained a major problem, a new

abdominal ultrasound was performed and pyloric haemophiliacs in 1966, Baenher & Strauss found thediagnosis of haemophilia was only made after astenosis was diagnosed. As preoperative laboratory

investigations confirmed aPTT persistent major bleeding event, and that less than 10% ofneonates and less than 40% of patients by one yearabnormalities, more complex investigation was

undertaken which revealed a deficit in factor VIII of age had actually been diagnosed (2). In a Swedishstudy on 140 haemophiliacs, diagnosis of(4%) defining moderate haemophilia A.

Surgery took place at ten days of life, after haemophilia was only made in 9% of children in theneonatal period while 20% of them had alreadyadministration of 30 IU·kg−1 of factor VIII, which

was given every eight h. Anaesthesia was induced exhibited an abnormal bleeding tendency during thefirst weeks of life (3). However, bleeding events arewith propofol (4 mg·kg−1) and suxamethonium

(2 mg·kg−1) for tracheal intubation, and maintained more frequently reported during the first year of lifeand diagnosis is made earlier (1). Indeed, in a recentwith isoflurane in a mixture of oxygen and nitrous

oxide (40/60). The lungs were mechanically study, Conway et al. (1) found that severely affectedneonates were diagnosed younger than one monthventilated. A single bolus of alfentanil (10 lg·kg−1)

was given before skin incision to ensure adequate significantly more often than in the 1966 study (2)(68.4% vs <10%) and patients not severely affectedanalgesia. At the end of surgery, a central venous

catheter was inserted in the right jugular vein, as were also diagnosed younger than one month (50%vs 2.5%). By the age of one year, all severely affectedsubstitution therapy with factor VIII was required

by the haematologists for ten days. children had been diagnosed (100% vs <40%) andpatients not severely affected were also diagnosedOn the sixth postoperative day, a septic syndrome

occurred and was attributed to central venous more frequently (72.2% vs 15%). Attention to familyhistory and recognition of early bleeding eventscatheter related Staphylococcus aureus septicaemia but

rapidly resolved after catheter removal and contributed to that earlier diagnosis.At birth, the most frequent manifestations areappropriate antibiotics. No haemorrhagic complica-

tion was observed during the postoperative period. extracranial bleeding (scalp haemorrhage, subgalealhaematomas, cephalohaematomas), and intracranialhaemorrhage (1,3–6). Other manifestations areDiscussiondescribed such as gastrointestinal bleeding (7),umbilical bleeding or haematuria. Some cases ofHaemophilia is a congenital bleeding disorder caused

by deficiency of coagulation factor VIII or IX, and haemorrhagic shock are reported on majorhaemophiliacs during the first days of life (8).accounts for approximately 95% of all inherited

disorders of coagulation. The diagnosis of Haemorrhage during surgery or after venouspuncture is another mode of presentation. Duringhaemophilia is difficult during the first year of life

owing to the paucity of usual clinical signs and to the first year of life, circumcision is the usualcircumstance of discovery (1). However, during thethe physiological abnormalities during this period of

life. The mode of inheritance is X-linked recessive. neonatal period, the majority of haemophiliacspresent only minimal bleeding after circumcision orTherefore, familial history is fundamental for the

diagnosis. However, sporadic haemophilia accounts no bleeding at all. When surgery takes place afterthirty days of life, moderate or major bleeding mayfor nearly 50% of the cases in recent series (1).

Haemophilia A is approximately four times more occur. This suggests that the newborn infant isrelatively protected compared to later in life. Therefrequent than haemophilia B. The severity of bleeding

1998 Blackwell Science Ltd, Paediatric Anaesthesia, 8, 245–247

HAEMOPHILIA DURING NEONATAL PERIOD 247

is no definite explanation for this fact, as it has been of precaution due to the complicated history of ourinfant. Intravenous DDAVP, that provokes a two- toproved that there is no transfer of factor VIII or IXfourfold increase in native factor VIII levels, may beacross the placenta (9).sufficient for patients with very mild haemophiliaDiagnosis of haemophilia is also difficult inundergoing minor surgical procedures.neonates owing to the physiological changes in

Complete haemostasis evaluation including factorsnormal values of some coagulation tests during theVIII and IX levels should therefore be undertakenfirst weeks of life (10,11) as well as the difficulties ofin a male neonate presenting with an unexplainedrepeated and extensive sampling. In the full termbleeding event and an increased activated partialnewborn, normal values of aPTT range between 28thromboplastin time. Reassessment of family historyand 55s, whereas the adult values are reachedis essential. Despite limitations of health carebetween two and nine months of age. In addition,resources, we believe that simple coagulation testsother haemostasis disorders more frequently(platelet count, PT and aPTT) should be performedobserved during this period such as vitamin Kbefore surgery in all neonates and infants even indeficiency or intravascular disseminated coagulationcases of minor surgery.may delay the diagnosis (8). Furthermore, in the

premature or full term infant, factor VIII level isequal to or greater than that measured in adults Referenceswhile factor IX level reaches the adult values between

1 Conway JH, Hilgartner MW. Initial presentations of pediatricsix and nine months of age. Thus haemophilia Ahemophiliacs. Arch Pediatr Adolesc Med 1994; 148: 589–594.

may be diagnosed during the neonatal period, while 2 Baehner RL, Strauss HS. Hemophilia in the first year of life. NEngl J Med 1966; 275: 524–528.haemophilia B is difficult to detect during the first

3 Ljung R, Petrini P, Nilsson IM. Diagnostic symptoms of severemonths of life.and moderate haemophilia A and B. Acta Paediatr Scand 1990;

Perioperative management requires a perfect 79: 196–200.4 Kletzel M, Miller Ch., Becton DL et al. Postdelivery headknowledge of the type and severity of haemophilia.

bleeding in hemophilic neonates AJDC 1989; 143: 1107–1110.Substitution therapy should be initiated before5 Rohyans JA, Miser AW, Miser JC. Subgaleal haemorrhage in

surgery and continued after surgery, the latter infants with hemophilia: report of two cases and review of thedepending on the type of surgery and the expected literature. Pediatrics, 1982; 70: 306–307.

6 Yoffe G, Buchanan GR. Intracranial hemorrhage in newbornduration of haemorrhagic risk. In general, it isand young infants with hemophilia. J Pediatr 1988; 113: 333–336.desirable to increase factor levels to normal before 7 Reish O, Nachum E, Naor N et al. Hemophilia B in a neonate:

and during surgery. Maximum level is quickly unusual early spontaneous gastrointestinal bleeding. Am JPerinatol 1994; 11: 192–193.reached after infusion, allowing surgery to be

8 Camboulives J, Barroyer D, Viard L et al. Coagulation intra-performed 15–30 min after the end of infusion.vasculaire disseminee masquant une hemophilie neonatale,

Because factor VIII half life is eight h, the infusion Ann Pediatr 1991; 38: 185–188.9 Oski FA, Naiman JL. Blood coagulation and its disorders inhas to be repeated three times a day. It is prudent

the new born. In: Oski FA, Naiman JL. eds, Hematologic problemsto confirm effectiveness of therapy by determiningin the newborn, Philadelphia: W.B. Saunders Co., 1982; 3rd edn,

postinfusion factor VIII levels preoperatively and 137–174.throughout the postoperative course. In the 10 Andrew M, Vegh P, Johnston M et al. Maturation of the

hemostatic system during childhod, Blood 1992; 80: 1998–2005.immediate postoperative period, levels should be11 Andrew M, Paes B, Johnston M. Development of the hemostaticgreater than 60–70%, while levels greater than 50% system in the neonate and young infant. Am J Pediatr Hematol

should be maintained until the end of the healing Oncol 1990; 12: 95–104.process. The ten days substitution programmerequired by our haematologist may reflect an excess Accepted 4 September 1997

1998 Blackwell Science Ltd, Paediatric Anaesthesia, 8, 245–247