O
ccipital osteodiastasis (OOD) is an
uncommon form of birth injury associated with posterior fossa subdural
haemorrhage and laceration of cerebellum. The lesion consists of traumatic
separation of the cartilaginous joint between the squamous and lateral
portion of the occipital bone. Although birth asphyxia is the most common
cause of neonatal death in India, birth injuries are rarely reported. We
report a non-fatal case of ODD resulting from difficult breech extraction.
Case Report
A female baby weighing 2750 g was born by difficult
breech extraction to a primigravida mother. Baby needed positive pressure
ventilation for 60 seconds, and was transferred to neonatal intensive care
unit for post-resuscitation care. There was no clinical evidence of
hypoxic-ischemic encephalopathy. Tube feeding was started on second day of
life and with breastfeeds were initiated on 4th postnatal day. However,
she was observed to have poor sucking ability and breastfeeding sessions
were relatively long, lasting more than 20 minutes. Physical examination
revealed mild generalized hypotonia. Blood sugar, calcium and serum
electrolytes were within normal range. Sepsis screen and blood culture
were not suggestive of sepsis. Neurosonogram showed echogenic focus in the
occipital horn. X-ray skull lateral view showed separation of
squamous and lateral portion of occipital bone(Fig.1).
Cranial CT scan revealed large extra axial bleed in the right
temporo-parieto-occipetal region and both sides of tentorium cerebelli.
Blood was also seen in the posterior inter-hemispheric fissure (Fig.2).
Based on perinatal history and investigations a diagnosis of occipital
osteodiastasis was made. The infant was managed conservatively and started
accepting breastfeeds by 15th day of postnatal life. She was discharged on
20th day.
|
Fig. 1 X ray skull showing diastasis of
occipital bones. |
|
|
Fig. 2 CT Scan showing large extra-axial
bleed on the right side (parieto-occipital region). |
Discussion
Occipital bone is composed of four ossified parts
around the foramen magnum: a squamous part, two laterals condylar parts
and a basilar part. Squamous and lateral parts are separated by a strip of
posterior intraoccipital cartilage. Ossification of this synchondrosis is
completed during 4th or 5th year of life. The synchondrosis is vulnerable
to injury during delivery. A tear along this point with separation of the
occipital squama from the condylar parts is referred as occipital
osteodiastasis. The injury is attributed to excessive pressure exerted
over the subocciput resulting in a forward and upward displacement of
anterior margin of the occipital squama into the posterior cranial fossa.
This displacement may cause posterior fossa hemorrhage and other
intracranial complications.
The clinical syndrome consists of three phases. In the
first phase, no neurological signs are apparent for a period of several
hours to 4 days after birth. Second phase is heralded with signs referable
to increased intracranial pressure. Third phase has signs referable to
brain stem disturbances(3). Depending on severity, OOD can have variable
presentation. A fatal form associated with delivery has been described by
Hemsath(1). Two additional cases similar to the fatal type but of
postnatal origin (a three month old male and a two year old girl) have
also been described by Currarino(2). With the advances in obstetrical
techniques, this severe form of injury has become quite rare(2). Two more
cases described(2) belong to less severe form of OOD, one of this case is
associated with vertex presentation with an occipito-posterior position.
The present case was a less severe form of OOD, which
is a rare. Wigglesworth(4,5) suggested that a minor separation of the
occipital bone with little displacement would be without consequence. This
form of OOD is rarely reported in literature. The first patient was
reported by Pape, et al.(6) in 1979. Second case, reported by
Roche, et al.(7) in 1990, was a term infant born by vaginal
delivery to a 21 year old primipara. Later, two more similar cases were
reported(2). Wigglesworth and Husemeyer(5) indicated that the diagnosis in
stillborn babies may be facilitated by dissection of the sub-occipital
region with incision of the atlanto-occipital membrane prior to opening
the skull; also preliminary lateral X-rays help to demonstrate the
lesion.
Severe OOD and its complications have been associated
with poor outcome. Psychomotor development and neurological prognosis in
survivors is reported to be favorable. Prognosis with convexity subdural
haemorrhage is relatively good, from 50% to 90% of affected infants are
well on follow up, others are left with focal cerebral signs and
occasionally hydrocephalus. Close surveillance alone is required in the
absence of major neurological signs(8).
Acknowledgment
Dr V Devgan, Head, Department of Paediatrics and Dr R B
Mittal, Medical Superindentant: Hindu Rao Hospital, Delhi, for the
permission to share this case; Dr Vishnu Goyal for help to draft this
report.
Contributors: AJ made the diagnosis, was
responsible for literature search. SD drafted and prepared the manuscript.
SD critically evaluated the paper and gave the final approval. NS from
radiology department helped confirm the diagnosis.
Funding: None.
Competing interests: None stated.
References
1. Hemsath FA . Birth injury of the occipital bone with
a report of thirty-two cases. Am J Obstet Gynecol 1934; 27: 194-203.
2. Currarino G. Occipital Osteodiastasis: presentation
of four cases and review of the literature. Pediatr Radiol 2000; 30:
823-829.
3. Volpe JJ. Intracranial hemorrhage; Subdural. Primary
subarachnoid, Cerebellar, Intraventricular (term infant), and
Miscellaneous. In: Volpe JJ. Neurology of the Newborn, 5th ed.
Philadelphia: WB Saunders; 2009. p. 489-491.
4. Wigglesworth JS. Perinatal Pathology, 2nd edn.
Philadelphia: Saunders; 1996. p. 92-93.
5. Wigglesworth JS, Husemeyer RP. Intracranial birth
trauma in vaginal breech delivery: The continued importance of injury to
the occipital bone. Br J Obstet Gynecol 1977; 84: 684-691.
6. Pape KE, Wigglesworth JS. Hemorrhage, ischemia and
the perinatal brain. Philadelphia: JB Lippincott; 1979. p. 66-71.
7. Roche MC, Velez A, Garcia Sanchez P, Castroviejo P.
Occipital osteodiastasis a rare complication in cephalic delivery. Acta
Paediatr Scand 1990; 79: 380-382.
8. Perrin RG, Rutka JT, Drake JM, . Management and
outcomes of posterior fossa subdural hematomas in neonates. Neurosurgery
1997; 40: 1190-1199.