Childs Nerv Syst DOI 10.1007/s00381-015-2808-1

ORIGINAL PAPER

Decompressive craniectomy for infants: a case series of five patients Muhammad Riyaz 1 & Muhammad Waqas 1 & Badar Uddin Ujjan 1 & Naveed Zaman Akhunzada 1 & Yousaf Bashir Hadi 1 & Gohar Javed 1 & Muhammad Ehsan Bari 1

Received: 2 May 2015 / Accepted: 24 June 2015 # Springer-Verlag Berlin Heidelberg 2015

Abstract Purpose Management of cerebral edema in infants is challenging. Decompressive craniectomy in young age has shown favorable outcomes for management of intracranial hypertension, but current literature is scarce and consists of only case reports or small series. The purpose of the current study is to report the challenges faced with this procedure and its complications in this peculiar age group. Methods This is a retrospective chart review of infants (less than 1 year of age) undergoing unilateral or bilateral decompressive craniotomy at a tertiary care hospital in Pakistan. Kochi score was used to score outcomes of five infants who underwent the procedure. Results Five infants were included in this series. Operative time for decompressive craniectomy (DC) ranged from 1 h and 40 min to 4 h. Three infants survived to undergo

* Muhammad Ehsan Bari [email protected] Muhammad Riyaz [email protected] Muhammad Waqas [email protected] Badar Uddin Ujjan [email protected] Naveed Zaman Akhunzada [email protected] Yousaf Bashir Hadi [email protected]

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cranioplasty. Two infants recovered with good Kochi scores of 5a and one infant developed hemiparesis (Kochi score 3b). Conclusions Decompressive craniectomy carries good outcomes in selected patients. Risk of bleeding and hemodynamic instability makes this procedure challenging. We found coagulopathy in four of the five patients which poses another challenge to the surgical management of these patients and has not been stressed enough in the previous literature. Keywords Decompressive craniectomy . Cranioplasty . Coagulopathy . Infants

Introduction Management of cerebral edema in infants is challenging [1]. The procedure of decompressive craniectomy is rarely needed in this age group. It is also technically challenging to perform decompressive craniectomy (DC) in this age group as they can rapidly decompensate from blood loss [2]. Flap necrosis and CSF leak from the wound, hydrocephalus, and empyema are dreaded complications of the procedure especially in this age group [1, 3]. Available literature is limited to case reports and case series of small number of patients. Our understanding of the subject is therefore limited. The purpose of the current study is to report the challenges faced with this procedure and its complications in this peculiar age group.

Gohar Javed [email protected]

Methodology

Section of Neurosurgery, The Aga Khan University Hospital, Stadium Road, PO Box 3500, Karachi, Pakistan

This is a retrospective chart review of infants (less than 1 year of age) undergoing unilateral or bilateral decompressive

1

5a 8 months

7

3

Cranioplasty (2 months and 3 days) U PC U FFP U PLT U PC U FFP U PLT Y Y 10/15 Left SDH + left frontoparietal fracture (road traffic accident)

Right MCA infarct + SDH (fall) 8 months

6 months

4

5

F

Left frontoparietal hemorrhage + SDH (Lt MCA aneurysm) 4 months 3

F

Bilateral Coagulopathic

1 1 1 4 4 4 Unilateral trauma flap Coagulopathic Y Y 8/15

Unilateral trauma flap Y 13/15

N

Unilateral trauma flap Coagulopathy Y Y 6/15 Left-sided large subdural (spontaneous) 4 months 2 days 2

F

Right-sided large acute SDH (spontaneous) 1 month 11 days 1

M

Diagnosis Gender Age S No

Table 1

Decompressive craniectomy procedures were performed at the Aga Khan University Hospital. Of these, we included five patients who were less than 1 year of age. The youngest child was 1-month and 11-day-old male baby while an 8-month female child was the eldest in the group. Two patients presented following a history of trauma while three cases were of spontaneous hemorrhage. A 4-month female child had large left-sided MCA aneurysm. A 1-month and 11-day-old child had hemorrhagic disease of newborn whereas one child had chronic liver failure. Demographic and clinical data of these patients is summarized in Table 1. CT scan of cases 1, 3, and 5 are shown in Figs. 1, 2, and 3, respectively. One patient expired during hospital stay while another passed away after getting discharge home. The rest of the three patients survived to undergo cranioplasty.

Patient demographic and clinical data

Results

M

3b 16 months 5

Cranioplasty (2 month and 6 days) VP shunt insertion (7 months after cranioplasty)

1 4 months 2

5a 40 months Cranioplasty (5 months) VP shunt insertion (3 months after cranioplasty) 4 11/15

Y

Y

Unilateral trauma flap Coagulopathic

2 1 1 1 4 2 6 1 3 3 6

U PC U FFP U Cryo U PLT U PC U PLT U FFP U Cryo U PC U FFP U PLT

Other procedures Preop GCS

Anisocoria

Coagulopathy

Procedure

Perioperative transfusion

craniotomy at the Aga Khan University Hospital Karachi in the last 5 years (1 January 2009 to 31 December 2014). We reviewed the data in February 2015. We retrieved online operating room record for all the decompressive craniectomies done over the study period. Age of each patient was checked using patient care inquiry from another hospital online database for identification of eligible patients. All the children within 1 year of age from either gender and any etiology were included. Medical records of identified patients were retrieved from medical record department. We pretested the pro forma which included variables of age, gender, mechanism of injury or history of illness, delay in presentation to emergency room, hematological and metabolic parameters at arrival with details of surgery, and account of intraoperative blood loss and transfusions. We focused on the details of coagulopathy and transfusion requirements as we believe it is an important aspect of management in this age group. Demographics, mechanisms of injury, and clinical assessment of neurosurgery team as documented by the resident attending surgeon were obtained from initial assessment form. Indication of surgery was recorded from attending’s note. We went through operating notes to record details of surgery such as size and shape of incision and bone flap, duraplasty, and technique of closure. Postoperative course was reviewed from daily progress notes. We specifically looked for postoperative CSF leak, hydrocephalus, neurological recovery, and GOS at discharge. We then called the patient families for and inquired about functional recovery of the kids. Since Kochi score has been used in previous studies, in such cases, we also applied the score to measure outcomes.

ICU stay

Kochi score and length of follow-up

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Childs Nerv Syst Fig. 1 Preoperative (upper) and postoperative (lower) CT scans of case 1. Upper: Preoperative CT scan of the 1-month-old child shows left-sided subdural hematoma, cerebral edema with a midline shift, and effacement ipsilateral basal cisterns and ventricles. Lower: The postoperative images show resolution of midline shift and a bony defect can be appreciated with herniation of the brain

Fig. 2 Preoperative (upper) and postoperative (lower) CT scans of case 3. Upper: Preoperative CT scan of the 3-month-old child shows a large frontotemporal, intracerebral, and subdural hematoma with a significant midline shift. Lower: CT scans show massive hydrocephalus. CSF collections and bony defect can be seen

Childs Nerv Syst Fig. 3 Preoperative (upper) and postoperative (lower) CT scans of case 5. Upper: Preoperative CT scan images of the 8-month-old child show left frontoparietal fracture and subdural hematoma represented by an arrow. Scalp swelling can also be appreciated. Lower: After decompressive craniectomy, CT scan shows resolution of subarachnoid hemorrhage; cerebral edema and scalp swelling can be noted, with significant reduction in left subdural hemorrhage. Craniectomy defect can also be seen

Preoperative consideration All three patients with spontaneous hemorrhage presented with history of seizures, altered consciousness, and vomiting. One patient with trauma also had seizures.

ventriculoperitoneal shunt. Two of the survivors recovered completely with Kochi score of 5a while another has leftsided hemiparesis with Kochi score 3b.

Discussion Operative considerations In all the patients, a question mark incision was given to raise trauma flap and a wide craniectomy was performed. In case of bilateral decompressive craniectomy, a coronal incision was given and a strip of bone was left in place over superior sagittal sinus. Durotomy was performed in all cases with duraplasty using the pericranium in two cases; in other cases surgicel was placed. All the patients had to be transfused intraoperatively. Postoperative consideration Three of our patients survived to undergo cranioplasty and two of those developed hydrocephalus requiring ventriculoperitoneal (VP) shunt insertion. However none of these patients developed flap necrosis or CSF leakage from the wound site. Operative time for DC ranged from 1 h and 40 min to 4 h. One of the patients expired in the hospital while another passed away after discharge. Three patients survived to undergo cranioplasty while two patients had to undergo

Management of increased intracranial pressure in infants and toddlers is challenging. Although guidelines have been established to address this problem [4], many of the current medical modalities like sedative hypnotics, osmotic agent, and ventriculostomies, among others, have their own complications and risks [1]. Moreover, many refractory cases of intracranial hypertension necessitate individual decision-making and use of second tier modalities for many patients [5]. Recently, decompressive craniectomy in young age groups has garnered much interest and has shown favorable outcomes for management of intracranial hypertension [6–8]. Decompressive craniectomy in this age group has been associated with a high rate of complications such as flap necrosis, empyema, and CSF leak from wound site [1, 3]. We report case series of five children less than 1 year of age who underwent DC in the past 5 years. Two of these five patients expired within 30 days. But those who survived showed good recovery with Kochi score of 5a in two cases and 3b in one case. This is the largest series for decompressive craniectomy in infants. Previous data consists of case reports or small case series. Adamo et al. in 2009 [1] published a case series of seven patients, of which two kids were less than 1 year of

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age while two were 12 months old. All of our patients were less than 12 months old with maximum age of 8 months at the time of surgery. None of the children in that series expired but the Kochi score of patients ranged from 3 to 4. One of the two patients that were deceased had a right-sided internal carotid artery full territory infarct along with a subdural hematoma after a history of fall. Her CT angiogram was reported normal. The child did not show any significant improvement postoperatively, and after understanding, the family decided that all the ventilator support should be withdrawn. The other child that passed away had underlying liver failure leading to deficiency of clotting factors. We did extensive work up for her underlying liver disease but could not diagnose the underlying etiology. The child was advised liver transplant which the family could not afford and the child died of liver failure. Another reason for the difference in outcomes in our study when compared to Adamo et al.’s study is our inclusion of different pathologies, unlike Adamo et al.’s study which only included children with traumatic brain injury. We did not encounter any case of flap necrosis or CSF leakage by fistula formation. Previously, authors have recommended the use of T shape incision to decrease the incidence of flap necrosis and fistulas [1]. However, in our experience, question mark shaped incision worked well, and no CSF fistulas were encountered. Meningitis, subgaleal and subdural empyema formation, and bone resorption around skin flap have previously been reported in some cases as complications of the procedure in children [8, 9]. Bulging of the skin flap is commonly seen following the procedure, especially in very young children. Yamaura et al. [3] have discussed 300 cases of cranioplasty following decompressive craniectomy, of which 7 patients were less than 24 months of age [3]. All of these patients developed troublesome bulging of flap. Five patients in their study, in whom dura was not repaired, developed extreme deformity. Two of these patients developed empyema. We performed duraplasty in all cases. Cranial repair after decompressive craniectomy is challenging in children of this age group. Three surviving patients underwent cranioplasty with autologous bone graft. Two of those three patients presented with hydrocephalus and were managed effectively by a VP shunt. One of the two children had a large subgaleal collection but none had significant subdural collections. In 2012, Frassanito studied complications of cranial repair in three infants and found subdural collections and hydrocephalus in all of the cases [10]. Four of our patients were coagulopathic at presentation. Coagulopathy has serious clinical implications. None of the available reports of decompressive craniectomy in children have highlighted this issue. Coagulopathy not only worsens primary brain injury but also has a bearing on the operative management of patients [11]. All these patients had to be transfused intraoperatively. We used packed cells, fresh frozen plasma, and platelets in the same ratio (1:1) in all patients. One

of the patients had an underlying chronic liver disease while others did not have a coagulation abnormality prior to the current illness. Yang et al. [12] recently reported unnecessary preoperative laboratory investigations and cross match in pediatric neurosurgical patients, but we believe that in the context of DC in infants, it is imperative to have adequate blood workup and prior arrangement of blood products. Greater need of blood products in infants and toddlers was even highlighted by Yang et al. [12]. Peiniger et al. [13] reported that up to 44 % of the children with severe traumatic brain injury have coagulopathy on presentation. It was also established by Bent Whittaker [14] that risk of mortality in those who have coagulopathy is much higher in children less than 2 years of age. Few studies on the topic in infants exist in the literature, and this study is an important addition. Since the existing studies have small samples, it is still difficult to make recommendations on the role of decompressive craniectomy in infants. We believe that a systematic review collecting all the reported cases on the topic will be very useful in this regard.

Conclusion Decompressive craniectomy carries good outcomes in selected patients. Risk of bleeding and hemodynamic instability make this procedure challenging. We found coagulopathy in four of the five patients which poses another challenge to the surgical management of these patients and has not been stressed enough in the previous literature. Since this is a small case series and previous data also consists of small clusters of patients, we suggest a review on the topic and encourage other authors to share their experiences.

Conflict of interest We have no affiliations with or involvement in any organization or entity with any financial interest, or non-financial interest, in the subject matter or materials discussed in this article.

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